Article
Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13).
Neuroscience - 2 May 2008
Hansen J, Corydon T J, Palmfeldt J, Dürr A, Fontaine B, Nielsen M N, Christensen J H, Gregersen N, Bross P
Abstract excerpt
The mitochondrial chaperonin heat shock protein 60 (Hsp60) assists the folding of a subset of proteins localized in mitochondria and is an essential component of the mitochondrial protein quality control system. Mutations in the HSPD1 gene that encodes Hsp60 have been identified in patients with an autosomal dominant form of hereditary spastic paraplegia (SPG13), a late-onset neurodegenerative disorder...
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