TY - JOUR
T1 - An update on complex I assembly
T2 - The assembly of players
AU - Vartak, Rasika S.
AU - Semwal, Manpreet Kaur
AU - Bai, Yidong
N1 - Funding Information:
Acknowledgment The related work carried out in the authors’ lab has been supported by grants from National Institute of Health (R21 NS072777, and R01 GM109434) to YB.
PY - 2014/8
Y1 - 2014/8
N2 - 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.
AB - 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.
KW - Assembly
KW - Assembly factors
KW - Complex I
KW - Mitochondrial disorders
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U2 - 10.1007/s10863-014-9564-x
DO - 10.1007/s10863-014-9564-x
M3 - Short survey
C2 - 25030182
AN - SCOPUS:84906333392
SN - 0145-479X
VL - 46
SP - 323
EP - 328
JO - Journal of Bioenergetics and Biomembranes
JF - Journal of Bioenergetics and Biomembranes
IS - 4
ER -