ArticleMolecular and cellular biology2012
Sam50 functions in mitochondrial intermembrane space bridging and biogenesis of respiratory complexes.
Article in Molecular and cellular biology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 125 papers.
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Who cites it
125 citing papers in PubMed, 241 citations in OpenAlex.
- Genome-wide scan revealed that polymorphisms in the PNPLA3, SAMM50, and PARVB genes are associated with development and progression of nonalcoholic fatty liver disease in Japan.Human genetics · 2013Trial
- Trypanosomal MICOS Is Assembled on Non-Respiring Mitochondrial Crista Precursors and Associates With Two Integral Microproteins.Molecular microbiology · 2026Article
- The molecular mechanisms of crista formation: how mitochondria give themselves breathing room.Biochemical Society transactions · 2026Review
- Mitochondrial quality control in health and disease: mechanisms and therapeutic targets.Signal transduction and targeted therapy · 2026Review
- Integrative structure determination of a human mitochondrial contact site and cristae organizing system (MICOS) sub-assembly.bioRxiv : the preprint server for biology · 2026Article
- MICOS and MIMAS, multifunctional assemblies linking mitochondrial biogenesis, architecture, and function.Protein science : a publication of the Protein Society · 2026Review
- Linking multipoint folding and stability with functional regulation in the mitochondrial transmembrane β-barrel Sam50.Nature communications · 2026Article
- IMMT downregulation promotes osteoarthritis development by inducing mitochondrial dysfunction.Journal of orthopaedic surgery and research · 2026Article
- Unconventional model organisms bend our view on mitochondrial cristae.Journal of cell science · 2026Review
- Nutrient State, Aging, and Diet Modulate SAM50-Dependent Mitochondrial Remodeling and Systemic Metabolic Signatures.Research square · 2026Article
- SARS-CoV-2 damages cardiomyocyte mitochondria and implicates long COVID-associated cardiovascular manifestations.Journal of advanced research · 2026Article
- CHCHD3(MIC19): mitochondrial cristae structure regulation and disease associations.Frontiers in molecular biosciences · 2026Review
- Mitochondria-Localized Nestin Protects Mesenchymal Stem Cells from Senescence by Maintaining Cristae Structure and Function.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Article
- A biobank-scale test of marginal epistasis reveals genome-wide signals of polygenic interaction effects.Nature genetics · 2025Article
- Article
- Ankyrin-B modulates mitochondrial fission in skeletal muscle and is required for optimal endurance exercise capacity.Nature communications · 2025Article
- Ancestral sequence reconstruction of the Mic60 Mitofilin domain reveals residues supporting respiration in yeast.Protein science : a publication of the Protein Society · 2025Article
- A data-driven model for mitochondrial inner membrane remodeling as a driving force of organelle shaping.Journal of cell science · 2025Article
- Structures and functions of the MICOS: Pathogenesis and therapeutic implications in Alzheimer's disease.Acta pharmaceutica Sinica. B · 2025Review
- Ancestral sequence reconstruction of the Mic60 Mitofilin domain reveals residues supporting respiration in yeast.bioRxiv : the preprint server for biology · 2025Article
65 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
11 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mitochondria possess an outer membrane (OMM) and an inner membrane (IMM), which folds into invaginations called cristae. Lipid composition, membrane potential, and proteins in the IMM influence organization of cristae. Here we show an essential role of the OMM protein Sam50 in the maintenance of the structure of cristae. Sam50 is a part of the sorting and assembly machinery (SAM) necessary for the assembly of β-barrel proteins in the OMM. We provide evidence that the SAM components exist in a large protein complex together with the IMM proteins mitofilin and CHCHD3, which we term the mitochondrial intermembrane space bridging (MIB) complex. Interactions between OMM and IMM components of the MIB complex are crucial for the preservation of cristae. After destabilization of the MIB complex, we observed deficiency in the assembly of respiratory chain complexes. Long-term depletion of Sam50 influences the amounts of proteins from all large respiratory complexes that contain mitochondrially encoded subunits, pointing to a connection between the structural integrity of cristae, assembly of respiratory complexes, and/or the maintenance of mitochondrial DNA (mtDNA).
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.