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. 1987 May 1;243(3):841–845. doi: 10.1042/bj2430841

Cystine storage in cultured myotubes from patients with nephropathic cystinosis.

G S Harper 1, I Bernardini 1, O Hurko 1, J Zuurveld 1, W A Gahl 1
PMCID: PMC1147933  PMID: 3663103

Abstract

Sorted muscle cells, cultured from a patient with nephropathic cystinosis, stored 100 times normal amounts of cystine. Subcellular fractionation and density-gradient centrifugation confirmed that the cystine was located in a lysosomal compartment. 2. Myoblasts from cystinotic patients in culture underwent fusion to myotubes in a normal fashion. 3. The free thiol cysteamine effectively depleted cystinotic-muscle cells of cystine. 4. Cultured myoblast and myotubes offered a unique system for investigating the effects of lysosomal storage on differentiated cell functions.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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