The Hsp60-(p.V98I) Mutation Associated with Hereditary Spastic Paraplegia SPG13 Compromises Chaperonin Function Both in Vitro and in Vivo

We have previously reported the association of a mutation (c.292G > A/p.V98I) in the human HSPD1 gene that encodes the mitochondrial Hsp60 chaperonin with a dominantly inherited form of hereditary spastic paraplegia. Here, we show that the purified Hsp60-(p.V98I) chaperonin displays decreased ATP...

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Библиографические подробности
Главные авторы: Bross, Peter, Naundrup, Søren, Hansen, Jakob, Nielsen, Marit Nyholm, Christensen, Jane Hvarregaard, Kruhøffer, Mogens, Palmfeldt, Johan, Corydon, Thomas Juhl, Gregersen, Niels, Ang, Debbie, Georgopoulos, Costa, Nielsen, Kåre Lehmann
Формат: Artigo
Язык:Inglês
Опубликовано: American Society for Biochemistry and Molecular Biology 2008
Предметы:
Protein Structure and Folding
Online-ссылка:https://ncbi.nlm.nih.gov/pmc/articles/PMC3259655/
https://ncbi.nlm.nih.gov/pubmed/18400758
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M800548200
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