title: An update on complex I assembly: the assembly of players.
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.
authors.affiliation: Department of Cellular and Structural Biology, UT Health Science Center, San Antonio, TX, USA.
authors.forename: Rasika S
authors.initials: RS
authors.lastname: Vartak
authors.forename: Manpreet Kaur
authors.initials: MK
authors.lastname: Semwal
authors.forename: Yidong
authors.initials: Y
authors.lastname: Bai
mesh_terms: D000818:Animals; D042967:Electron Transport Complex I / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D028361:Mitochondrial Diseases / Q000201:enzymology* / Q000235:genetics / Q000473:pathology; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism*; D009154:Mutation
chemical_list: D024101:Mitochondrial Proteins; D042967:Electron Transport Complex I