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- Publisher
- Springer Journals
- Copyright
- Copyright © 2014 by Springer Science+Business Media New York
- Subject
- Chemistry; Bioorganic Chemistry; Biochemistry, general; Animal Anatomy / Morphology / Histology; Animal Biochemistry; Organic Chemistry
- ISSN
- 0145-479X
- eISSN
- 1573-6881
- DOI
- 10.1007/s10863-014-9564-x
- pmid
- 25030182
- Publisher site
- See Article on Publisher Site
Abstract
Defects in Complex I assembly is one of the emerging underlying causes of severe mitochondrial disorders. The assembly of Complex I has been difficult to understand due to its large size, dual genetic control and the number of proteins involved. Mutations in Complex I subunits as well as assembly factors have been reported to hinder its assembly and give rise to a range of mitochondria disorders. In this review, we summarize the recent progress made in understanding the Complex I assembly pathway. In particularly, we focus on the known as well as novel assembly factors and their role in assembly of Complex I and human disease.
Journal
Journal of Bioenergetics and Biomembranes – Springer Journals
Published: Jul 17, 2014