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Cystine storage in cultured myotubes from patients with nephropathic cystinosis.


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.

SUBMITTER: Harper GS 

PROVIDER: S-EPMC1147933 | biostudies-other | 1987 May

REPOSITORIES: biostudies-other

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