Cystine storage in cultured myotubes from patients with nephropathic cystinosis
- 1 May 1987
- journal article
- research article
- Published by Portland Press Ltd. in Biochemical Journal
- Vol. 243 (3), 841-845
- https://doi.org/10.1042/bj2430841
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 myoblasts and myotubes offered a unique system for investigating the effects of lysosomal storage on diffentiated cell functions.This publication has 20 references indexed in Scilit:
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