Pantethine depletes cystinotic fibroblasts of cystine. 1983

J D Butler, and M Zatz

UI MeSH Term Description Entries
D010204 Pantetheine An intermediate in the pathway of coenzyme A formation in mammalian liver and some microorganisms.
D002478 Cells, Cultured Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others. Cultured Cells,Cell, Cultured,Cultured Cell
D003538 Cystamine A radiation-protective agent that interferes with sulfhydryl enzymes. It may also protect against carbon tetrachloride liver damage. Cystineamine,Decarboxycystine,Diaminodiethyldisulfide,2,2'-Dithiobisethanamine,Cystamine Calcium Salt,Cystamine Diacetate,Cystamine Dihydrobromide,Cystamine Dihydrochloride,Cystamine Hydrobromide,Cystamine Hydrochloride,Cystamine Sulfate,Cystamine Sulfate (1:1),Cysteinamine Disulfide,Cystinamin,2,2' Dithiobisethanamine,Calcium Salt, Cystamine,Diacetate, Cystamine,Dihydrobromide, Cystamine,Dihydrochloride, Cystamine,Disulfide, Cysteinamine,Hydrobromide, Cystamine,Hydrochloride, Cystamine,Sulfate, Cystamine
D003553 Cystine A covalently linked dimeric nonessential amino acid formed by the oxidation of CYSTEINE. Two molecules of cysteine are joined together by a disulfide bridge to form cystine. Copper Cystinate,L-Cystine,L Cystine
D003554 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. Cystine Diathesis,Cystine Disease,Cystine Storage Disease,Cystinoses,Cystinosin, Defect of,Cystinosis, Nephropathic,Lysosomal Cystine Transport Protein, Defect Of,Nephropathic Cystinosis,Cystine Diatheses,Cystine Diseases,Cystine Storage Diseases,Cystinoses, Nephropathic,Defect of Cystinosin,Diatheses, Cystine,Diathesis, Cystine,Nephropathic Cystinoses,Storage Disease, Cystine,Storage Diseases, Cystine
D004305 Dose-Response Relationship, Drug The relationship between the dose of an administered drug and the response of the organism to the drug. Dose Response Relationship, Drug,Dose-Response Relationships, Drug,Drug Dose-Response Relationship,Drug Dose-Response Relationships,Relationship, Drug Dose-Response,Relationships, Drug Dose-Response
D005347 Fibroblasts Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules. Fibroblast
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013438 Sulfhydryl Compounds Compounds containing the -SH radical. Mercaptan,Mercapto Compounds,Sulfhydryl Compound,Thiol,Thiols,Mercaptans,Compound, Sulfhydryl,Compounds, Mercapto,Compounds, Sulfhydryl

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