Oxidative Stress in Cystinosis Patients
نویسندگان
چکیده
منابع مشابه
Oxidative Stress in Cystinosis Patients
BACKGROUND/AIMS Nephropathic cystinosis (NC) is a severe systemic disease and cysteamine improves its prognosis. Lysosomal cystine accumulation is the hallmark of cystinosis and is regarded as the primary defect due to mutations in the CTNS gene. However, there is great evidence that cystine accumulation itself is not responsible for all abnormalities observed in NC. Studies have demonstrated a...
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ژورنال
عنوان ژورنال: Nephron Extra
سال: 2011
ISSN: 1664-5529
DOI: 10.1159/000331445