The cardiac-enriched microprotein mitolamban regulates mitochondrial respiratory complex assembly and function in mice.

ABSTRACT: Emerging evidence indicates that a subset of RNA molecules annotated as noncoding contain short open reading frames that code for small functional proteins called microproteins, which have largely been overlooked due to their small size. To search for cardiac-expressed microproteins, we used a comparative genomics approach and identified mitolamban (Mtlbn) as a highly conserved 47-amino acid transmembrane protein that is abundantly expressed in the heart. Mtlbn localizes specifically to the inner mitochondrial membrane where it interacts with subunits of complex III of the electron transport chain and with mitochondrial respiratory supercomplexes. Genetic deletion of Mtlbn in mice altered complex III assembly dynamics and reduced complex III activity. Unbiased metabolomic analysis of heart

SUBMITTER: Makarewich CA 

PROVIDER: S-EPMC8833175 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature