Page last updated: 2024-11-07

penicillamine and Cystinosis

penicillamine has been researched along with Cystinosis in 23 studies

Penicillamine: 3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease.
penicillamine : An alpha-amino acid having the structure of valine substituted at the beta position with a sulfanyl group.

Cystinosis: A metabolic disease characterized by the defective transport of CYSTINE across the lysosomal membrane due to mutation of a membrane protein cystinosin. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. In the KIDNEY, nephropathic cystinosis is a common cause of RENAL FANCONI SYNDROME.

Research Excerpts

ExcerptRelevanceReference
"In cultured fibroblasts from individuals with cystinosis vacuolation is induced by exposure to L-cysteine-D-penicillamine disulfide."7.65Cystinosis: selective induction of vacuolation in fibroblasts by L-cysteine-D-penicillamine disulfide. ( Bradley, KH; Schulman, JD, 1970)
"In cultured fibroblasts from individuals with cystinosis vacuolation is induced by exposure to L-cysteine-D-penicillamine disulfide."3.65Cystinosis: selective induction of vacuolation in fibroblasts by L-cysteine-D-penicillamine disulfide. ( Bradley, KH; Schulman, JD, 1970)

Research

Studies (23)

TimeframeStudies, this research(%)All Research%
pre-199023 (100.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
MILNE, MD1
WALSHE, JM1
ROSSIER, A1
CALDERA, R1
ODIEVRE, M1
SCHEINBERG, IH1
BERGER, H1
ANTENER, I3
BRECHBUEHLER, T1
STALDER, G1
SCHAERER, K1
PATRICK, AD1
Broyer, M1
Berger, R1
Hambraeus, L1
Broberger, O1
Thoene, JG1
Oshima, RG2
Crawhall, JC3
Schneider, JA4
Hsiung, M1
Yeo, YY1
Itiaba, K1
Schulman, JD4
Zimmerman, TJ1
Hood, CI1
Gasset, AR1
Willis, RC1
Furlong, CE1
Bradley, KH3
Schaub, J1
Bremer, HJ1
Christensen, MF1
Nielsen, JA1
Henriksen, O1
Seegmiller, JE2
Friedmann, T1
Harrison, HE1
Wong, V1
Lietman, PS1
Zoratto, E1
Cocuzza, S1
Bauer, B1

Reviews

4 reviews available for penicillamine and Cystinosis

ArticleYear
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; S

1977
Cystinosis--a review.
    Scottish medical journal, 1975, Volume: 20, Issue:1

    Topics: Animals; Birds; Cystine; Cystinosis; Dimercaprol; Homocystine; Humans; Hydrogen Sulfide; Kidney Tran

1975
"Adolescent" cystinosis. A case presentation and review of the recent literature.
    Archives of ophthalmology (Chicago, Ill. : 1960), 1974, Volume: 92, Issue:3

    Topics: Adolescent; Biopsy; Bone Marrow; Chromosomes; Conjunctiva; Cornea; Cystine; Cystinosis; Diet Therapy

1974
[Diet therapy of inborn errors of metabolism].
    Medizinische Klinik, 1970, Aug-01, Volume: 65, Issue:32

    Topics: Adult; Carbohydrate Metabolism, Inborn Errors; Child; Cystinosis; Diet Therapy; Dietary Carbohydrate

1970

Other Studies

19 other studies available for penicillamine and Cystinosis

ArticleYear
DISORDERS OF AMINO-ACID TRANSPORT.
    British medical journal, 1964, Feb-08, Volume: 1, Issue:5379

    Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cystinosis; Cystinuria; Dog Diseases; Dogs; Kidney Di

1964
CURRENT THERAPEUTICS. 192. PENICILLAMINE.
    The Practitioner, 1963, Volume: 191

    Topics: Chemical Phenomena; Chemistry; Child; Copper; Cystinosis; Cystinuria; Hepatolenticular Degeneration;

1963
[RECENT IDEAS ON CYSTINOSIS].
    Bulletins et memoires de la Societe medicale des hopitaux de Paris, 1963, Nov-15, Volume: 114

    Topics: Amino Acid Metabolism, Inborn Errors; Blood Chemical Analysis; Citric Acid Cycle; Cystinosis; Dimerc

1963
D-PENICILLAMINE, WITH PARTICULAR RELATION TO WILSON'S DISEASE.
    Journal of chronic diseases, 1964, Volume: 17

    Topics: Body Fluids; Copper; Cystinosis; Edetic Acid; Hepatolenticular Degeneration; Humans; Immunologic Tes

1964
[CONTRIBUTION TO THE STUDY OF CYSTINOSIS. OBSERVATIONS ON THE DETERMINATION OF CYSTIN IN THE SERUM AND THERAPEUTIC RESULTS WITH PENICILLAMINE AND ANABOLIC STEROIDS].
    Annales paediatrici. International review of pediatrics, 1964, Volume: 202

    Topics: Amino Acids; Anabolic Agents; Blood; Cystine; Cystinosis; Drug Therapy; Humans; Infant; Penicillamin

1964
[ON THE BIOCHEMISTRY AND THERAPY OF CYSTINOSIS].
    Annales paediatrici. International review of pediatrics, 1964, Volume: 203

    Topics: Body Fluids; Cystinosis; Diet; Diet Therapy; Humans; Methionine; Penicillamine; Pyruvates; Urine

1964
DEFICIENCIES OF -SH-DEPENDENT ENZYMES IN CYSTINOSIS.
    Clinical science, 1965, Volume: 28

    Topics: Alcohol Oxidoreductases; Alkaline Phosphatase; Child; Coenzyme A; Cysteine; Cystine; Cystinosis; Dim

1965
[From genes to enzymes...hereditary diseases of metabolism. I. Cystinosis].
    La Presse medicale, 1967, Jun-10, Volume: 75, Issue:28

    Topics: Cystinosis; Humans; Methionine; Penicillamine

1967
Penicillamine treatment of cystinosis.
    Acta paediatrica Scandinavica, 1967, Volume: 56, Issue:3

    Topics: Amino Acids; Autoanalysis; Cystinosis; Cystinuria; Humans; Infant; Male; Penicillamine; Sulfides

1967
Cysteamine, penicillamine, glutathione, and their derivatives analyzed by automated ion exchange column chromatography.
    Biochemical medicine, 1978, Volume: 19, Issue:3

    Topics: Animals; Autoanalysis; Chromatography, Ion Exchange; Cysteamine; Cystinosis; Ethylmaleimide; Fibrobl

1978
Binding assays for amino acids. The utilization of a cystine binding protein from Escherichia coli for the determination of acid-soluble cystine in small physiological samples.
    The Journal of biological chemistry, 1974, Oct-10, Volume: 249, Issue:19

    Topics: Amino Acids; Amino Acids, Diamino; Bacterial Proteins; Binding Sites; Binding, Competitive; Carbon R

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; Hum

1970
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; Cysti

1970
The metabolism of amino acids, peptides, and disulfides in lysosomes of fibroblasts cultured from normal individuals and those with cystinosis.
    The Journal of experimental medicine, 1970, Dec-01, Volume: 132, Issue:6

    Topics: Amino Acids; Cell Membrane Permeability; Culture Techniques; Cystinosis; Fibroblasts; Humans; Lysoso

1970
Cystinosis: therapeutic implications of in vitro studies of cultured fibroblasts.
    The Journal of pediatrics, 1971, Volume: 78, Issue:5

    Topics: Biopsy; Child; Chromatography; Culture Techniques; Cystine; Cystinosis; Diet Therapy; Fibroblasts; G

1971
Cystinosis. Combined clinical staff conference at the National Institutes of Health.
    Annals of internal medicine, 1968, Volume: 68, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Biopsy; Bone Marrow; Child; Chronic Kidney Disease-Mineral and

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; Cysti

1968
[On a case of cystine renal calculosis].
    Minerva pediatrica, 1968, Oct-13, Volume: 21, Issue:29

    Topics: Child, Preschool; Cystinosis; Humans; Kidney Calculi; Male; Penicillamine; Radiography

1968
[An effective dietetic and medicinal treatment of cystinosis].
    Helvetica paediatrica acta, 1966, Volume: 21, Issue:1

    Topics: Amino Acids; Child, Preschool; Cystine; Cystinosis; Diet Therapy; Humans; Kidney Function Tests; Mal

1966