Impaired clearance of free cystine from lysosome-enriched granular fractions of I-cell-disease fibroblasts.

Cultured fibroblasts from patients with I-cell disease (mucolipidosis II) accumulate excessive amounts of free cystine, similarly to cells from patients with nephropathic cystinosis, a disorder of lysosomal cystine transport. To clarify whether the intralysosomal accumulation of cystine in I-cell-di...

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Detaylı Bibliyografya
Asıl Yazarlar: Tietze, F, Rome, L H, Butler, J D, Harper, G S, Gahl, W A
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: 1986
Konular:
Research Article
Online Erişim:https://ncbi.nlm.nih.gov/pmc/articles/PMC1146941/
https://ncbi.nlm.nih.gov/pubmed/3800891
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