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Article

Lysosomal cystine augments apoptosis and causes the phenotype in cystinosis.

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences - 18 Feb 2005

Thoene Jess G

Abstract excerpt

Nephropathic cystinosis is a lethal inborn error of metabolism that destroys kidney function by age 10 years. It is characterized by lysosomal cystine accumulation. How the cystine causes the phenotype is an open question. We propose that during apoptosis, permeablized lysosomes permit cystine to reach the cytosol where mixed disulfide formation occurs, augmenting apoptosis by interaction with a variety of...

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PMID: 15719032

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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