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Journal List > Biochem J > v.243(3); May 1, 1987

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Biochem J. 1987 May 1; 243(3): 841–845.
PMCID: PMC1147933
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Cystine storage in cultured myotubes from patients with nephropathic cystinosis.
G S Harper, I Bernardini, O Hurko, J Zuurveld, and W A Gahl
Human Genetics Branch, NICHD, Bethesda, MD 20892.
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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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