cysteine and Cystinosis

cysteine has been researched along with Cystinosis in 57 studies

Research

Studies (57)

Timeframe	Studies, this research(%)	All Research%
pre-1990	37 (64.91)	18.7374
1990's	5 (8.77)	18.2507
2000's	5 (8.77)	29.6817
2010's	6 (10.53)	24.3611
2020's	4 (7.02)	2.80

Authors

Authors	Studies
Bazin, D; Belletti, G; Colombo, E; Daudon, M; Frochot, V; Hessou, EP; Li, K; Lucas, IT; Moussadik, A; Quaino, P; Rouzière, S; Su, Y; Tielens, F	1
Cairns, D; Hector, E; Michael Wall, G	1
Csidey, M; Csorba, A; Imre, L; Knézy, K; Maka, E; Maneschg, OA; Nagy, ZZ; Resch, M; Szabó, A; Szentmáry, N	1
Adelmann, CH; Chan, SH; Chen, B; Condon, KJ; Kunchok, T; Lewis, CA; Sabatini, DM; Traunbauer, AK	1
Ben-Omran, T; Blom, HJ; Dewik, AN; Gallego-Villar, L; Häberle, J; Hannibal, L; Kruger, WD; Nasrallah, GK; Thöny, B	1
Banerjee, R; Thoene, J; Vitvitsky, V; Witcher, M	1
Bellomo, F; Corallini, S; Emma, F; Laurenzi, C; Palma, A; Pastore, A; Taranta, A	1
Bachhawat, AK; Kumar, A	1
Cherqui, S; Gubler, MC; Harrison, F; Koziol, JA; Salomon, DR; Yeagy, BA	1
Pinkernell, K	1
Helip-Wooley, A; Park, M; Thoene, J	1
ANTENER, I; SCHAERER, K	1
LINNEWEH, F; WILLENBOCKEL, U	1
LINNEWEH, F	1
PATRICK, AD	2
ISRAELS, S; SUDERMAN, HJ	1
Bass, D; Berry, G; DiDomenico, P; Fridge, J; Sarwal, M	1
Junius, S; Kerisit, KG; Park, MA; Pejovic, V; Thoene, JG	1
Fraser-Bell, S; Fung, AT; Ojaimi, E; Sutton, G	1
Antignac, C; Arndt, C; Cazevieille, C; Hamel, C; Hippert, C; Kalatzis, V; Kremer, EJ; Malecaze, F; Maurice, T; Müller, A; Payet, O; Serratrice, N	1
Bradley, K; Schneider, JA; Seegmiller, JE	1
Lemons, RM; Thoene, JG	1
Greene, AA; Jonas, AJ; Schneider, JA; Smith, ML	1
Gahl, WA; Modesti, A; Schulman, JD; Steinherz, R; Tietze, F; Triche, T	1
Bashan, N; Gahl, WA; Schulman, JD; Steinherz, R; Tietze, F	1
Bashan, N; Bernardini, I; Gahl, WA; Schulman, JD; Tietze, F	1
Butler, JD; Foley, B; Mukherjee, AB; Orloff, S; Schulman, JD	1
Briones, P; Camacho, JA; Farré, C; Mas, A; Vilaseca, MA	1
Cambria, RJ; Gordon, RE; Heller, AN; Heller, DS; Schwimmer, A	1
Fromentoux, S; Gobin, E; Guillet, G; Leroy, JP; Sassolas, B	1
Crawhall, JC; Oshima, RG; Schneider, JA; Thoene, JG	1
Kong, CM	1
Schneider, JA; Schulman, JD	1
Patrick, AD; Willcox, P	1
Bowie, L; Crawhall, JC; Gochman, N; Johnson, K; Schneider, JA	1
Cahill, TC; David, DN; Dean, M; Greene, AA; Kaskel, FJ; Pellett, OL; Schneider, JA; Smith, ML; Smolin, LA; Weissbecker, K	1
Berger, R; Bijsterveld, K; Chapman, TE; Reijngoud, DJ; Schierbeek, H; van Luijk, WH	1
Chen, KH; Chi, CS; Ho, WL; Hsiao, KJ; Lee, SH; Lin, E	1
Kohn, G; Makov, N; Rachmel, A; Steinherz, R	1
Clark, KF; Schneider, JA; Smolin, LA	1
Lloyd, JB	1
Bump, EA; Greene, AA; Harms, E; Jonas, AJ; Miller, AL; Pellett, OL; Schneider, JA; Smith, ML	1
Becker, FL; Kroll, WA; Schneider, JA	1
Segal, S; States, B	1
Harris, D; Segal, S; States, B	1
Becker, FL; Garvin, AJ; Horger, EO; Jacobson, C; Kroll, WA; Pellett, OL; Schneider, JA; Spear, GS; Verroust, FM; Wong, VG	1
Schmickel, RD; Waterson, JR; Winter, WP	1
Bradley, KH; Schulman, JD	1
Bradley, KH; Schneider, JA; Schulman, JD; Seegmiller, JE	2
Christensen, MF; Henriksen, O; Nielsen, JA	1
Bradley, KH; Schulman, JD; Seegmiller, JE	1
Copeland, W; Kelly, S; Leikhim, E	1
Bradley, KH; Schneider, JA; Seegmiller, JE	2
Crawhall, JC; Lietman, PS; Schneider, JA; Seegmiller, JE	1

Reviews

3 review(s) available for cysteine and Cystinosis

Article	Year
Cysteamine revisited: repair of arginine to cysteine mutations. Journal of inherited metabolic disease, 2017, Volume: 40, Issue:4 Topics: Animals; Apolipoprotein E3; Arginine; Argininosuccinate Lyase; Cystathionine beta-Synthase; Cysteamine; Cysteine; Cystinosis; Homeostasis; Humans; Hydrogen-Ion Concentration; Molecular Conformation; Mutation; Mutation, Missense; Oxidation-Reduction; Sulfhydryl Compounds; Thromboplastin	2017
Cystine depletion of cystinotic cells by aminothiols. Proceedings of the Royal Society of Medicine, 1977, Volume: 70 Suppl 3 Topics: Cell-Free System; Cystamine; Cysteamine; Cysteine; Cystinosis; Fibroblasts; Humans; Penicillamine; Sulfhydryl Compounds	1977
Cystinosis: a review. Metabolism: clinical and experimental, 1977, Volume: 26, Issue:7 Topics: Biological Transport; Cells, Cultured; Child; Child, Preschool; Cysteine; Cystine; Cystinosis; Eye; Female; Heterozygote; Humans; Infant; Kidney; Kidney Diseases; Kidney Transplantation; Leukocytes; Oxidation-Reduction; Pregnancy; Prenatal Diagnosis; Transplantation, Homologous	1977

Other Studies

54 other study(ies) available for cysteine and Cystinosis

Article	Year
Crystalline structures of L-cysteine and L-cystine: a combined theoretical and experimental characterization. Amino acids, 2022, Volume: 54, Issue:8 Topics: Cysteine; Cystine; Cystinosis; Cystinuria; Disulfides; Humans	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
Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study. BMC ophthalmology, 2020, Feb-26, Volume: 20, Issue:1 Topics: Adolescent; Adult; Anterior Eye Segment; Child; Cornea; Corneal Diseases; Cross-Sectional Studies; Crystallization; Cysteine; Cystinosis; Female; Humans; Male; Microscopy, Confocal; Tomography, Optical Coherence; Visual Acuity; Young Adult	2020
MFSD12 mediates the import of cysteine into melanosomes and lysosomes. Nature, 2020, Volume: 588, Issue:7839 Topics: Biological Transport; Cell Fractionation; Cell Line; Cysteine; Cystine; Cystinosis; Fibroblasts; Humans; Lysosomes; Melanins; Melanosomes; Membrane Proteins; Oxidation-Reduction	2020
The redox status of cystinotic fibroblasts. Molecular genetics and metabolism, 2010, Volume: 99, Issue:4 Topics: Cells, Cultured; Child, Preschool; Cysteine; Cystinosis; Fibroblasts; Glutathione; Glutathione Disulfide; Humans; Lung; Oxidation-Reduction; Skin	2010
Modulation of CTNS gene expression by intracellular thiols. Free radical biology & medicine, 2010, Apr-01, Volume: 48, Issue:7 Topics: Amino Acid Transport Systems, Neutral; Cell Line; Cloning, Molecular; Cysteine; Cystinosis; Humans; Kidney; Oxidation-Reduction; Oxidative Stress; RNA, Small Interfering; Sulfhydryl Compounds; Transgenes	2010
A futile cycle, formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase: the cause of cellular ATP depletion in nephrotic cystinosis? Journal of biosciences, 2010, Volume: 35, Issue:1 Topics: Adenosine Triphosphate; Amino Acid Transport Systems, Neutral; Animals; Cysteine; Cystinosis; Disease Models, Animal; Glutamate-Cysteine Ligase; Glutamic Acid; Humans; Mice; Models, Biological; Pyroglutamate Hydrolase; Pyrrolidonecarboxylic Acid	2010
Kidney preservation by bone marrow cell transplantation in hereditary nephropathy. Kidney international, 2011, Volume: 79, Issue:11 Topics: Alkaline Phosphatase; Amino Acid Transport Systems, Neutral; Animals; Biomarkers; Bone Marrow Transplantation; Cell Differentiation; Cells, Cultured; Chronic Disease; Creatinine; Cysteine; Cystinosis; Disease Models, Animal; Green Fluorescent Proteins; Hematopoietic Stem Cell Transplantation; Kidney; Kidney Diseases; Linear Models; Mice; Mice, Inbred C57BL; Mice, Knockout; Mice, Transgenic; Phosphates; Proteinuria; Time Factors; Transplantation Chimera; Urea	2011
Cellular therapies: what is still missing? Kidney international, 2011, Volume: 79, Issue:11 Topics: Animals; Biomarkers; Bone Marrow Transplantation; Cell Differentiation; Chronic Disease; Cysteine; Cystinosis; Disease Models, Animal; Hematopoietic Stem Cell Transplantation; Humans; Kidney; Kidney Diseases; Mice; Proteinuria; Time Factors; Transplantation Chimera	2011
Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells. Journal of the American Society of Nephrology : JASN, 2002, Volume: 13, Issue:12 Topics: Apoptosis; Cells, Cultured; Cysteine; Cystinosis; Epithelial Cells; Fibroblasts; Humans; Kidney Diseases; Kidney Tubules, Proximal; Lysosomes; Reference Values	2002
[On the therapy and biochemistry of cystinosis]. Annales paediatrici. International review of pediatrics, 1963, Volume: 200 Topics: Biochemistry; Cholecalciferol; Citric Acid Cycle; Corrinoids; Cysteine; Cystinosis; Hematinics; Methionine; Pyruvates; Sulfhydryl Compounds; Vitamin B 12	1963
[THE TOXICITY OF SULPHUR-CONTAINING AMINO-ACIDS AND OF SOME OF THEIR METABOLITES. A CONTRIBUTION TO THE PATHOGENESIS OF CYSTINOSIS]. Annales paediatrici. International review of pediatrics, 1964, Volume: 202 Topics: Alkaline Phosphatase; Amino Acids; Animals; Cysteine; Cystine; Cystinosis; Histocytochemistry; Intestines; Kidney; Lagomorpha; Methionine; Rabbits; Research; Sulfonic Acids; Sulfur; Tissue Extracts; Toxicology	1964
[THE POSSIBILITY OF A DIETARY INFLUENCE ON CYSTINOSIS]. Klinische Wochenschrift, 1964, Jul-15, Volume: 42 Topics: Caseins; Cysteine; Cystine; Cystinosis; Diet; Diet Therapy; Dietary Proteins; Humans; Methionine	1964
DEFICIENCIES OF -SH-DEPENDENT ENZYMES IN CYSTINOSIS. Clinical science, 1965, Volume: 28 Topics: Alcohol Oxidoreductases; Alkaline Phosphatase; Child; Coenzyme A; Cysteine; Cystine; Cystinosis; Dimercaprol; Electron Transport Complex II; Enzymes; Glucose-6-Phosphatase; Glycerolphosphate Dehydrogenase; Hexokinase; Hydro-Lyases; Kidney; Leucyl Aminopeptidase; Liver; Muscles; Penicillamine; Pharmacology; Phosphogluconate Dehydrogenase; Succinate Dehydrogenase; Sulfhydryl Compounds; Tissue Extracts; Transaminases	1965
Cystinosis. The Journal of pediatrics, 1955, Volume: 47, Issue:1 Topics: Child; Cysteine; Cystinosis; Humans; Infant; Metabolic Diseases; Proteins	1955
The degradative metabolism of L-cysteine and L-cystine in vitro by liver in cystinosis. The Biochemical journal, 1962, Volume: 83 Topics: Cysteine; Cystine; Cystinosis; Humans; In Vitro Techniques; Liver	1962
Noncirrhotic portal hypertension in association with juvenile nephropathic cystinosis: case presentation and review of the literature. Journal of inherited metabolic disease, 2004, Volume: 27, Issue:5 Topics: Adult; Biopsy; Creatinine; Cysteamine; Cysteine; Cystinosis; Female; Humans; Hypertension, Portal; Kidney Transplantation; Kupffer Cells; Liver; Liver Diseases; Tomography, X-Ray Computed	2004
Increased apoptosis in cystinotic fibroblasts and renal proximal tubule epithelial cells results from cysteinylation of protein kinase Cdelta. Journal of the American Society of Nephrology : JASN, 2006, Volume: 17, Issue:11 Topics: Apoptosis; Cells, Cultured; Cysteine; Cystinosis; Epithelial Cells; Fibroblasts; Humans; Kidney Tubules, Proximal; Protein Kinase C-delta	2006
In vivo confocal microscopy and polarizing microscopy of the cornea in a patient with nephropathic cystinosis. Clinical & experimental ophthalmology, 2007, Volume: 35, Issue:3 Topics: Adult; Cornea; Corneal Diseases; Crystallization; Cysteine; Cystinosis; Female; Humans; Keratoplasty, Penetrating; Kidney Diseases; Microscopy, Confocal; Microscopy, Polarization	2007
The ocular anomalies in a cystinosis animal model mimic disease pathogenesis. Pediatric research, 2007, Volume: 62, Issue:2 Topics: Amino Acid Transport Systems, Neutral; Animals; Choroid; Ciliary Body; Cornea; Cysteine; Cystinosis; Disease Models, Animal; Disease Progression; Electroretinography; Eye; Eye Diseases; Intraocular Pressure; Iris; Lens, Crystalline; Mice; Mice, Inbred C57BL; Mice, Knockout; Photophobia; Reproducibility of Results; Retina; Sclera; Time Factors	2007
Increased cystine in leukocytes from individuals homozygous and heterozygous for cystinosis. Science (New York, N.Y.), 1967, Sep-15, Volume: 157, Issue:3794 Topics: Acid Phosphatase; Cell Nucleus; Cysteine; Cystine; Cystinosis; Cytoplasmic Granules; Ethylmaleimide; Humans; Leukocytes; Molecular Biology	1967
Cystine accumulation in cystinotic fibroblasts from free and protein-linked cystine but not cysteine. The Biochemical journal, 1982, Dec-15, Volume: 208, Issue:3 Topics: Cell Line; Cysteine; Cystine; Cystinosis; Fibroblasts; Humans; Insulin; Lysosomes; Proteins; Serum Albumin, Bovine	1982
Cystine accumulation and loss in normal, heterozygous, and cystinotic fibroblasts. Proceedings of the National Academy of Sciences of the United States of America, 1982, Volume: 79, Issue:14 Topics: Cell Survival; Cells, Cultured; Cysteine; Cystine; Cystinosis; Disulfides; Fibroblasts; Glutathione; Heterozygote; Humans; Lysosomes	1982
Heterozygote detection in cystinosis, using leukocytes exposed to cystine dimethyl ester. The New England journal of medicine, 1982, Jun-17, Volume: 306, Issue:24 Topics: Cysteine; Cystine; Cystinosis; Female; Genetic Carrier Screening; Humans; Leukocytes; Male; Sulfur Radioisotopes	1982
Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes. The Journal of biological chemistry, 1982, Aug-25, Volume: 257, Issue:16 Topics: Cysteine; Cystine; Cystinosis; Ethylmaleimide; Half-Life; Heterozygote; Humans; Leukocytes; Lysosomes	1982
Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis. Science (New York, N.Y.), 1982, Sep-24, Volume: 217, Issue:4566 Topics: Biological Transport; Carrier Proteins; Cysteine; Cystine; Cystinosis; Ethylmaleimide; Humans; Kinetics; Leukocytes; Lysosomes	1982
Cystinotic and normal fibroblasts: differential susceptibility to cysteine toxicity in vitro. In vitro, 1980, Volume: 16, Issue:8 Topics: Cell Count; Cell Line; Cell Survival; Child; Cysteine; Cystinosis; Fibroblasts; Humans	1980
Biochemical follow-up in late-treated nephropathic cystinosis. Journal of inherited metabolic disease, 1995, Volume: 18, Issue:2 Topics: Carnitine; Creatinine; Cysteamine; Cysteine; Cystinosis; Disease Progression; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Kidney Function Tests; Male; Oxidants	1995
Cystinosis and gingival hyperplasia: demonstration of cystine crystals in gingival tissue and unusual aspects of management. Journal of periodontology, 1994, Volume: 65, Issue:12 Topics: Adult; Anti-Bacterial Agents; Crystallization; Cyclosporine; Cysteine; Cystinosis; Dental Care for Chronically Ill; Gingival Diseases; Gingival Hyperplasia; Humans; Kidney Transplantation; Male; Premedication	1994
Skin storage of cystine and premature skin ageing in cystinosis. Lancet (London, England), 1998, Oct-31, Volume: 352, Issue:9138 Topics: Adult; Cysteine; Cystinosis; Fibroblasts; Humans; Immunosuppressive Agents; Kidney Transplantation; Male; Skin; Skin Aging	1998
Cystinosis: A review. R.I. medical journal, 1975, Volume: 58, Issue:1 Topics: Crystallization; Cysteine; Cystine; Cystinosis; Humans; Kidney Transplantation; Transplantation, Homologous; Uremia	1975
Biochemical diagnosis of cystinosis using leukocytes. Acta paediatrica Scandinavica, 1975, Volume: 64, Issue:1 Topics: Autoradiography; Chromatography, Thin Layer; Cysteine; Cystine; Cystinosis; Glutathione; Humans; Leukocytes; Sulfur Radioisotopes	1975
Automated microanalysis of sulfur containing amino acids and their derivatives. Clinica chimica acta; international journal of clinical chemistry, 1976, May-03, Volume: 68, Issue:3 Topics: Amino Acids; Autoanalysis; Cells, Cultured; Cysteine; Cystinosis; Disulfides; Ethylmaleimide; Fibroblasts; Glutathione; Homocysteine; Humans; Methionine; Methods; Sulfur	1976
Biochemical and genetic analysis of a child with cystic fibrosis and cystinosis. American journal of medical genetics, 1991, Apr-01, Volume: 39, Issue:1 Topics: Cysteine; Cystic Fibrosis; Cystinosis; Female; Humans; Infant, Newborn; Male; Mutation; Pedigree	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
Late-onset type cystinosis: report of a case. Taiwan yi xue hui za zhi. Journal of the Formosan Medical Association, 1987, Volume: 86, Issue:12 Topics: Child; Conjunctiva; Cornea; Crystallization; Cysteine; Cystinosis; Female; Humans	1987
Cystinosis phenotypes have identical defective cystine clearance pattern. Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie, 1987, Volume: 41, Issue:9-10 Topics: Adult; Cells, Cultured; Cysteine; Cystine; Cystinosis; Female; Fibroblasts; Heterozygote; Humans; Infant; Lysosomes; Male; Phenotype; Sulfur Radioisotopes	1987
An improved method for heterozygote detection of cystinosis, using polymorphonuclear leukocytes. American journal of human genetics, 1987, Volume: 41, Issue:2 Topics: Cysteine; Cystinosis; Genetic Carrier Screening; Humans; Neutrophils	1987
Disulphide reduction in lysosomes. The role of cysteine. The Biochemical journal, 1986, Jul-01, Volume: 237, Issue:1 Topics: Cysteine; Cystine; Cystinosis; Disulfides; Humans; Lysosomes; Models, Biological; Oxidation-Reduction; Proteins	1986
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
Measurement of intracellular amino acids in cultured skin fibroblasts. The effect of storage on cystine recovery and evaluation of three methods of cell preparation. Biochemical medicine, 1974, Volume: 10, Issue:4 Topics: Alanine; Amino Acids; Cells, Cultured; Cysteine; Cystinosis; Edetic Acid; Female; Fibroblasts; Freezing; Glutamates; Glycine; Heterozygote; Humans; Isoleucine; Leucine; Male; Methods; Skin; Temperature; Trypsin; Valine	1974
Quantitation of cyst(e)ine in human fibroblasts and separation of cysteines sulfinic acid, cysteic acid and taurine. Clinica chimica acta; international journal of clinical chemistry, 1973, Jan-10, Volume: 43, Issue:1 Topics: Alanine; Chromatography, Ion Exchange; Chromatography, Paper; Cysteine; Cystine; Cystinosis; Fibroblasts; Humans; Methods; Skin; Spectrophotometry; Sulfinic Acids; Sulfonic Acids; Sulfur Isotopes; Taurine	1973
Uptake and utilization of exogenous cystine by cystinotic and normal fibroblasts. The Journal of clinical investigation, 1974, Volume: 53, Issue:4 Topics: Adolescent; Biological Transport; Carbon Radioisotopes; Cells, Cultured; Child; Child, Preschool; Culture Media; Cysteine; Cystine; Cystinosis; Fibroblasts; Glutathione; Humans; In Vitro Techniques; Infant; Kinetics; Methionine; Sulfur Radioisotopes; Time Factors; Tritium	1974
Prenatal diagnosis of cystinosis. The New England journal of medicine, 1974, Apr-18, Volume: 290, Issue:16 Topics: Amniocentesis; Amniotic Fluid; Autoradiography; Cells, Cultured; Chromatography, Thin Layer; Crystallization; Cysteine; Cystine; Cystinosis; Electrophoresis, Paper; Female; Fetal Diseases; Fetus; Gestational Age; Humans; Kidney; Liver; Placenta; Pregnancy; Prenatal Diagnosis; Retina; Spleen; Thymus Gland	1974
Cysteine activation in cultured cystinotic cells. The specific activity of cysteinyl-tRNA synthetase and tRNACys and the determination of the Michaelis-Menten constants for cysteinyl-tRNA synthetase. The Journal of clinical investigation, 1974, Volume: 54, Issue:1 Topics: Amino Acids; Cells, Cultured; Chemical Phenomena; Chemistry, Physical; Cysteine; Cystine; Cystinosis; DNA-Directed RNA Polymerases; Histocytochemistry; In Vitro Techniques; Kinetics; Mathematics; RNA, Transfer; Sulfur Radioisotopes	1974
Cystinosis: selective induction of vacuolation in fibroblasts by L-cysteine-D-penicillamine disulfide. Science (New York, N.Y.), 1970, Aug-07, Volume: 169, Issue:3945 Topics: Biological Transport; Culture Techniques; Cysteine; Cystine; Cystinosis; Cytoplasm; Fibroblasts; Humans; Lysosomes; Microscopy, Phase-Contrast; Penicillamine; Sulfides	1970
Cystine, cysteine, and glutathione metabolism in normal and cystinotic fibroblasts in vitro, and in cultured normal amniotic fluid cells. Clinica chimica acta; international journal of clinical chemistry, 1972, Volume: 37 Topics: Amniotic Fluid; Cells, Cultured; Cysteine; Cystine; Cystinosis; Female; Fetal Diseases; Fibroblasts; Glutathione; Half-Life; Heterozygote; Humans; Kinetics; Pregnancy; Skin; Sulfur Isotopes	1972
Cystine, cysteine, and glutathione metabolism in normal and cystinotic fibroblasts in vitro, and in cultured normal amniotic fluid cells. Clinica chimica acta; international journal of clinical chemistry, 1971, Volume: 35, Issue:2 Topics: Amniotic Fluid; Cells, Cultured; Cysteine; Cystine; Cystinosis; Female; Fibroblasts; Glutathione; Humans; Pregnancy; Sulfur Isotopes	1971
Treatment of cystinosis with a diet poor in cystine and methionine. Acta paediatrica Scandinavica, 1970, Volume: 59, Issue:6 Topics: Amino Acids; Body Height; Body Weight; Child; Child, Preschool; Cysteine; Cystine; Cystinosis; Cystinuria; Diet Therapy; Dwarfism; Ergocalciferols; Female; Humans; Ketoglutaric Acids; Male; Methionine; Pedigree; Penicillamine; Pyruvates; Renal Tubular Transport, Inborn Errors	1970
Cystine: compartmentalization within lysosomes in cystinotic leukocytes. Science (New York, N.Y.), 1969, Nov-28, Volume: 166, Issue:3909 Topics: Centrifugation, Density Gradient; Cysteine; Cystine; Cystinosis; Humans; Leukocytes; Lysosomes; Sucrose; Sulfur Isotopes	1969
Amino acid excretion patterns in the offspring of a 'doubly-heterozygous' cystine stone former. Experientia, 1970, Dec-15, Volume: 26, Issue:12 Topics: Alleles; Amino Acid Metabolism, Inborn Errors; Amino Acids; Arginine; Child; Child, Preschool; Chromatography, Paper; Cysteine; Cystinosis; Cystinuria; Female; Ferrocyanides; Heterozygote; Homocysteine; Humans; Lysine; Molecular Biology; Ornithine	1970
Identification and measurement of cysteine-homocysteine mixed disulfide in plasma. The Journal of laboratory and clinical medicine, 1968, Volume: 71, Issue:1 Topics: Amino Acids; Chromatography, Ion Exchange; Cysteine; Cystine; Cystinosis; Homocysteine; Homocystine; Homocystinuria; Humans; Methionine; Sulfides	1968
Cystinosis. Plasma cystine and cysteine concentrations and the effect of D-penicillamine and dietary treatment. The American journal of medicine, 1968, Volume: 44, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Blood Chemical Analysis; Cysteine; Cystine; Cystinosis; Cystinuria; Diet Therapy; Female; Humans; Infant; Male; Methionine; Nitrogen; Penicillamine; Pyruvates	1968
Transport and intracellular fate of cysteine-35S in leukocytes from normal subjects and patients with cystinosis. Pediatric research, 1968, Volume: 2, Issue:6 Topics: Adult; Biological Transport; Child; Child, Preschool; Culture Techniques; Cysteine; Cystine; Cystinosis; Electrophoresis; Female; Glutathione; Humans; Infant; Leukocytes; Male; Sulfur Isotopes	1968