ArticleMolecular biology of the cell2010
Ribosome-binding proteins Mdm38 and Mba1 display overlapping functions for regulation of mitochondrial translation.
Article in Molecular biology of the cell, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers.
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Who cites it
30 citing papers in PubMed, 67 citations in OpenAlex.
- The mitochondrial proteome of diplonemids: from conventional pathways to eccentric RNA editing and transcript processing.BMC genomics · 2025Article
- Dissecting structure and function of the monovalent cation/HJournal of bacteriology · 2024Article
- Bi-allelic LETM1 variants perturb mitochondrial ion homeostasis leading to a clinical spectrum with predominant nervous system involvement.American journal of human genetics · 2022Article
- Yme2, a putative RNA recognition motif and AAA+ domain containing protein, genetically interacts with the mitochondrial protein export machinery.Biological chemistry · 2022Article
- Mitochondrial calcium exchange in physiology and disease.Physiological reviews · 2022Review
- Prime Real Estate: Metals, Cofactors and MICOS.Frontiers in cell and developmental biology · 2022Review
- ATAD3A has a scaffolding role regulating mitochondria inner membrane structure and protein assembly.Cell reports · 2021Article
- Chronic Activation of AMPK Induces Mitochondrial Biogenesis through Differential Phosphorylation and Abundance of Mitochondrial Proteins inInternational journal of molecular sciences · 2021Article
- Mechanism of membrane-tethered mitochondrial protein synthesis.Science (New York, N.Y.) · 2021Article
- Article
- Insertion Defects of Mitochondrially Encoded Proteins Burden the Mitochondrial Quality Control System.Cells · 2018Article
- Pathways to balance mitochondrial translation and protein import.Genes & development · 2018Review
- The ribosome receptors Mrx15 and Mba1 jointly organize cotranslational insertion and protein biogenesis in mitochondria.Molecular biology of the cell · 2018Article
- LETM1 couples mitochondrial DNA metabolism and nutrient preference.EMBO molecular medicine · 2018Article
- Yeast Mitoribosome Large Subunit Assembly Proceeds by Hierarchical Incorporation of Protein Clusters and Modules on the Inner Membrane.Cell metabolism · 2018Article
- Ribosome-Associated Mba1 Escorts Cox2 from Insertion Machinery to Maturing Assembly Intermediates.Molecular and cellular biology · 2016Article
- Oms1 associates with cytochrome c oxidase assembly intermediates to stabilize newly synthesized Cox1.Molecular biology of the cell · 2016Article
- Calcium Flux across Plant Mitochondrial Membranes: Possible Molecular Players.Frontiers in plant science · 2016Review
- Mam33 promotes cytochrome c oxidase subunit I translation in Saccharomyces cerevisiae mitochondria.Molecular biology of the cell · 2015Article
- Mitochondrial ribosome assembly in health and disease.Cell cycle (Georgetown, Tex.) · 2015Review
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Biogenesis of respiratory chain complexes depends on the expression of mitochondrial-encoded subunits. Their synthesis occurs on membrane-associated ribosomes and is probably coupled to their membrane insertion. Defects in expression of mitochondrial translation products are among the major causes of mitochondrial disorders. Mdm38 is related to Letm1, a protein affected in Wolf-Hirschhorn syndrome patients. Like Mba1 and Oxa1, Mdm38 is an inner membrane protein that interacts with ribosomes and is involved in respiratory chain biogenesis. We find that simultaneous loss of Mba1 and Mdm38 causes severe synthetic defects in the biogenesis of cytochrome reductase and cytochrome oxidase. These defects are not due to a compromised membrane binding of ribosomes but the consequence of a mis-regulation in the synthesis of Cox1 and cytochrome b. Cox1 expression is restored by replacing Cox1-specific regulatory regions in the mRNA. We conclude, that Mdm38 and Mba1 exhibit overlapping regulatory functions in translation of selected mitochondrial mRNAs.
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