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SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease

Authors :
Philipp Gut
Sanna Matilainen
Jesse G. Meyer
Pieti Pällijeff
Joy Richard
Christopher J. Carroll
Liliya Euro
Christopher B. Jackson
Pirjo Isohanni
Berge A. Minassian
Reem A. Alkhater
Elsebet Østergaard
Gabriele Civiletto
Alice Parisi
Jonathan Thevenet
Matthew J. Rardin
Wenjuan He
Yuya Nishida
John C. Newman
Xiaojing Liu
Stefan Christen
Sofia Moco
Jason W. Locasale
Birgit Schilling
Anu Suomalainen
Eric Verdin
Source :
Nature Communications, Vol 11, Iss 1, Pp 1-14 (2020)
Publication Year :
2020
Publisher :
Nature Portfolio, 2020.

Abstract

The pathomechanism of succinyl-CoA ligase (SCL) deficiency, a hereditary mitochondrial disease, is not fully understood. Here, the authors show that increased succinyl-CoA levels contribute to SCL pathology by causing global protein hyper-succinylation.

Subjects

Subjects :
Science

Details

Language :
English
ISSN :
20411723
Volume :
11
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Nature Communications
Publication Type :
Academic Journal
Accession number :
edsdoj.21b100a7321c4480b27d69ad8980a816
Document Type :
article
Full Text :
https://doi.org/10.1038/s41467-020-19743-4