Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
about
Long-term tracking of neurological complications of encephalopathy and myopathy in a patient with nephropathic cystinosis: a case report and review of the literatureLAAT-1 is the lysosomal lysine/arginine transporter that maintains amino acid homeostasisCTNS mutations in an American-based population of cystinosis patientsSialic acid storage diseases. A multiple lysosomal transport defect for acidic monosaccharidesCystinosis: a reviewHeptahelical protein PQLC2 is a lysosomal cationic amino acid exporter underlying the action of cysteamine in cystinosis therapyCTNS mutations in patients with cystinosisCysteamine enhances the procoagulant activity of Factor VIII-East Hartford, a dysfunctional protein due to a light chain thrombin cleavage site mutation (arginine-1689 to cysteine)Role of thiols in degradation of proteins by cathepsinsReduction of the secretory response to Escherichia coli heat-stable enterotoxin by thiol and disulfide compounds.The redox status of cystinotic fibroblasts.Cystamine protects from 3-nitropropionic acid lesioning via induction of nf-e2 related factor 2 mediated transcription.Treatment of corneal cystine crystal accumulation in patients with cystinosis.Inhibitors of protein synthesis also inhibit lysosomal proteolysis. Studies using cystinotic fibroblasts.Metabolism of pantethine in cystinosis.Plasma and muscle free carnitine deficiency due to renal Fanconi syndrome.Repeated renal failure with use of captopril in a cystinotic renal allograft recipientCystine depletion of cystinotic cells by aminothiols.Cystinotic fibroblasts accumulate cystine from intracellular protein degradation.Chemotherapeutics and hormesis.Studies in normal and chronic granulomatous disease neutrophils indicate a correlation of tubulin tyrosinolation with the cellular redox state.The history of cystinosis: lessons for clinical management.Effects of oral phosphocysteamine and rectal cysteamine in cystinosis.Ophthalmic manifestations and histopathology of infantile nephropathic cystinosis: report of a case and review of the literature.Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome.Improvement in the renal prognosis in nephropathic cystinosisPharmacokinetics of cysteamine bitartrate following gastrointestinal infusion.Cysteamine therapy: a treatment for cystinosis, not a cure.A cysteine-specific lysosomal transport system provides a major route for the delivery of thiol to human fibroblast lysosomes: possible role in supporting lysosomal proteolysis.Cystine accumulation and loss in normal, heterozygous, and cystinotic fibroblasts.Cystine accumulation and clearance by normal and cystinotic leukocytes exposed to cystine dimethyl ester.Pantethine and cystamine deplete cystine from cystinotic fibroblasts via efflux of cysteamine-cysteine mixed disulfide.Enhancement of residual arylsulfatase B activity in feline mucopolysaccharidosis VI by thiol-induced subunit associationUse of lipophilic probes of membrane potential to assess human neutrophil activation. Abnormality in chronic granulomatous disease.The pathogenesis of cystinosis: mechanisms beyond cystine accumulation.Improving the prognosis of nephropathic cystinosis.Cystinosis: a new perspective.The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives.Evaluation of topical cysteamine therapy in the CTNS(-/-) knockout mouse using in vivo confocal microscopy.Clinical Practice: A Proposed Standardized Ophthalmological Assessment for Patients with Cystinosis.
P2860
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P2860
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
description
1976 nî lūn-bûn
@nan
1976年の論文
@ja
1976年論文
@yue
1976年論文
@zh-hant
1976年論文
@zh-hk
1976年論文
@zh-mo
1976年論文
@zh-tw
1976年论文
@wuu
1976年论文
@zh
1976年论文
@zh-cn
name
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
@ast
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
@en
type
label
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
@ast
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
@en
prefLabel
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
@ast
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
@en
P2093
P2860
P356
P1476
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
@en
P2093
Crawhall JC
Schneider JA
P2860
P304
P356
10.1172/JCI108448
P407
P577
1976-07-01T00:00:00Z