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.
Excerpt | Relevance | Reference |
---|---|---|
"In cultured fibroblasts from individuals with cystinosis vacuolation is induced by exposure to L-cysteine-D-penicillamine disulfide." | 7.65 | Cystinosis: 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." | 3.65 | Cystinosis: selective induction of vacuolation in fibroblasts by L-cysteine-D-penicillamine disulfide. ( Bradley, KH; Schulman, JD, 1970) |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 23 (100.00) | 18.7374 |
1990's | 0 (0.00) | 18.2507 |
2000's | 0 (0.00) | 29.6817 |
2010's | 0 (0.00) | 24.3611 |
2020's | 0 (0.00) | 2.80 |
Authors | Studies |
---|---|
MILNE, MD | 1 |
WALSHE, JM | 1 |
ROSSIER, A | 1 |
CALDERA, R | 1 |
ODIEVRE, M | 1 |
SCHEINBERG, IH | 1 |
BERGER, H | 1 |
ANTENER, I | 3 |
BRECHBUEHLER, T | 1 |
STALDER, G | 1 |
SCHAERER, K | 1 |
PATRICK, AD | 1 |
Broyer, M | 1 |
Berger, R | 1 |
Hambraeus, L | 1 |
Broberger, O | 1 |
Thoene, JG | 1 |
Oshima, RG | 2 |
Crawhall, JC | 3 |
Schneider, JA | 4 |
Hsiung, M | 1 |
Yeo, YY | 1 |
Itiaba, K | 1 |
Schulman, JD | 4 |
Zimmerman, TJ | 1 |
Hood, CI | 1 |
Gasset, AR | 1 |
Willis, RC | 1 |
Furlong, CE | 1 |
Bradley, KH | 3 |
Schaub, J | 1 |
Bremer, HJ | 1 |
Christensen, MF | 1 |
Nielsen, JA | 1 |
Henriksen, O | 1 |
Seegmiller, JE | 2 |
Friedmann, T | 1 |
Harrison, HE | 1 |
Wong, V | 1 |
Lietman, PS | 1 |
Zoratto, E | 1 |
Cocuzza, S | 1 |
Bauer, B | 1 |
4 reviews available for penicillamine and Cystinosis
Article | Year |
---|---|
Cystine depletion of cystinotic cells by aminothiols.
Topics: Cell-Free System; Cystamine; Cysteamine; Cysteine; Cystinosis; Fibroblasts; Humans; Penicillamine; S | 1977 |
Cystinosis--a review.
Topics: Animals; Birds; Cystine; Cystinosis; Dimercaprol; Homocystine; Humans; Hydrogen Sulfide; Kidney Tran | 1975 |
"Adolescent" cystinosis. A case presentation and review of the recent literature.
Topics: Adolescent; Biopsy; Bone Marrow; Chromosomes; Conjunctiva; Cornea; Cystine; Cystinosis; Diet Therapy | 1974 |
[Diet therapy of inborn errors of metabolism].
Topics: Adult; Carbohydrate Metabolism, Inborn Errors; Child; Cystinosis; Diet Therapy; Dietary Carbohydrate | 1970 |
19 other studies available for penicillamine and Cystinosis
Article | Year |
---|---|
DISORDERS OF AMINO-ACID TRANSPORT.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cystinosis; Cystinuria; Dog Diseases; Dogs; Kidney Di | 1964 |
CURRENT THERAPEUTICS. 192. PENICILLAMINE.
Topics: Chemical Phenomena; Chemistry; Child; Copper; Cystinosis; Cystinuria; Hepatolenticular Degeneration; | 1963 |
[RECENT IDEAS ON CYSTINOSIS].
Topics: Amino Acid Metabolism, Inborn Errors; Blood Chemical Analysis; Citric Acid Cycle; Cystinosis; Dimerc | 1963 |
D-PENICILLAMINE, WITH PARTICULAR RELATION TO WILSON'S DISEASE.
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].
Topics: Amino Acids; Anabolic Agents; Blood; Cystine; Cystinosis; Drug Therapy; Humans; Infant; Penicillamin | 1964 |
[ON THE BIOCHEMISTRY AND THERAPY OF CYSTINOSIS].
Topics: Body Fluids; Cystinosis; Diet; Diet Therapy; Humans; Methionine; Penicillamine; Pyruvates; Urine | 1964 |
DEFICIENCIES OF -SH-DEPENDENT ENZYMES IN CYSTINOSIS.
Topics: Alcohol Oxidoreductases; Alkaline Phosphatase; Child; Coenzyme A; Cysteine; Cystine; Cystinosis; Dim | 1965 |
[From genes to enzymes...hereditary diseases of metabolism. I. Cystinosis].
Topics: Cystinosis; Humans; Methionine; Penicillamine | 1967 |
Penicillamine treatment of cystinosis.
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.
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.
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.
Topics: Biological Transport; Culture Techniques; Cysteine; Cystine; Cystinosis; Cytoplasm; Fibroblasts; Hum | 1970 |
Treatment of cystinosis with a diet poor in cystine and methionine.
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.
Topics: Amino Acids; Cell Membrane Permeability; Culture Techniques; Cystinosis; Fibroblasts; Humans; Lysoso | 1970 |
Cystinosis: therapeutic implications of in vitro studies of cultured fibroblasts.
Topics: Biopsy; Child; Chromatography; Culture Techniques; Cystine; Cystinosis; Diet Therapy; Fibroblasts; G | 1971 |
Cystinosis. Combined clinical staff conference at the National Institutes of Health.
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.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Blood Chemical Analysis; Cysteine; Cystine; Cysti | 1968 |
[On a case of cystine renal calculosis].
Topics: Child, Preschool; Cystinosis; Humans; Kidney Calculi; Male; Penicillamine; Radiography | 1968 |
[An effective dietetic and medicinal treatment of cystinosis].
Topics: Amino Acids; Child, Preschool; Cystine; Cystinosis; Diet Therapy; Humans; Kidney Function Tests; Mal | 1966 |