This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Determine whether the myopathy associated with nephropathic cystinosis is accompanied by mitochondrial dysfunction. Determine if daily supplements of carnitine, Coenzyme Q10, biotin, riboflavin and thiamine have a beneficial effect on the myopathy in these patients, perhaps improved mitochondrial function.
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