OMA1 mediates local and global stress responses against protein misfolding in CHCHD10 mitochondrial...
OMA1 mediates local and global stress responses against protein misfolding in CHCHD10 mitochondrial myopathy
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Author / Creator
Shammas, Mario K , Huang, Xiaoping , Wu, Beverly P , Fessler, Evelyn , Song, Insung , Randolph, Nicholas P , Li, Yan , Bleck, Christopher Ke , Springer, Danielle A , Fratter, Carl , Barbosa, Ines A , Powers, Andrew F , Quirós, Pedro M , Lopez-Otin, Carlos , Jae, Lucas T , Poulton, Joanna and Narendra, Derek P
Publisher
United States: American Society for Clinical Investigation
Journal title
Language
English
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Publication information
Publisher
United States: American Society for Clinical Investigation
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Scope and Contents
Contents
Mitochondrial stress triggers a response in the cell's mitochondria and nucleus, but how these stress responses are coordinated in vivo is poorly understood. Here, we characterize a family with myopathy caused by a dominant p.G58R mutation in the mitochondrial protein CHCHD10. To understand the disease etiology, we developed a knock-in mouse model...
Alternative Titles
Full title
OMA1 mediates local and global stress responses against protein misfolding in CHCHD10 mitochondrial myopathy
Authors, Artists and Contributors
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Record Identifier
TN_cdi_doaj_primary_oai_doaj_org_article_ef70aa06d2af4caebf5831735eb5446d
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_ef70aa06d2af4caebf5831735eb5446d
Other Identifiers
ISSN
1558-8238,0021-9738
E-ISSN
1558-8238
DOI
10.1172/JCI157504
