Mitochondrial function and morphology are impaired inparkin-mutant fibroblasts
نویسندگان
چکیده
منابع مشابه
Mutant Parkin Impairs Mitochondrial Function and Morphology in Human Fibroblasts
BACKGROUND Mutations in Parkin are the most common cause of autosomal recessive Parkinson disease (PD). The mitochondrially localized E3 ubiquitin-protein ligase Parkin has been reported to be involved in respiratory chain function and mitochondrial dynamics. More recent publications also described a link between Parkin and mitophagy. METHODOLOGY/PRINCIPAL FINDINGS In this study, we investiga...
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Mutations in the parkin gene are the most common cause of early-onset Parkinson's disease (PD). Parkin, an E3 ubiquitin ligase, is involved in respiratory chain function, mitophagy, and mitochondrial dynamics. Human cellular models with parkin null mutations are particularly valuable for investigating the mitochondrial functions of parkin. However, published results reporting on patient-derived...
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Mutations in parkin cause autosomal recessive Parkinsonism and mitochondrial defects. A recent drug screen identified a class of steroid-like hydrophobic compounds able to rescue mitochondrial function in parkin-mutant fibroblasts. Whilst these possess therapeutic potential, the size and high hydrophobicity of some may limit their ability to penetrate the blood-brain barrier from systemic circu...
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ژورنال
عنوان ژورنال: Annals of Neurology
سال: 2008
ISSN: 0364-5134,1531-8249
DOI: 10.1002/ana.21492