The LYR protein subunit NB4M/NDUFA6 of mitochondrial complex I anchors an acyl carrier protein and is essential for catalytic activity

被引：89

作者：

Angerer, Heike ^{[1
]}

Radermacher, Michael ^{[2
]}

Mankowska, Michalina ^{[1
]}

Steger, Mirco ^{[3
]}

Zwicker, Klaus ^{[4
]}

Heide, Heinrich ^{[3
]}

Wittig, Ilka ^{[3
,5
]}

Brandt, Ulrich ^{[5
,6
]}

Zickermann, Volker ^{[1
,5
]}

机构：

[1] Goethe Univ Frankfurt, Inst Biochem 2, Struct Bioenerget Grp, Sch Med, D-60438 Frankfurt, Germany

[2] Univ Vermont, Dept Mol Physiol & Biophys, Coll Med, Burlington, VT 05405 USA

[3] Goethe Univ Frankfurt, SFB Core Unit 815, D-60590 Frankfurt, Germany

[4] Goethe Univ Frankfurt, Inst Biochem 1, Sch Med, D-60590 Frankfurt, Germany

[5] Goethe Univ Frankfurt, Cluster Excellence Macromol Complexes, D-60590 Frankfurt, Germany

[6] Radboud Univ Nijmegen, Med Ctr, Nijmegen Ctr Mitochondrial Disorders, NL-6525 GA Nijmegen, Netherlands

来源：

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA | 2014年 / 111卷 / 14期

基金：

美国国家卫生研究院;

关键词：

NADH-UBIQUINONE OXIDOREDUCTASE; BOVINE HEART-MITOCHONDRIA; YARROWIA-LIPOLYTICA; ACCESSORY SUBUNITS; ENZYME TRANSITION; STRUCTURAL BASIS; IDENTIFICATION; COMPONENT; SYNTHASE; BINDING;

D O I：

10.1073/pnas.1322438111

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

070301 [无机化学]; 070403 [天体物理学]; 070507 [自然资源与国土空间规划学]; 090105 [作物生产系统与生态工程];

摘要：

Mitochondrial complex I is the largest and most complicated enzyme of the oxidative phosphorylation system. It comprises a number of so-called accessory subunits of largely unknown structure and function. Here we studied subunit NB4M [NDUFA6, LYR motif containing protein 6 (LYRM6)], a member of the LYRM family of proteins. Chromosomal deletion of the corresponding gene in the yeast Yarrowia lipolytica caused concomitant loss of the mitochondrial acyl carrier protein subunit ACPM1 from the enzyme complex and paralyzed ubiquinone reductase activity. Exchanging the LYR motif and an associated conserved phenylalanine by alanines in subunit NB4M also abolished the activity and binding of subunit ACPM1. We show, by single-particle electron microscopy and structural modeling, that subunits NB4M and ACPM1 form a subdomain that protrudes from the peripheral arm in the vicinity of central subunit domains known to be involved in controlling the catalytic activity of complex I.

引用

页码：5207 / 5212

页数：6

共 37 条

[1]

The superfamily of mitochondrial Complex1_LYR motif-containing (LYRM) proteins [J].