Late onset motoneuron disorder caused by mitochondrial Hsp60 chaperone deficiency in mice

Late onset motoneuron disorder caused by mitochondrial Hsp60 chaperone deficiency in mice

Cells rely on efficient protein quality control systems (PQCs) to maintain proper activity of mitochondrial proteins. As part of this system, the mitochondrial chaperone Hsp60 assists folding of matrix proteins and it is an essential protein in all organisms. Mutations in Hspd1, the gene encoding Hs...

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Bibliographic Details
Main Authors: Raffaella Magnoni, Johan Palmfeldt, Jane H. Christensen, Majken Sand, Francesca Maltecca, Thomas J. Corydon, Mark West, Giorgio Casari, Peter Bross
Format: Article
Language:English
Published: Elsevier 2013-06-01
Series:Neurobiology of Disease
Subjects:
Hereditary spastic paraplegia
Animal model
Heat shock protein
Mitochondrial dysfunction
Neurodegenerative disorders
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996113000740

Internet

http://www.sciencedirect.com/science/article/pii/S0969996113000740

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