Compounds > cystine

cystine and cysteamine

cystine has been researched along with cysteamine in 133 studies

Research

Studies (133)

TimeframeStudies, this research(%)All Research%
pre-199043 (32.33)18.7374
1990's23 (17.29)18.2507
2000's23 (17.29)29.6817
2010's22 (16.54)24.3611
2020's22 (16.54)2.80

Authors

AuthorsStudies
Butler, JD; Pellefigue, F; Schulman, JD; Spielberg, SP; Tietze, F1
Pollard, AC; Roy, LP1
Crawhall, JC; Olson, DL; Oshima, RG; Schneider, JA; Thoene, JG1
Kamoshita, S; Watanabe, H1
Bernardini, I; Charnas, L; Dalakas, MC; Gahl, WA; Ishak, KG; Markello, TC1
Ayadi, A; Besbes, A; Hammami, M; Pousse, H; Sfar, MT; Slimane, MN1
Gräf, M; Grote, A; Wagner, F1
Choughuley, AS; Shenoy, NR1
Baker, T; Chantler, C; Dalton, RN; Duke, LC; Haycock, GB; Smith, SP; van't Hoff, WG1
Broyer, M; Ged, C; Jean, G; Kamoun, P; Tete, MJ1
Bradbury, JA; Brocklebank, T; Danjoux, JP; Spencer, M; Voller, J1
Berger, R; Bijsterveld, K; Chapman, TE; Reijngoud, DJ; Schierbeek, H; van Luijk, WH1
Issels, RD; Nagele, A1
Brodie, AE; Reed, DJ1
Broyer, M; Tete, MJ1
Forster, S; Lloyd, JB; Scarlett, L1
Butler, JD1
Foreman, JW1
Butler, JD; Gahl, WA; Tietze, F1
Di Cola, D; Sacchetta, P1
Gahl, WA; Kaiser-Kupfer, MI; Kuwabara, T; O'Regan, S; Schneider, JA; Thoene, JG1
Eckert, KG; Issels, RD; Nagele, A; Wilmanns, W1
Hengartner, H; Schott, H; Schwendener, RA; Seeling, R1
Clark, KF; Gahl, WA; Schneider, JA; Smolin, LA; Thoene, JG1
Corden, BJ; Denman, DW; Gahl, WA; Jonas, AJ; Reed, GF; Rizzo, WB; Schlesselman, JJ; Schneider, JA; Schulman, JD; Thoene, JG1
Bernardini, I; Gahl, WA; Harper, GS; Hurko, O; Zuurveld, J1
Danpure, CJ; Fyfe, DA; Jennings, PR1
Butler, JD; Gahl, WA; Schulman, JD; Tietze, F1
Butler, JD; Gahl, WA; Thoene, JG; Wittwer, CT; Zatz, M1
Blumberg, A; da Silva, VA; Lavanchy, P; Lüthy, CM; Oetliker, OH; Suter, H; Wyss, SR; Zurbrügg, RP1
Bump, EA; Greene, AA; Harms, E; Jonas, AJ; Miller, AL; Pellett, OL; Schneider, JA; Smith, ML1
Dertinger, H; Nicolau, C1
Kallistratos, G; Malorny, G1
Arghittu, C1
Saxena, VP; Wetlaufer, DB1
Bacon, VA; Halpern, B; Jellum, E; Patton, W; Pereira, W1
Barboni, E; Cavallini, D; Federici, G1
Gleason, JG; Harpp, DN1
Neumann, H; Smith, RA1
Airee, SK; Gorin, G; Quintiliani, M1
Dunn, JA; Greenberg, RN; Guerrant, RL1
Dumoulin, R; Frederich, A; Guibaud, P; Louis, JJ; Zabot, MT1
Baltassat, P; Bureau, J; Dumoulin, R; Frederich, A; Guibaud, P; Larbre, F; Louis, JJ; Parchoux, B; Zabot, MT1
Schulman, JD; Steinherz, R; Tietze, F1
Harms, E; Oude Elferink, RP; Strijland, A; Tager, JM1
Jonas, AJ; Pellett, OL; Schneider, JA; Smith, ML; Thoene, JG1
Lee, J; Segal, S; States, B2
Ohmori, H; Yamamoto, I1
Bergonzi, E; Bühlmann, C; Herren, A; Lavanchy, P; Lüthy, C; Oetliker, O; Wyss, SR1
Lemons, R; Thoene, JG1
Issels, RD; Meier, T1
Camici, M; Cappiello, M; Del Corso, A; Mura, U1
Bernardini, I; Gahl, WA; Hyman, PE; Ingelfinger, J; Mohan, P; Tangerman, A1
Ohno, K; Yano, T1
Gretz, N; van't Hoff, WG1
Bianchetti, MG; Kernland, K; Lüthy, CM; Wermuth, B1
de Matos, DG; Furnus, CC; Moses, DF1
Scheraga, HA; Sherwood, RW; Thannhauser, TW1
Blom, H; de Graaf-Hess, A; Trijbels, F1
Gahl, WA; Schneider, JA; Thoene, JG1
López-Bejar, M; Mertens, MJ; Paramio, MT; Rodríguez-González, E1
COLETTA, M; DE MARCO, C1
BUDKE, L; VERGROESEN, AJ; VOS, O1
BONKER, GJ; TONGE, BL1
SHAMAKHMUDOV, A1
ELDJARN, L; HAMBRAEUS, L1
AKERFELDT, S; LOEVGREN, G1
Blom, H; de Graaf-Hess, AC; Levtchenko, EN; Monnens, LA; van den Heuvel, LP; Wilmer, M1
de Souza Wyse, AT; Rosa, TG; Wajner, M; Wannmacher, CM1
Blom, H; de Graaf-Hess, A; Levtchenko, E; Monnens, L; van den Heuvel, L; Wilmer, M1
Schneider, JA1
Bernardini, I; Gahl, WA; Kleta, R; Krasnewich, D; Phornphutkul, C; Ueda, M; Varade, WS1
de Souza Wyse, AT; Dutra-Filho, CS; Fleck, RM; Giacomazzi, J; Parissoto, D; Rodrigues Junior, V; Wajner, M; Wannmacher, CM1
Boccia, L; Di Palo, R; Donnay, I; Gasparrini, B; George, F; Marchandise, J; Zicarelli, L1
Blom, HJ; de Graaf-Hess, AC; Levtchenko, EN; Monnens, LA; van Dael, CM; van den Heuvel, LP; Wilmer, MJ1
Barshop, BA; Deutsch, R; Dohil, R; Fidler, M; Gangoiti, J; Martin, M; Schneider, JA1
Ehrich, JH1
Zachwieja, J1
Arevalo do Amaral, MF; Dutra-Filho, CS; Duval Wannmacher, CM; Feksa, LR; Koch, GW; Rech, VC; Terezinha de Souza Wyse, A; Wajner, M1
Gao, D; Lan, GC; Li, Q; Tan, JH; Wang, G; Wu, YG; Zhou, P1
Athaydes, GA; Dornelles, PK; Feksa, LR; Fleck, RM; Rech, VC; Rodrigues-Junior, V; Wannmacher, CM1
Borowitz, MJ; Burns, KH; Confer, B; Emadi, A; Streiff, MB1
Lewerenz, J; Lozano, C; Maher, P; Torres, JL1
Besouw, MT; Janssen, MC; Kremer, JA; Levtchenko, EN1
Berger, JR; Dillon, DA; Goldstein, SJ; Nelson, P; Young, BA1
Feksa, LR; Figueiredo, VC; Wannmacher, CM2
Coelho, D; Macário, MC; Murta, J; Quadrado, MJ; Tavares, R; Torres, A1
Dakshinamurty, KV; Prasad, N; Ram, R; Reddy, PN; Swarnalatha, G1
Li, Q; Luo, MJ; Tan, JH; Wang, G; Wei, DL; Wu, YG; Zhang, J; Zhou, P1
Barshop, BA; Cabrera, BL; Dohil, R; Fidler, M; Gangoiti, JA; Schneider, JA1
Besouw, M; Levtchenko, E1
Casas, J; Jáuregui, O; Martín-Venegas, R; Selga, A; Torres, JL; Vinardell, MP1
Flynn, KJ; Jester, JV; Nien, CJ; Simpson, JL1
Bouazza, N; Chadefaux-Vekemans, B; Deschenes, G; Niaudet, P; Ottolenghi, C; Ricquier, D; Tréluyer, JM; Urien, S1
Bagger, MJ; Cochat, P; Cornelissen, E; Deschênes, G; Gaillard, S; Greenbaum, LA; Grimm, P; Langman, CB; Matossian, D; Morin, D; Niaudet, P; Rioux, P; Sarwal, M1
Buchan, B; Cairns, D; Kay, G; Matthews, KH1
Arias, A; García-Villoria, J; Hernández-Pérez, JM; Ribes, A1
Cao, XY; Cui, W; Han, D; Jiao, GZ; Lian, HY; Miao, YL; Tan, JH; Wu, XF1
Gao, Y; Jiao, GZ; Li, H; Lian, HY; Sun, MJ; Tan, JH; Wang, TY; Wu, XF1
Cui, W; Jiao, GZ; Li, H; Li, Q; Tan, JH; Wang, TY; Zhu, J1
Baudouin, C; Charbit, M; Deschênes, G; Guest, G; Labbé, A; Loirat, C; Niaudet, P1
Bernardini, I; Gahl, WA; Nesterova, G; Williams, C1
Lyou, Y; Nangia, CS; Zhao, X1
McEvoy, B; McMorrow, T; Newsholme, P; Sumayao, R1
Barshop, BA; Deschênes, G; Emma, F; Goodyer, P; Langman, CB; Levtchenko, EN; Lipkin, G; Midgley, JP; Ottolenghi, C; Servais, A; Soliman, NA; Thoene, JG1
Ahlenstiel-Grunow, T; Drube, J; Froede, K; Kanzelmeyer, NK; Kreuzer, M; Lerch, C; Pape, L1
Acharya, G; Isenhart, LC; Jester, JV; Lee, B; Li, F; Liu, X; Marcano, DC; Pflugfelder, SC; Shin, CS; Simpson, J1
Baudouin, C; Labbé, A; Le Mouhaër, J; Liang, H; Plisson, C1
Brand, E; Canaan-Kuehl, S; Kaufeld, J; Kurschat, C; Oh, J; Pape, L; Weber, LT1
Anglicheau, D; Antignac, C; Boddaert, N; Boisgontier, J; Funalot, B; Hummel, A; Legendre, C; Lemaitre, H; Pontoizeau, C; Saitovitch, A; Sberro-Soussan, R; Scemla, A; Servais, A; Snanoudj, R1
D'Souza, RF; Davidson, AJ; Harrison, PT; Hollywood, JA; Holm, TM; Przepiorski, A; Sreebhavan, S; Wolvetang, EJ1
Cornelissen, EAM; Elmonem, MA; Hohenfellner, K; Janssen, MC; Levtchenko, E; Prencipe, G; van den Heuvel, LP; Van Dyck, M; Veys, KRP1
Topaloglu, R1
Akhan, O; Çiftci, T; Demir, H; Demir, N; Gülhan, B; Gültekingil, A; Ozaltin, F; Temucin, ÇM; Topaloglu, R; Yuce, A1
Antignac, C; Ariceta, G; Bertholet-Thomas, A; Bettini, C; Bockenhauer, D; Collin, S; Cornelissen, E; Deschênes, G; Devuyst, O; Emma, F; Greco, M; Haffner, D; Hoff, WV; Hohenfellner, K; Hulton, S; Janssen, M; Levtchenko, E; Niaudet, P; Novo, R; Oh, J; Ozaltin, F; Pape, L; Ravà, L; Servais, A; Topaloglu, R; Veys, K1
Grebe, J; Grüneberg, M; Harms, E; Klank, S; Marquardt, T; Ottolenghi, C; Reunert, J; van Stein, C1
Hohenfellner, K; Keidel, L; Luft, N; Priglinger, C; Priglinger, S; Schworm, B1
Harms, E; Herzig, N; Hohenfellner, K; Holla, H; Knerr, C; Köppl, C; Landfarth, T; Müller-Felber, W; Steidle, G; Vill, K1
Boddaert, N; Boisgontier, J; Hummel, A; Saitovitch, A; Servais, A1
Csorba, A; Kelen, K; Maka, E; Nagy, ZZ; Reusz, G; Szabó, A1
Canalini, F; Cassiman, D; Emma, F; Goffredo, BM; Levtchenko, E; Montini, G; Pennesi, M; Peruzzi, L; Rossi, S; Verrina, E1
Cairns, D; Hector, E; Michael Wall, G1
Gahl, WA; Haffner, D; Hohenfellner, K; Nießl, C; Oh, J; Okorn, C; Palm, K; Schlingmann, KP; Wygoda, S1
Beck, BB; Bieniaś, B; Grenda, R; Kieć-Wilk, B; Kowalczyk, M; Marquardt, T; Nosek, H; Rubik, J; Sikora, P; Surowiec, P; Szymczak, M; Zaniew, M1
Battafarano, G; Bellomo, F; Cairoli, S; Camassei, FD; D'Oria, V; De Leo, E; Del Fattore, A; Emma, F; Goffredo, BM; Pezzullo, M; Polishchuk, E; Polishchuk, R; Raso, R; Rega, LR; Taranta, A1
Addobbati, R; Decorti, G; Del Savio, R; Franzin, M; Rossetto, S; Ruoso, R; Stocco, G1
Al-Sabban, E; Alsaad, KO; Berlingerio, SP; Bondue, T; Kouraich, A; Levtchenko, E; Marie, S; van den Heuvel, L; Veys, K1
Haffner, D; Hohenfellner, K; Zerell, K1
Aase, SA; Bjerre, A; Brackman, D; Forsberg, B; Gudmundsdottir, H; Radtke, M; Siva, C; Woldseth, B1
Bachhawat, AK; Deshpande, AA; Kanakaraj, A; Ravichandran, R; Vashist, N1
Azimpour, K; Carlot, S; Game, D; Lashilola, S; McCarthy, M; van der Voort, J; Xu, W1

Reviews

11 review(s) available for cystine and cysteamine

ArticleYear
Cystinosis.
    Seminars in nephrology, 1989, Volume: 9, Issue:1

    Topics: Cysteamine; Cystine; Cystinosis; Humans; Kidney

1989
NIH conference. Cystinosis: progress in a prototypic disease.
    Annals of internal medicine, 1988, Oct-01, Volume: 109, Issue:7

    Topics: Adolescent; Adult; Biological Transport; Child; Child, Preschool; Crystallization; Cystaphos; Cysteamine; Cystine; Cystinosis; Eye Diseases; Female; Growth; Humans; Kidney Transplantation; Male; Ophthalmic Solutions; Prodrugs; Quebec

1988
[New data in the field of chemical radioprotection. The use and effectiveness of combined radiation protective agents].
    Minerva medica, 1970, Sep-19, Volume: 61, Issue:75

    Topics: Acetylcholine; Animals; Barbiturates; beta-Aminoethyl Isothiourea; Cysteamine; Cystine; Drug Synergism; Mice; Propiophenones; Radiation Injuries, Experimental; Radiation-Protective Agents; Rats; Serotonin

1970
[Lysosomal membrane transport disorders--cystinosis and sialic acid storage disorders (Salla disease, ISSD)].
    Nihon rinsho. Japanese journal of clinical medicine, 1995, Volume: 53, Issue:12

    Topics: Biological Transport; Cysteamine; Cystine; Cystinosis; Humans; Lysosomal Storage Diseases; N-Acetylneuraminic Acid; Sialic Acids

1995
Cystinosis.
    The New England journal of medicine, 2002, Jul-11, Volume: 347, Issue:2

    Topics: Amino Acid Transport Systems, Neutral; Cysteamine; Cystine; Cystinosis; Glycoproteins; Humans; Membrane Proteins; Membrane Transport Proteins; Mutation

2002
[From gene to disease: cystinosis].
    Nederlands tijdschrift voor geneeskunde, 2004, Mar-06, Volume: 148, Issue:10

    Topics: Amino Acid Transport Systems, Neutral; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Gene Deletion; Glycoproteins; Humans; Membrane Proteins; Membrane Transport Proteins; Renal Insufficiency

2004
[Cystinosis--an orphan disease. Pathogenesis and current treatment].
    Przeglad lekarski, 2006, Volume: 63 Suppl 3

    Topics: Adolescent; Adult; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Humans; Infant; Infant, Newborn; Kidney Diseases; Rare Diseases; Renal Insufficiency

2006
[Cystinosis : Diagnosis, cystine-depleting therapy, and transition].
    Der Internist, 2018, Volume: 59, Issue:8

    Topics: Adult; Child; Child, Preschool; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Humans; Kidney; Lysosomes; Male; Quality of Life

2018
Nephropathic cystinosis: an update on genetic conditioning.
    Pediatric nephrology (Berlin, Germany), 2021, Volume: 36, Issue:6

    Topics: Amino Acid Transport Systems, Neutral; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Mutation

2021
Central Nervous System Complications in Cystinosis: The Role of Neuroimaging.
    Cells, 2022, 02-15, Volume: 11, Issue:4

    Topics: Adult; Central Nervous System Diseases; Child; Cysteamine; Cystine; Cystinosis; Diffusion Tensor Imaging; Gray Matter; Humans

2022
Biomarkers in Nephropathic Cystinosis: Current and Future Perspectives.
    Cells, 2022, 06-04, Volume: 11, Issue:11

    Topics: Biomarkers; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Quality of Life

2022

Trials

5 trial(s) available for cystine and cysteamine

ArticleYear
A randomised placebo-controlled trial of topical cysteamine therapy in patients with nephropathic cystinosis.
    Eye (London, England), 1991, Volume: 5 ( Pt 6)

    Topics: Adolescent; Child; Contrast Sensitivity; Cornea; Cysteamine; Cystine; Cystinosis; Double-Blind Method; Female; Humans; Kidney Failure, Chronic; Male; Ophthalmic Solutions; Visual Acuity

1991
Cysteamine therapy for children with nephropathic cystinosis.
    The New England journal of medicine, 1987, Apr-16, Volume: 316, Issue:16

    Topics: Administration, Oral; Child; Child, Preschool; Clinical Trials as Topic; Cysteamine; Cystine; Cystinosis; Growth; Humans; Kidney Diseases; Kidney Glomerulus; Kidney Tubules

1987
Population pharmacokinetics and pharmacodynamics of cysteamine in nephropathic cystinosis patients.
    Orphanet journal of rare diseases, 2011, Dec-23, Volume: 6

    Topics: Adolescent; Adult; Body Weight; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Fanconi Syndrome; Female; Humans; Infant; Male; Nephrotic Syndrome; Radiation-Protective Agents; Treatment Outcome; Young Adult

2011
A randomized controlled crossover trial with delayed-release cysteamine bitartrate in nephropathic cystinosis: effectiveness on white blood cell cystine levels and comparison of safety.
    Clinical journal of the American Society of Nephrology : CJASN, 2012, Volume: 7, Issue:7

    Topics: Adolescent; Adult; Child; Cross-Over Studies; Cysteamine; Cystine; Cystinosis; Delayed-Action Preparations; Fanconi Syndrome; Female; Humans; Leukocytes; Male; Nephrotic Syndrome; Young Adult

2012
A New Viscous Cysteamine Eye Drops Treatment for Ophthalmic Cystinosis: An Open-Label Randomized Comparative Phase III Pivotal Study.
    Investigative ophthalmology & visual science, 2017, 04-01, Volume: 58, Issue:4

    Topics: Adolescent; Cornea; Corneal Diseases; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Humans; Male; Microscopy, Confocal; Ophthalmic Solutions; Retrospective Studies; Tomography, Optical Coherence; Treatment Outcome; Visual Acuity; Young Adult

2017

Other Studies

117 other study(ies) available for cystine and cysteamine

ArticleYear
Depletion of cystine in cystinotic fibroblasts by drugs enclosed in liposomes.
    Pediatric research, 1978, Volume: 12, Issue:1

    Topics: Animals; Culture Techniques; Cysteamine; Cystine; Cystinosis; Fibroblasts; Hydrogen-Ion Concentration; Liposomes; Mice; Phosphatidylcholines

1978
Cysteamine therapy for cystinosis.
    Lancet (London, England), 1978, Sep-30, Volume: 2, Issue:8092 Pt 1

    Topics: Administration, Oral; Child, Preschool; Cysteamine; Cystine; Cystinosis; Female; Follow-Up Studies; Humans; Infant; Leukocytes; Male

1978
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
    The Journal of clinical investigation, 1976, Volume: 58, Issue:1

    Topics: Child; Cystamine; Cysteamine; Cystine; Cystinosis; Female; Fibroblasts; Glutathione; Humans; Leukocytes; Models, Biological; Oxidation-Reduction

1976
[Cystinosis].
    Nihon rinsho. Japanese journal of clinical medicine, 1992, Volume: 50, Issue:7

    Topics: Administration, Oral; Cysteamine; Cystine; Cystinosis; Eye; Genes, Recessive; Humans; Kidney; Kidney Transplantation; Lysosomes

1992
Parenchymal organ cystine depletion with long-term cysteamine therapy.
    Biochemical medicine and metabolic biology, 1992, Volume: 48, Issue:3

    Topics: Adolescent; Child; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Infant; Kidney; Kidney Diseases; Liver; Lung; Male; Microscopy, Electron, Scanning; Muscles; Pancreas; Spleen

1992
[Infantile cystinosis. A new Tunisian case].
    Annales de pediatrie, 1992, Volume: 39, Issue:6

    Topics: Child, Preschool; Cysteamine; Cystine; Cystinosis; Humans; Leukocytes; Male; Ophthalmoscopy; Radiography

1992
[Cysteamine eyedrops for treatment of corneal cysteine deposits in infantile cystinosis].
    Klinische Monatsblatter fur Augenheilkunde, 1992, Volume: 201, Issue:1

    Topics: Child, Preschool; Cornea; Corneal Opacity; Crystallization; Cysteamine; Cystine; Cystinosis; Humans; Male; Ophthalmic Solutions

1992
Inhibitory effect of diet related sulphydryl compounds on the formation of carcinogenic nitrosamines.
    Cancer letters, 1992, Aug-31, Volume: 65, Issue:3

    Topics: Allium; Anticarcinogenic Agents; Chromatography, Gas; Cystamine; Cysteamine; Cysteic Acid; Cysteine; Cystine; Diet; Garlic; Glutathione; Humans; Morpholines; Nitrosamines; Nitrosation; Piperidines; Plants, Medicinal; Pyrrolidines; Sulfhydryl Compounds; Thioglycolates

1992
Effects of oral phosphocysteamine and rectal cysteamine in cystinosis.
    Archives of disease in childhood, 1991, Volume: 66, Issue:12

    Topics: Administration, Oral; Administration, Rectal; Adolescent; Child; Child, Preschool; Circadian Rhythm; Cystaphos; Cysteamine; Cystine; Cystinosis; Female; Humans; Infant; Leukocytes; Male; Time Factors

1991
[Intra-leukocyte cystine in cystinosis treated with cysteamine].
    Annales de biologie clinique, 1991, Volume: 49, Issue:9

    Topics: Adolescent; Adult; Binding, Competitive; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Female; Humans; Infant; Leukocytes; Male; Patient Compliance; Radioimmunoassay

1991
Stable isotope dilution analysis of cystine in granulocyte suspensions as cysteine: a powerful method for the diagnosis, the follow-up, and treatment of patients with cystinosis.
    Clinica chimica acta; international journal of clinical chemistry, 1990, Oct-31, Volume: 191, Issue:1-2

    Topics: Adolescent; Adult; Cell Separation; Child; Cysteamine; Cysteine; Cystine; Cystinosis; Gas Chromatography-Mass Spectrometry; Granulocytes; Humans; Indicator Dilution Techniques; Reference Values

1990
Influence of thiols on thermosensitivity of mammalian cells in vitro.
    Methods in enzymology, 1990, Volume: 186

    Topics: Amifostine; Animals; Biological Transport; Catalase; Cell Division; Cell Line; Cell Survival; Culture Techniques; Cysteamine; Cystine; Glutathione; Glutathione Disulfide; Hot Temperature

1990
Cellular recovery of glyceraldehyde-3-phosphate dehydrogenase activity and thiol status after exposure to hydroperoxides.
    Archives of biochemistry and biophysics, 1990, Volume: 276, Issue:1

    Topics: Cell Line; Cysteamine; Cysteine; Cystine; Dithiothreitol; Ethacrynic Acid; Ethylmaleimide; Glutathione; Glutathione Disulfide; Glyceraldehyde-3-Phosphate Dehydrogenases; Humans; Hydrogen Peroxide; Kinetics; Lung Neoplasms; Oxidation-Reduction; Peroxides; Sulfhydryl Compounds; tert-Butylhydroperoxide; Ultrasonics

1990
[Treatment of cystinosis using cysteamine].
    Annales de pediatrie, 1990, Volume: 37, Issue:2

    Topics: Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Female; Growth; Humans; Infant; Kidney; Leukocytes; Male; Time Factors

1990
Mechanism of cystine reaccumulation by cystinotic fibroblasts in vitro.
    Bioscience reports, 1990, Volume: 10, Issue:2

    Topics: Biological Transport; Cell Membrane; Cells, Cultured; Colchicine; Cysteamine; Cystine; Cystinosis; Fibroblasts; Glutamates; Humans; Kinetics; Lysosomes

1990
Depletion of cystine in cystinotic fibroblasts by homocysteine. Synergism of cysteamine with various reducing agents in depletion of cystine from cystinotic fibroblasts.
    Biochemical pharmacology, 1990, Aug-15, Volume: 40, Issue:4

    Topics: Ascorbic Acid; Cells, Cultured; Cysteamine; Cystine; Cystinosis; Fibroblasts; Glutathione; Homocysteine; Humans

1990
Cystine depletion by WR-1065 in cystinotic cells. Mechanism of action.
    Biochemical pharmacology, 1985, Jun-15, Volume: 34, Issue:12

    Topics: Amifostine; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Humans; In Vitro Techniques; Mercaptoethylamines; Radiation-Protective Agents; Sulfur Radioisotopes

1985
Irreversible inactivation of calcium-dependent proteinases from rat liver by biological disulfides.
    FEBS letters, 1987, Jan-01, Volume: 210, Issue:1

    Topics: Animals; Calpain; Cystamine; Cysteamine; Cysteine; Cystine; Glutathione; Glutathione Disulfide; Kinetics; Liver; Male; Rats; Rats, Inbred Strains; Sulfhydryl Compounds

1987
Promotion of cystine uptake and its utilization for glutathione biosynthesis induced by cysteamine and N-acetylcysteine.
    Biochemical pharmacology, 1988, Mar-01, Volume: 37, Issue:5

    Topics: Acetylcysteine; Animals; Chromatography, High Pressure Liquid; Cricetinae; Cricetulus; Cysteamine; Cystine; Female; Glutathione; Glutathione Disulfide; Hydrogen-Ion Concentration; Ovary; Temperature

1988
Palmitoyl derivatives of L-cysteine, cysteamine, L-cystine, cystamine and their incorporation into the bilayers of unilamellar liposomes.
    Biochimica et biophysica acta, 1988, May-09, Volume: 940, Issue:1

    Topics: Amino Acids; Cystamine; Cysteamine; Cystine; Indicators and Reagents; Lipid Bilayers; Liposomes; Models, Biological; Palmitic Acids

1988
A comparison of the effectiveness of cysteamine and phosphocysteamine in elevating plasma cysteamine concentration and decreasing leukocyte free cystine in nephropathic cystinosis.
    Pediatric research, 1988, Volume: 23, Issue:6

    Topics: Administration, Oral; Child; Child, Preschool; Cystaphos; Cysteamine; Cystine; Cystinosis; Gastrointestinal Contents; Humans; Infant; Leukocytes; Organothiophosphorus Compounds; Patient Acceptance of Health Care; Vomiting

1988
Cystine storage in cultured myotubes from patients with nephropathic cystinosis.
    The Biochemical journal, 1987, May-01, Volume: 243, Issue:3

    Topics: Cells, Cultured; Centrifugation, Density Gradient; Cysteamine; Cystine; Cystinosis; Humans; Muscles

1987
Further studies on the effect of chloroquine on the uptake, metabolism and intracellular translocation of [35S]cystine in cystinotic fibroblasts.
    Biochimica et biophysica acta, 1986, Mar-14, Volume: 885, Issue:3

    Topics: Biological Transport; Cells, Cultured; Chloroquine; Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Hydrogen-Ion Concentration; Lysosomes

1986
Cysteamine depletes cystinotic leucocyte granular fractions of cystine by the mechanism of disulphide interchange.
    The Biochemical journal, 1985, Jun-15, Volume: 228, Issue:3

    Topics: Cystamine; Cysteamine; Cystine; Cystinosis; Cytoplasmic Granules; Disulfides; Ethylmaleimide; Half-Life; Humans; Leukocytes

1985
Metabolism of pantethine in cystinosis.
    The Journal of clinical investigation, 1985, Volume: 76, Issue:4

    Topics: Adolescent; Amidohydrolases; Animals; Child; Cysteamine; Cystine; Cystinosis; Diarrhea; Female; GPI-Linked Proteins; Humans; Intestinal Absorption; Intestinal Mucosa; Kinetics; Leukocytes; Male; Pantetheine; Rats; Rats, Inbred Strains; Sulfhydryl Compounds

1985
Long-term treatment of infantile nephropathic cystinosis with cysteamine.
    The New England journal of medicine, 1985, Dec-05, Volume: 313, Issue:23

    Topics: Bone Marrow; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Growth; Humans; Infant; Infant, Newborn; Kidney Failure, Chronic; Leukocytes; Male

1985
Lysosomal cystine storage in cystinosis and mucolipidosis type II.
    Pediatric research, 1985, Volume: 19, Issue:11

    Topics: Cells, Cultured; Cysteamine; Cysteine; Cystine; Cystinosis; Fibroblasts; Humans; Leukocytes; Liver; Lysosomes; Mucolipidoses

1985
EPR of fast repair reactions by sulfhydryl and disulfide compounds in aqueous solutions.
    Radiation research, 1970, Volume: 42, Issue:1

    Topics: Caffeine; Chemical Phenomena; Chemistry; Cystamine; Cysteamine; Cystine; Electron Spin Resonance Spectroscopy; Ethanol; Glucose; Hydroxides; Methods; Sulfhydryl Compounds; Sulfides; Thymine

1970
[Experimental studies on the chemical dissolution of cystine stones].
    Arzneimittel-Forschung, 1972, Volume: 22, Issue:9

    Topics: Animals; Cysteamine; Cysteine; Cystine; Disulfides; Dithiothreitol; Glutathione; Glycine; Isomerism; Kidney Calculi; Lethal Dose 50; Mercaptopurine; Mice; Penicillamine; Rabbits; Rats; Solubility; Spectrophotometry, Infrared; Sulfhydryl Compounds; Water

1972
Formation of three-dimensional structure in proteins. I. Rapid nonenzymic reactivation of reduced lysozyme.
    Biochemistry, 1970, Dec-08, Volume: 9, Issue:25

    Topics: Air; Animals; Chemical Phenomena; Chemistry; Chickens; Chromatography, Ion Exchange; Circular Dichroism; Cystamine; Cysteamine; Cysteine; Cystine; Edetic Acid; Glutathione; Kinetics; Models, Chemical; Muramidase; Oxidation-Reduction; Protein Denaturation; Sulfhydryl Compounds; Sulfides; Temperature; Time Factors

1970
Quantitative determination of biologically important thiols and disulfides by gas-liquid chromatography.
    Analytical biochemistry, 1969, Oct-01, Volume: 31, Issue:1

    Topics: Aldehydes; Chromatography, Gas; Cystamine; Cysteamine; Cysteine; Cystine; Homocysteine; Homocystine; Hot Temperature; Hydrogen-Ion Concentration; Lactones; Methionine; Methods; Penicillamine; Sulfhydryl Compounds; Sulfides

1969
Interaction of proteins with sulfide.
    European journal of biochemistry, 1970, May-01, Volume: 14, Issue:1

    Topics: Boron Compounds; Chemical Phenomena; Chemistry; Cyanides; Cysteamine; Cystine; Detergents; Guanidines; Hydrogen-Ion Concentration; Kinetics; Protein Denaturation; Proteins; Spectrophotometry; Sulfides; Sulfuric Acids; Taurine; Urea

1970
Preparation and mass spectral properties of cystine and lanthionine derivatives. A novel synthesis of L-lanthionine by selective desulfurization.
    The Journal of organic chemistry, 1971, Jan-15, Volume: 36, Issue:1

    Topics: Amino Acids; Chemical Phenomena; Chemistry; Cysteamine; Cystine

1971
Cleavage of the disulfide bonds of cystine and oxidized glutathione by phosphorothioate.
    Archives of biochemistry and biophysics, 1967, Volume: 122, Issue:2

    Topics: Alkylation; Chemical Phenomena; Chemistry; Chromatography, Ion Exchange; Cysteamine; Cystine; Electrophoresis; Glutathione; Models, Chemical; Oxidation-Reduction; Phosphorus; Phosphorus Isotopes; Spectrophotometry; Sulfhydryl Compounds; Sulfides; Sulfur Isotopes

1967
The inactivation of alcohol dehydrogenase by x-rays and the dose-modifying effect of some added substances.
    Radiation research, 1967, Volume: 32, Issue:4

    Topics: Alcohol Oxidoreductases; Cysteamine; Cysteine; Cystine; Radiation Effects; Radiation-Protective Agents; Radiation-Sensitizing Agents; Radiometry; Sulfur Isotopes; Yeasts

1967
Reduction of the secretory response to Escherichia coli heat-stable enterotoxin by thiol and disulfide compounds.
    Infection and immunity, 1983, Volume: 41, Issue:1

    Topics: Acetylcysteine; Animals; Bacterial Toxins; Body Fluids; Cyclic GMP; Cystamine; Cystathionine; Cysteamine; Cysteine; Cystine; Disulfides; Enterotoxins; Enzyme Activation; Escherichia coli Proteins; Guanylate Cyclase; Intestinal Mucosa; Mice; Rats; Rats, Inbred Strains; Sulfhydryl Compounds

1983
[Treatment of cystinosis with cysteamine].
    Presse medicale (Paris, France : 1983), 1983, Jan-22, Volume: 12, Issue:3

    Topics: Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; In Vitro Techniques; Infant; Male

1983
[Cysteamine in the treatment of cystinosis in children. In vitro and in vivo studies].
    Pediatrie, 1984, Volume: 39, Issue:8

    Topics: Cells, Cultured; Child; Child, Preschool; Cysteamine; Cystine; Cystinosis; Eye Diseases; Female; Fibroblasts; Growth; Humans; Infant; Kidney; Leukocytes; Male

1984
Effects of cysteamine and other thiols on cystine egress from isolated lysosome-rich fractions of cystinotic and normal leucocytes.
    Clinica chimica acta; international journal of clinical chemistry, 1983, Jul-31, Volume: 132, Issue:1

    Topics: Amino Acids; Ascorbic Acid; Cysteamine; Cystine; Cystinosis; Glutathione; Half-Life; Humans; In Vitro Techniques; Leukocytes; Lysosomes; Sulfhydryl Compounds

1983
The intralysosomal pH in cultured human skin fibroblasts in relation to cystine accumulation in patients with cystinosis.
    Biochemical and biophysical research communications, 1983, Oct-14, Volume: 116, Issue:1

    Topics: Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Hydrogen-Ion Concentration; Lysosomes

1983
Renal cell culture using autopsy material from children with cystinosis.
    In vitro, 1984, Volume: 20, Issue:1

    Topics: Autopsy; Cells, Cultured; Child, Preschool; Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Kidney; Male; Pantetheine

1984
Handling of L-[35S]cystine by cysteamine-pretreated cystinotic and normal fibroblasts.
    Archives of biochemistry and biophysics, 1983, Feb-01, Volume: 220, Issue:2

    Topics: Cell Line; Cycloheximide; Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Isotope Labeling; Sulfur Radioisotopes

1983
Mechanism of augmentation of the antibody response in vitro by 2-mercaptoethanol in murine lymphocytes. II. A major role of the mixed disulfide between 2-mercaptoethanol and cysteine.
    Cellular immunology, 1983, Jul-01, Volume: 79, Issue:1

    Topics: Adjuvants, Immunologic; Amino Acids; Animals; Cysteamine; Cystine; Dithiothreitol; Female; Isoantibodies; Lymphocyte Activation; Lymphocytes; Mercaptoethanol; Mice; Mice, Inbred BALB C

1983
Treatment of cystinosis with cysteamine. A pilot study determining dose and form of application.
    Helvetica paediatrica acta, 1981, Volume: 36, Issue:5

    Topics: Child, Preschool; Cysteamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Humans; Infant; Kidney Transplantation; Pilot Projects; Postoperative Complications; Renal Dialysis

1981
Uptake of cystine by cystine-depleted fibroblasts from patients with cystinosis.
    Biochemical and biophysical research communications, 1981, Jan-15, Volume: 98, Issue:1

    Topics: Biological Transport; Cell Line; Child; Cysteamine; Cystine; Cystinosis; Fibroblasts; Humans; Kinetics; Skin

1981
Cystine depletion of cystinotic tissues by phosphocysteamine (WR638).
    The Journal of pediatrics, 1980, Volume: 96, Issue:6

    Topics: Child; Cystaphos; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Fibroblasts; Humans; Male; Organothiophosphorus Compounds

1980
Promotion of cyst(e)ine uptake.
    Methods in enzymology, 1995, Volume: 252

    Topics: Acetylcysteine; Animals; Biological Transport; Cell Adhesion; CHO Cells; Cricetinae; Cysteamine; Cysteine; Cystine; Glutathione; Sulfhydryl Compounds

1995
Thiol and disulfide determination by free zone capillary electrophoresis.
    Journal of biochemical and biophysical methods, 1993, Volume: 26, Issue:4

    Topics: Animals; Cattle; Cystamine; Cysteamine; Cysteine; Cystine; Disulfides; Dithiothreitol; Electrophoresis; Glutathione; Glutathione Disulfide; Oxidation-Reduction; Sensitivity and Specificity; Sulfhydryl Compounds

1993
Intravenous cysteamine therapy for nephropathic cystinosis.
    Pediatric research, 1995, Volume: 38, Issue:4

    Topics: Child, Preschool; Cysteamine; Cystine; Cystinosis; Gastrointestinal Diseases; Gastrointestinal Motility; Humans; Injections, Intravenous; Leukocytes; Male; Microscopy, Electron; Sulfides

1995
The treatment of cystinosis with cysteamine and phosphocysteamine in the United Kingdom and Eire.
    Pediatric nephrology (Berlin, Germany), 1995, Volume: 9, Issue:6

    Topics: Adolescent; Child; Child, Preschool; Cystaphos; Cysteamine; Cystine; Cystinosis; Female; Growth; Humans; Infant; Ireland; Kidney Function Tests; Leukocytes; Male; Retrospective Studies; United Kingdom

1995
Effectiveness of cysteamine and mesna in decreasing intracellular cystine content in cystinosis.
    Nephron, 1996, Volume: 74, Issue:1

    Topics: Cells, Cultured; Cysteamine; Cystine; Cystinosis; Expectorants; Humans; Mesna; Radiation-Protective Agents

1996
Glutathione synthesis during in vitro maturation of bovine oocytes: role of cumulus cells.
    Biology of reproduction, 1997, Volume: 57, Issue:6

    Topics: Acetylcysteine; Animals; Cattle; Cells, Cultured; Coculture Techniques; Cysteamine; Cysteine; Cystine; Female; Glutathione; Oocytes; Ovarian Follicle

1997
Determination of the cysteine and cystine content of proteins by amino acid analysis: application to the characterization of disulfide-coupled folding intermediates.
    Journal of protein chemistry, 1998, Volume: 17, Issue:1

    Topics: Cysteamine; Cysteic Acid; Cysteine; Cystine; Disulfides; Hirudins; Oxidation-Reduction; Protein Folding; Proteins; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Taurine

1998
New method for determining cystine in leukocytes and fibroblasts.
    Clinical chemistry, 1999, Volume: 45, Issue:12

    Topics: Adult; Borohydrides; Bridged Bicyclo Compounds; Cell Extracts; Chromatography, High Pressure Liquid; Cysteamine; Cystine; Cystinosis; Ethylmaleimide; Fibroblasts; Fluorometry; Humans; Leukocytes; Middle Aged; Reproducibility of Results

1999
Effects on in vitro embryo development and intracellular glutathione content of the presence of thiol compounds during maturation of prepubertal goat oocytes.
    Molecular reproduction and development, 2003, Volume: 65, Issue:4

    Topics: Animals; Blastocyst; Cysteamine; Cysteine; Cystine; Embryonic and Fetal Development; Fertilization in Vitro; Glutathione; Goats; Male; Mercaptoethanol; Oocytes; Sperm Head; Sulfhydryl Compounds

2003
Production of S-sulfo-cysteine and S-sulfo-cysteamine from cystine and cystamine, coupled to enzymic transamination and deamination of cysteinesulfinic acid.
    Biochimica et biophysica acta, 1961, Feb-18, Volume: 47

    Topics: Amidohydrolases; Cystamine; Cysteamine; Cysteine; Cystine; Deamination; Mercaptoethylamines; Sulfanilamide; Sulfanilamides; Sulfonamides; Transaminases

1961
Protection of tissueculture cells against ionizing radiation. I. The effect of biological amines, disulphide compounds and thiols.
    International journal of radiation biology and related studies in physics, chemistry, and medicine, 1962, Volume: 5

    Topics: Amines; Cystamine; Cysteamine; Cysteine; Cystine; Epinephrine; Histamine; Norepinephrine; Radiation-Protective Agents; Radiation, Ionizing; Serotonin; Sulfhydryl Compounds; Tissue Culture Techniques

1962
SEPARATION OF CYSTEINE AND ITS OXIDATION PRODUCTS BY PAPER AND THIN-LAYER CHROMATOGRAPHY.
    Journal of chromatography, 1963, Volume: 12

    Topics: Autoradiography; Chromatography; Chromatography, Thin Layer; Cystamine; Cysteamine; Cysteine; Cystine; Oxidation-Reduction; Research; Sulfur Isotopes

1963
[PROTECTIVE EFFECT OF MERCAMINE AND CYSTAMINE IN REPEATED IRRADIATIONS].
    Meditsinskii zhurnal Uzbekistana, 1963, Volume: 8

    Topics: Animals; Cystamine; Cysteamine; Cystine; Growth; Leukocyte Count; Mice; Radiation Effects; Radiation Injuries; Radiation Injuries, Experimental; Rats; Research

1963
THE RATIONALE OF MIXED DISULPHIDES IN THE TREATMENT OF CYSTINURIA.
    Scandinavian journal of clinical and laboratory investigation, 1964, Volume: 16

    Topics: Calculi; Cysteamine; Cystine; Cystinuria; Glutathione; Humans; Metabolism; Penicillamine

1964
SPECTROPHOTOMETRIC DETERMINATION OF DISULFIDES, SULFINIC ACIDS, THIO ETHERS, AND THIOLS WITH THE PALLADIUM (II) ION.
    Analytical biochemistry, 1964, Volume: 8

    Topics: Benzoates; Cysteamine; Cysteine; Cystine; Disulfides; Ethers; Glutathione; Homocysteine; Lactones; Methionine; Palladium; Penicillamine; Phosphates; Research; Spectrophotometry; Sulfhydryl Compounds; Sulfides; Sulfinic Acids; Sulfites; Thiosulfates

1964
Cysteamine prevents and reverses the inhibition of pyruvate kinase activity caused by cystine in rat heart.
    Biochimica et biophysica acta, 2004, Jun-28, Volume: 1689, Issue:2

    Topics: Animals; Cysteamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Enzyme Activation; Heart; Myocardium; Organ Culture Techniques; Pyruvate Kinase; Rats; Rats, Wistar

2004
Comparison of cystine determination in mixed leukocytes vs polymorphonuclear leukocytes for diagnosis of cystinosis and monitoring of cysteamine therapy.
    Clinical chemistry, 2004, Volume: 50, Issue:9

    Topics: Cysteamine; Cystine; Cystinosis; Humans; Neutrophils

2004
Treatment of cystinosis: simple in principle, difficult in practice.
    The Journal of pediatrics, 2004, Volume: 145, Issue:4

    Topics: Adolescent; Child; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Humans; Patient Compliance

2004
Long-term follow-up of well-treated nephropathic cystinosis patients.
    The Journal of pediatrics, 2004, Volume: 145, Issue:4

    Topics: Cysteamine; Cystine; Fanconi Syndrome; Female; Follow-Up Studies; Glomerular Filtration Rate; Growth Disorders; Humans; Infant; Infant, Newborn; Male; Time Factors; Treatment Outcome

2004
Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex.
    Neurochemistry international, 2005, Volume: 46, Issue:5

    Topics: Adenosine Diphosphate; Animals; Binding, Competitive; Cerebral Cortex; Creatine Kinase; Cysteamine; Cystine; Cystinosis; Cytosol; Dose-Response Relationship, Drug; Enzyme Activation; Enzyme Inhibitors; Glutathione; Mitochondria; Nerve Degeneration; Phosphocreatine; Rats; Rats, Wistar; Subcellular Fractions

2005
Enrichment of in vitro maturation medium for buffalo (Bubalus bubalis) oocytes with thiol compounds: effects of cystine on glutathione synthesis and embryo development.
    Theriogenology, 2006, Jan-20, Volume: 65, Issue:2

    Topics: Animals; Buffaloes; Cell Nucleus; Cleavage Stage, Ovum; Culture Media; Cysteamine; Cystine; Efficiency; Embryonic Development; Female; Fertilization; Fertilization in Vitro; Glutathione; Male

2006
Strict cysteamine dose regimen is required to prevent nocturnal cystine accumulation in cystinosis.
    Pediatric nephrology (Berlin, Germany), 2006, Volume: 21, Issue:1

    Topics: Adolescent; Child; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Female; Humans; Male; Neutrophils; Time Factors

2006
Understanding intestinal cysteamine bitartrate absorption.
    The Journal of pediatrics, 2006, Volume: 148, Issue:6

    Topics: Adolescent; Adult; Area Under Curve; Cysteamine; Cystine; Cystinosis; Delayed-Action Preparations; Female; Gastrins; Humans; Intestinal Absorption; Leukocytes; Male; Quality of Life

2006
Cysteamine dose regimen.
    Pediatric nephrology (Berlin, Germany), 2006, Volume: 21, Issue:8

    Topics: Child; Cysteamine; Cystine; Drug Administration Schedule; Humans; Neutrophils

2006
Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester.
    Pediatric nephrology (Berlin, Germany), 2007, Volume: 22, Issue:8

    Topics: Animals; Catalase; Cysteamine; Cystine; Cystinosis; Drug Interactions; Fluoresceins; Glutathione Peroxidase; Hydrogen Peroxide; Kidney; Lipid Peroxidation; Oxidation-Reduction; Oxidative Stress; Proteins; Random Allocation; Rats; Rats, Wistar; Superoxide Dismutase; Thiobarbituric Acid Reactive Substances

2007
The interactions between cysteamine, cystine and cumulus cells increase the intracellular glutathione level and developmental capacity of goat cumulus-denuded oocytes.
    Reproduction (Cambridge, England), 2008, Volume: 135, Issue:5

    Topics: Animals; Blastocyst; Cells, Cultured; Cleavage Stage, Ovum; Cysteamine; Cystine; Drug Synergism; Embryonic Development; Female; Fertilization in Vitro; Glutathione; Goats; Oocytes; Pregnancy

2008
Cysteamine prevents inhibition of thiol-containing enzymes caused by cystine or cystine dimethylester loading in rat brain cortex.
    Metabolic brain disease, 2008, Volume: 23, Issue:2

    Topics: Animals; Antioxidants; Cerebral Cortex; Creatine Kinase; Cysteamine; Cystine; Cystinosis; Disease Models, Animal; Enzyme Inhibitors; Lysosomes; Oxidation-Reduction; Oxidative Stress; Pyruvate Kinase; Rats; Rats, Wistar; Sulfhydryl Compounds

2008
Hematological manifestations of nephropathic cystinosis.
    Acta haematologica, 2008, Volume: 119, Issue:3

    Topics: Adult; Antilymphocyte Serum; Bone Marrow; Combined Modality Therapy; Crystallization; Cysteamine; Cystine; Cystinosis; Humans; Immunosuppressive Agents; Kidney Failure, Chronic; Kidney Transplantation; Macrophages; Male; Pancytopenia; Postoperative Complications; T-Lymphocytes

2008
A novel approach to enhancing cellular glutathione levels.
    Journal of neurochemistry, 2008, Volume: 107, Issue:3

    Topics: Animals; Antioxidants; Biological Transport; Catechin; Chromatography, High Pressure Liquid; Cysteamine; Cysteine; Cystine; Glutamic Acid; Glutathione; Neurons; Oxidative Stress; Rats

2008
Fertility status in male cystinosis patients treated with cysteamine.
    Fertility and sterility, 2010, Volume: 93, Issue:6

    Topics: Adult; Azoospermia; Cysteamine; Cystine; Cystinosis; Female; Fertility; Follicle Stimulating Hormone; Glomerular Filtration Rate; Humans; Luteinizing Hormone; Male; Organ Size; Pregnancy; Semen Analysis; Testis; Testosterone; Young Adult

2010
Cystinosis of the brain and spinal cord with associated vasculopathy.
    Journal of the neurological sciences, 2009, Sep-15, Volume: 284, Issue:1-2

    Topics: Adult; Anti-Inflammatory Agents; Biopsy, Needle; Central Nervous System; Crystallization; Cysteamine; Cystine; Cystinosis; Gait Disorders, Neurologic; Humans; Immunosuppressive Agents; Kidney Transplantation; Magnetic Resonance Imaging; Male; Myelitis; Postoperative Complications; Reoperation; Sensation Disorders; Spinal Cord Compression; Vasculitis, Central Nervous System

2009
Cysteamine prevents inhibition of adenylate kinase caused by cystine in rat brain cortex.
    Metabolic brain disease, 2009, Volume: 24, Issue:4

    Topics: Adenylyl Cyclases; Animals; Brain Chemistry; Brain Diseases, Metabolic; Cerebral Cortex; Creatine Kinase; Cysteamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Drug Interactions; Energy Metabolism; Homeostasis; Lysosomes; Rats; Rats, Wistar

2009
Evaluation of treatment with cysteamine eyedrops for cystinosis with confocal microscopy.
    Cornea, 2009, Volume: 28, Issue:8

    Topics: Corneal Diseases; Crystallization; Crystallography; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Female; Humans; Kidney Diseases; Kidney Transplantation; Medical Records; Microscopy, Confocal; Ophthalmic Solutions; Photophobia; Treatment Outcome; Visual Acuity; Young Adult

2009
Cysteamine prevents inhibition of adenylate kinase caused by cystine in rat brain cortex.
    Metabolic brain disease, 2009, Volume: 24, Issue:3

    Topics: Adenylate Kinase; Animals; Cerebral Cortex; Cysteamine; Cystine; Enzyme Inhibitors; Nerve Tissue Proteins; Rats; Rats, Wistar

2009
Quiz page. Fanconi syndrome caused by cystinosis, intermediate type.
    American journal of kidney diseases : the official journal of the National Kidney Foundation, 2009, Volume: 54, Issue:6

    Topics: Adult; Cysteamine; Cystine; Cystinosis; Diagnosis, Differential; Fanconi Syndrome; Humans; Male; Nephritis, Interstitial; Radiation-Protective Agents; Sjogren's Syndrome

2009
Mouse cumulus-denuded oocytes restore developmental capacity completely when matured with optimal supplementation of cysteamine, cystine, and cumulus cells.
    Biology of reproduction, 2010, Volume: 82, Issue:4

    Topics: Animals; Cell Culture Techniques; Cell Membrane; Cells, Cultured; Culture Media; Cumulus Cells; Cysteamine; Cystine; Embryo Culture Techniques; Female; Fertilization in Vitro; Glutathione; Goats; Male; Mice; Mice, Inbred C57BL; Oocytes; Oogenesis; Pregnancy

2010
Long-term treatment of cystinosis in children with twice-daily cysteamine.
    The Journal of pediatrics, 2010, Volume: 156, Issue:5

    Topics: Adolescent; Child; Creatinine; Cysteamine; Cystine; Cystinosis; Drug Administration Schedule; Female; Humans; Leukocytes; Male; Tablets, Enteric-Coated

2010
Growth retardation in children with cystinosis.
    Minerva pediatrica, 2010, Volume: 62, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Child; Cysteamine; Cystine; Forecasting; Growth Disorders; Human Growth Hormone; Humans; Lysosomes; Recombinant Proteins

2010
Absorption and metabolization of cytoprotective epicatechin thio conjugates in rats.
    Drug metabolism and disposition: the biological fate of chemicals, 2010, Volume: 38, Issue:12

    Topics: Animals; Catechin; Cysteamine; Cysteine; Cystine; Cytoprotection; Intestinal Absorption; Male; Rats; Rats, Sprague-Dawley; Tissue Distribution

2010
Evaluation of topical cysteamine therapy in the CTNS(-/-) knockout mouse using in vivo confocal microscopy.
    Molecular vision, 2011, Volume: 17

    Topics: Administration, Ophthalmic; Amino Acid Transport Systems, Neutral; Animals; Cornea; Crystallization; Cysteamine; Cystine; Cystinosis; Disease Models, Animal; Humans; Mice; Mice, Knockout; Microscopy, Confocal; Ophthalmic Solutions; Treatment Outcome; Visual Acuity

2011
Suppository formulations as a potential treatment for nephropathic cystinosis.
    Journal of pharmaceutical sciences, 2012, Volume: 101, Issue:10

    Topics: Chemistry, Pharmaceutical; Cysteamine; Cystine; Cystinosis; Drug Stability; Excipients; Fanconi Syndrome; Nephrotic Syndrome; Polyethylene Glycols; Solubility; Suppositories

2012
Improvement of the cystine measurement in granulocytes by liquid chromatograhy-tandem mass spectrometry.
    Clinical biochemistry, 2013, Volume: 46, Issue:3

    Topics: Case-Control Studies; Child, Preschool; Chromatography, High Pressure Liquid; Cysteamine; Cystine; Cystinosis; Granulocytes; Humans; Infant; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Time Factors

2013
Developmental potential of prepubertal mouse oocytes is compromised due mainly to their impaired synthesis of glutathione.
    PloS one, 2013, Volume: 8, Issue:3

    Topics: Age Factors; Animals; Blastocyst; Calcium; Cells, Cultured; Chorionic Gonadotropin; Culture Media; Cysteamine; Cystine; Drug Combinations; Embryo Culture Techniques; Embryo, Mammalian; Embryonic Development; Female; Fertilization in Vitro; Glutathione; Gonadotropins, Equine; Mice; Oocytes; Oxidative Stress; Reactive Oxygen Species; Sexual Maturation

2013
Antioxidant supplementation overcomes the deleterious effects of maternal restraint stress-induced oxidative stress on mouse oocytes.
    Reproduction (Cambridge, England), 2013, Volume: 146, Issue:6

    Topics: Animals; Antioxidants; Cells, Cultured; Cysteamine; Cystine; Cytoprotection; Dietary Supplements; Embryo, Mammalian; Embryonic Development; Female; Male; Mice; Oocytes; Oxidative Stress; Pregnancy; Restraint, Physical; Stress, Psychological

2013
Non-frozen preservation protocols for mature mouse oocytes dramatically extend their developmental competence by reducing oxidative stress.
    Molecular human reproduction, 2014, Volume: 20, Issue:4

    Topics: alpha-Tocopherol; Animals; Antioxidants; Blastocyst; Cells, Cultured; Cellular Senescence; Cysteamine; Cystine; Embryonic Development; Female; Glutathione; Mice; Oocytes; Organ Preservation Solutions; Oxidative Stress; Pregnancy; Temperature; Tissue Preservation

2014
A new gel formulation of topical cysteamine for the treatment of corneal cystine crystals in cystinosis: the Cystadrops OCT-1 study.
    Molecular genetics and metabolism, 2014, Volume: 111, Issue:3

    Topics: Administration, Topical; Adolescent; Adult; Child; Cornea; Crystallization; Cysteamine; Cystine; Cystinosis; Female; Gels; Humans; Male; Ophthalmic Solutions; Young Adult

2014
Cystinosis: renal glomerular and renal tubular function in relation to compliance with cystine-depleting therapy.
    Pediatric nephrology (Berlin, Germany), 2015, Volume: 30, Issue:6

    Topics: Administration, Oral; Adolescent; Adult; Age Factors; Biomarkers; Child; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Female; Humans; Kidney; Kidney Failure, Chronic; Kidney Function Tests; Leukocytes; Linear Models; Male; Maximum Tolerated Dose; Medication Adherence; Middle Aged; Retrospective Studies; Risk Factors; Time Factors; Treatment Outcome; Young Adult

2015
Pancytopenia in a patient with cystinosis secondary to myelosuppression from cystine crystal deposition: a case report.
    Journal of medical case reports, 2015, Sep-17, Volume: 9

    Topics: Adult; Bone Marrow; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Female; Flow Cytometry; Humans; Pancytopenia

2015
Lysosomal cystine accumulation promotes mitochondrial depolarization and induction of redox-sensitive genes in human kidney proximal tubular cells.
    The Journal of physiology, 2016, 06-15, Volume: 594, Issue:12

    Topics: Adenosine Triphosphate; Amino Acid Transport Systems, Neutral; Apoptosis; Calcium; Cell Line; Cell Survival; Cysteamine; Cystine; Gene Silencing; Glutathione; Humans; Kidney Tubules, Proximal; Lysosomes; Membrane Potential, Mitochondrial; Mitochondria; Oxidation-Reduction; Oxidative Stress; Reactive Oxygen Species

2016
Controversies and research agenda in nephropathic cystinosis: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.
    Kidney international, 2016, Volume: 89, Issue:6

    Topics: Adolescent; Adult; Age Factors; Amino Acid Transport Systems, Neutral; Child; Congresses as Topic; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Genetic Testing; Genetic Therapy; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppression Therapy; Infant; Kidney Failure, Chronic; Kidney Transplantation; Lysosomes; Mutation; Rare Diseases; Renal Dialysis

2016
Switching from immediate- to extended-release cysteamine in nephropathic cystinosis patients: a retrospective real-life single-center study.
    Pediatric nephrology (Berlin, Germany), 2017, Volume: 32, Issue:1

    Topics: Adolescent; Child; Child, Preschool; Cohort Studies; Cysteamine; Cystine; Cystinosis; Delayed-Action Preparations; Drug Compounding; Female; Glomerular Filtration Rate; Humans; Infant; Leukocytes; Male; Renal Agents; Retrospective Studies; Treatment Outcome

2017
Synergistic Cysteamine Delivery Nanowafer as an Efficacious Treatment Modality for Corneal Cystinosis.
    Molecular pharmaceutics, 2016, 10-03, Volume: 13, Issue:10

    Topics: Animals; Cornea; Cysteamine; Cystine; Cystinosis; Drug Delivery Systems; Female; Mass Spectrometry; Mice; Mice, Inbred C57BL; Ophthalmic Solutions; Treatment Outcome

2016
Central nervous system complications in adult cystinosis patients.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:2

    Topics: Adolescent; Adult; Aged; Case-Control Studies; Central Nervous System Diseases; Cerebrovascular Circulation; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Female; Gray Matter; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Young Adult

2020
Use of Human Induced Pluripotent Stem Cells and Kidney Organoids To Develop a Cysteamine/mTOR Inhibition Combination Therapy for Cystinosis.
    Journal of the American Society of Nephrology : JASN, 2020, Volume: 31, Issue:5

    Topics: Amino Acid Transport Systems, Neutral; Animals; Autophagy; Cell Line; CRISPR-Cas Systems; Cysteamine; Cystine; Cystinosis; Disease Models, Animal; Drug Evaluation, Preclinical; Drug Therapy, Combination; Everolimus; Gene Editing; Heterografts; Humans; Induced Pluripotent Stem Cells; Lysosomes; Mice; Mice, SCID; Organoids; Phenotype; TOR Serine-Threonine Kinases

2020
Chitotriosidase as a Novel Biomarker for Therapeutic Monitoring of Nephropathic Cystinosis.
    Journal of the American Society of Nephrology : JASN, 2020, Volume: 31, Issue:5

    Topics: Adolescent; Adult; Biomarkers; Child; Cysteamine; Cystine; Cystinosis; Drug Monitoring; Female; Hexosaminidases; Humans; Inflammation; Interleukin-18; Interleukin-1beta; Interleukin-6; Leukocytes; Macrophage Activation; Male; Medication Adherence; Peptide Fragments; Prospective Studies; Young Adult

2020
Cystinosis beyond kidneys: gastrointestinal system and muscle involvement.
    BMC gastroenterology, 2020, Jul-29, Volume: 20, Issue:1

    Topics: Child; Cysteamine; Cystine; Cystinosis; Humans; Infant; Kidney; Muscles

2020
An international cohort study spanning five decades assessed outcomes of nephropathic cystinosis.
    Kidney international, 2021, Volume: 100, Issue:5

    Topics: Adult; Child, Preschool; Cohort Studies; Cysteamine; Cystine; Cystine Depleting Agents; Cystinosis; Fanconi Syndrome; Humans

2021
A comparison of immediate release and delayed release cysteamine in 17 patients with nephropathic cystinosis.
    Orphanet journal of rare diseases, 2021, 09-14, Volume: 16, Issue:1

    Topics: Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Quality of Life; Retrospective Studies

2021
Spectral domain optical coherence tomography-based retinochoroidal cystine crystal score: a window into infantile nephropathic cystinosis.
    The British journal of ophthalmology, 2023, Volume: 107, Issue:2

    Topics: Adolescent; Adult; Child; Cornea; Cross-Sectional Studies; Cysteamine; Cystine; Cystinosis; Female; Humans; Male; Tomography, Optical Coherence; Young Adult

2023
Neuromuscular conditions and the impact of cystine-depleting therapy in infantile nephropathic cystinosis: A cross-sectional analysis of 55 patients.
    Journal of inherited metabolic disease, 2022, Volume: 45, Issue:2

    Topics: Adult; Cross-Sectional Studies; Cysteamine; Cystine; Cystinosis; Humans; Neuromuscular Diseases

2022
Ocular treatment of cystinosis with eye drop containing cysteamine
    Orvosi hetilap, 2022, May-22, Volume: 163, Issue:21

    Topics: Cornea; Corneal Diseases; Cysteamine; Cystine; Cystinosis; Humans; Photophobia; Quality of Life; Visual Acuity

2022
Evaluation of NACA and diNACA in human cystinosis fibroblast cell cultures as potential treatments for cystinosis.
    Orphanet journal of rare diseases, 2022, 06-16, Volume: 17, Issue:1

    Topics: Cell Culture Techniques; Cysteamine; Cysteine; Cystine; Cystinosis; Fibroblasts; Humans

2022
Beneficial effects of starting oral cysteamine treatment in the first 2 months of life on glomerular and tubular kidney function in infantile nephropathic cystinosis.
    Molecular genetics and metabolism, 2022, Volume: 136, Issue:4

    Topics: Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Infant; Infant, Newborn; Kidney

2022
Nephropathic cystinosis in Poland: a 40-year retrospective study.
    Polish archives of internal medicine, 2022, 11-25, Volume: 132, Issue:11

    Topics: Adult; Child; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Kidney Failure, Chronic; Poland; Retrospective Studies; Young Adult

2022
Genistein improves renal disease in a mouse model of nephropathic cystinosis: a comparison study with cysteamine.
    Human molecular genetics, 2023, 03-20, Volume: 32, Issue:7

    Topics: Animals; Cysteamine; Cystine; Cystinosis; Disease Models, Animal; Female; Genistein; Kidney; Kidney Diseases; Mice

2023
A new proof of evidence of cysteamine quantification for therapeutic drug monitoring in patients with cystinosis.
    Orphanet journal of rare diseases, 2022, 11-03, Volume: 17, Issue:1

    Topics: Cysteamine; Cystine; Cystinosis; Drug Monitoring; Humans

2022
The Pitfall of White Blood Cell Cystine Measurement to Diagnose Juvenile Cystinosis.
    International journal of molecular sciences, 2023, Jan-09, Volume: 24, Issue:2

    Topics: Adolescent; Adult; Amino Acid Transport Systems, Neutral; Cysteamine; Cystine; Cystinosis; Humans; Leukocytes

2023
Cystinosis.
    Klinische Monatsblatter fur Augenheilkunde, 2023, Volume: 240, Issue:3

    Topics: Cysteamine; Cystine; Cystinosis; Humans; Mutation

2023
The effects of transitioning from immediate release to extended release cysteamine therapy in Norwegian patients with nephropathic cystinosis: a retrospective study.
    Pediatric nephrology (Berlin, Germany), 2023, Volume: 38, Issue:11

    Topics: Adult; Child; Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Retrospective Studies

2023
Cystinosis: Status of research and treatment in India and the world.
    Journal of biosciences, 2023, Volume: 48

    Topics: Cysteamine; Cystine; Cystinosis; Humans; India; Mutation

2023
Impact of compliance to oral cysteamine treatment on the costs of Kidney failure in patients with nephropathic cystinosis in the United Kingdom.
    BMC nephrology, 2023, Nov-29, Volume: 24, Issue:1

    Topics: Cysteamine; Cystine; Cystinosis; Fanconi Syndrome; Humans; Patient Compliance; Renal Dialysis; Renal Insufficiency; United Kingdom

2023