LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells.

TitleLYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells.
Publication TypeJournal Article
Year of Publication2013
AuthorsSánchez, E, Lobo, T, Fox, JL, Zeviani, M, Winge, DR, Fernandez-Vizarra, E
JournalBiochim Biophys Acta
Volume1827
Issue3
Pagination285-93
Date Published2013 Mar
ISSN0006-3002
KeywordsAmino Acid Sequence, Animals, Apoptosis Regulatory Proteins, ATPases Associated with Diverse Cellular Activities, Electron Transport Complex III, HEK293 Cells, HeLa Cells, Humans, Iron-Sulfur Proteins, Mice, Mitochondrial Proteins, Molecular Chaperones, Molecular Sequence Data, Saccharomyces cerevisiae, Saccharomyces cerevisiae Proteins
Abstract

The mammalian Complex III (CIII) assembly process is yet to be completely understood. There is still a lack in understanding of how the structural subunits are put together and which additional factors are involved. Here we describe the identification and characterization of LYRM7, a human protein displaying high sequence homology to the Saccharomyces cerevisiae protein Mzm1, which was recently shown as an assembly factor for Rieske Fe-S protein incorporation into the yeast cytochrome bc(1) complex. We conclude that human LYRM7, which we propose to be renamed MZM1L (MZM1-like), works as a human Rieske Fe-S protein (UQCRFS1) chaperone, binding to this subunit within the mitochondrial matrix and stabilizing it prior to its translocation and insertion into the late CIII dimeric intermediate within the mitochondrial inner membrane. Thus, LYRM7/MZM1L is a novel human CIII assembly factor involved in the UQCRFS1 insertion step, which enables formation of the mature and functional CIII enzyme.

DOI10.1016/j.bbabio.2012.11.003
Alternate JournalBiochim. Biophys. Acta
Citation Key10.1016/j.bbabio.2012.11.003
PubMed ID23168492
PubMed Central IDPMC3570683
Grant ListR01 GM083292 / GM / NIGMS NIH HHS / United States
T32 HL007576 / HL / NHLBI NIH HHS / United States
GM083292 / GM / NIGMS NIH HHS / United States
T32 HL007576-25 / HL / NHLBI NIH HHS / United States