Mitochondria are composed of many small proteins that control protein synthesis, complex assembly, metabolism, and ion and reactive oxygen species (ROS) handling. We show that a skeletal muscle- and heart-enriched long non-coding RNA, LINC00116, encodes a highly conserved 56-amino-acid microprotein that we named mitoregulin (Mtln). Mtln localizes to the inner mitochondrial membrane, where it binds cardiolipin and influences protein complex assembly. In cultured cells, Mtln overexpression increases mitochondrial membrane potential, respiration rates, and Ca2+ retention capacity while decreasing mitochondrial ROS and matrix-free Ca2+. Mtln-knockout mice display perturbations in mitochondrial respiratory (super)complex formation and activity, fatty acid oxidation, tricarboxylic acid (TCA) cycle enzymes, and Ca2+ retention capacity. Blue-native gel electrophoresis revealed that Mtln co-migrates alongside several complexes, including the complex I assembly module, complex V, and supercomplexes. Under denaturing conditions, Mtln remains in high-molecular-weight complexes, supporting its role as a sticky molecular tether that enhances respiratory efficiency by bolstering protein complex assembly and/or stability.
Pubmed ID: 29949756 RIS Download
Publication data is provided by the National Library of Medicine ® and PubMed ®. Data is retrieved from PubMed ® on a weekly schedule. For terms and conditions see the National Library of Medicine Terms and Conditions.
Portal to interactively visualize genomic data. Provides reference sequences and working draft assemblies for collection of genomes and access to ENCODE and Neanderthal projects. Includes collection of vertebrate and model organism assemblies and annotations, along with suite of tools for viewing, analyzing and downloading data.
View all literature mentionsWeb application for the prediction of transmembrane helices in proteins using Hidden Markov Models. FASTA formatted sequences can be uploaded via file or copy-paste, and output can be formatted as extensive with graphics, extensive without graphics, or one line per protein. Submissions are limited to 10,000 sequences and 4,000,000 amino acids - each sequence is limited to no more than 8,000 amino acids.
View all literature mentionsWeb application for combined transmembrane topology and signal peptide prediction. Used for whole genome annotation of signal peptides and transmembrane regions. Predictor is based on hidden Markov model (HMM) that models different sequence regions of signal peptide and different regions of transmembrane protein in series of interconnected states.
View all literature mentionsThis monoclonal targets Ubiquinol-Cytochrome C Reductase Core Protein I antibody [16D10AD9AH5]
View all literature mentionsThis monoclonal targets FLAG
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis monoclonal targets PDHA1
View all literature mentionsThis polyclonal targets GAPDH
View all literature mentionsThis polyclonal targets LETM1
View all literature mentionsThis monoclonal targets NDUFA9
View all literature mentionsThis polyclonal targets Rabbit IgG (H+L)
View all literature mentionsThis polyclonal targets Mouse IgG (H+L)
View all literature mentionsThis monoclonal targets Glutathione Peroxidase 4 antibody [EPNCIR144]
View all literature mentionsThis polyclonal targets Glutathione Peroxidase 1
View all literature mentionsThis polyclonal targets VLCAD
View all literature mentionsThis unknown targets OXPHOS
View all literature mentionsThis monoclonal targets VDAC1
View all literature mentionsThis monoclonal targets OxPhos Complex IV subunit IV
View all literature mentionsMus musculus with name C57BL/6J from IMSR.
View all literature mentionsMus musculus with name B6SJLF1/J from IMSR.
View all literature mentionsMus musculus with name C57BL/6J from IMSR.
View all literature mentionsCell line Neuro-2a is a Cancer cell line with a species of origin Mus musculus
View all literature mentionsCell line HeLa is a Cancer cell line with a species of origin Homo sapiens
View all literature mentionsCell line C2C12 is a Spontaneously immortalized cell line with a species of origin Mus musculus (Mouse)
View all literature mentionsMus musculus with name B6SJLF1/J from IMSR.
View all literature mentionsThis monoclonal targets Ubiquinol-Cytochrome C Reductase Core Protein I antibody [16D10AD9AH5]
View all literature mentionsThis monoclonal targets FLAG
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis monoclonal targets PDHA1
View all literature mentionsThis polyclonal targets GAPDH
View all literature mentionsThis polyclonal targets LETM1
View all literature mentionsThis monoclonal targets NDUFA9
View all literature mentionsThis polyclonal targets Rabbit IgG (H+L)
View all literature mentionsThis polyclonal targets Mouse IgG (H+L)
View all literature mentionsThis monoclonal targets Glutathione Peroxidase 4 antibody [EPNCIR144]
View all literature mentionsThis polyclonal targets Glutathione Peroxidase 1
View all literature mentionsThis polyclonal targets VLCAD
View all literature mentionsThis unknown targets OXPHOS
View all literature mentionsThis monoclonal targets VDAC1
View all literature mentionsThis monoclonal targets OxPhos Complex IV subunit IV
View all literature mentions