A novel isoform of the human mitochondrial complex I subunit NDUFV3

被引：21
|
作者：
Dibley, Marris G. [1 ]
Ryan, Michael T. [1 ]
Stroud, David A. [1 ]
机构：
[1] Monash Univ, Monash Biomed Discovery Inst, Dept Biochem & Mol Biol, Melbourne, Vic, Australia
来源：
FEBS LETTERS | 2017年 / 591卷 / 01期
基金：
澳大利亚国家健康与医学研究理事会; 英国医学研究理事会;
关键词：
complex I; metabolism; mitochondria; NADH dehydrogenase; OXPHOS; POLYACRYLAMIDE-GEL ELECTROPHORESIS; CHAIN; PROTEINS; DISEASE; SUPERCOMPLEXES; ARCHITECTURE; PREDICTION; REGIONS; RANGE; ARM;
D O I：
10.1002/1873-3468.12527
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
Human mitochondrial complex I is the first enzyme of the mitochondrial respiratory chain. Complex I is composed of 45 subunits, seven encoded by mitochondrial DNA, while the remainder are encoded by nuclear DNA. All nuclear-encoded subunits are thought to be expressed as a single isoform. Here we reveal subunit NDUFV3 to be present in both the canonical 10 kDa and a novel 50 kDa isoform, generated through alternative splicing. Both isoforms assemble into complex I and their levels vary in different tissues. While the 50 kDa isoform appears to be dominant in HEK293T cells, we find either isoform alone is sufficient for assembly of mature complex I. NDUFV3 represents the first known complex I subunit present in two functional isoforms.
引用
收藏
页码：109 / 117
页数：9
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