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...
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: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S0969996113000740 |
Similar Items
-
Hereditary Spastic Paraplegia: An Update
by: Arun Meyyazhagan, et al.
Published: (2022-02-01) -
Reduced acetylated α-tubulin in SPAST hereditary spastic paraplegia patient PBMCs
by: Gautam Wali, et al.
Published: (2023-03-01) -
Genetic and clinical features of pediatric-onset hereditary spastic paraplegia: a single-center study in Japan
by: Azusa Ikeda, et al.
Published: (2023-05-01) -
Clinical Spectrum of Hereditary Spastic Paraplegia in Children : A study of 74 cases
by: Roshan Koul, et al.
Published: (2013-08-01) -
Hereditary Spastic Paraparesis with Thin Corpus Callosum: Characteristic MRI Findings
by: Eser S Sanverdi, et al.
Published: (2011-12-01)