ArticleMolecular and cellular biology2012
The channel-forming Sym1 protein is transported by the TIM23 complex in a presequence-independent manner.
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 25 papers.
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Who cites it
25 citing papers in PubMed, 48 citations in OpenAlex.
- Article
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- Drug Drop Test: How to Quickly Identify Potential Therapeutic Compounds for Mitochondrial Diseases Using YeastInternational journal of molecular sciences · 2023Review
- Ovarian carcinoma immunoreactive antigen-like protein 2 (OCIAD2) is a novel complex III-specific assembly factor in mitochondria.Molecular biology of the cell · 2022Article
- The Mitochondrial Routing of the Kv1.3 Channel.Frontiers in oncology · 2022Article
- Review
- A Yeast-Based Repurposing Approach for the Treatment of Mitochondrial DNA Depletion Syndromes Led to the Identification of Molecules Able to Modulate the dNTP Pool.International journal of molecular sciences · 2021Article
- Opa1 Overexpression Protects from Early-Onset Mpv17Molecular therapy : the journal of the American Society of Gene Therapy · 2020Article
- Biogenesis of Mitochondrial Metabolite Carriers.Biomolecules · 2020Review
- Physiology and molecular biology of plants : an international journal of functional plant biology · 2020Article
- Hrd1 forms the retrotranslocation pore regulated by auto-ubiquitination and binding of misfolded proteins.Nature cell biology · 2020Article
- The mitochondrial carrier pathway transports non-canonical substrates with an odd number of transmembrane segments.BMC biology · 2020Article
- MPV17 does not control cancer cell proliferation.PloS one · 2020Article
- Plant peroxisomal solute transporter proteins.Journal of integrative plant biology · 2019Review
- Article
- Cation selectivity of the presequence translocase channel Tim23 is crucial for efficient protein import.eLife · 2017Article
- Revisiting trends on mitochondrial mega-channels for the import of proteins and nucleic acids.Journal of bioenergetics and biomembranes · 2017Review
- A monoclonal antibody raised against bacterially expressed MPV17 sequences shows peroxisomal, endosomal and lysosomal localisation in U2OS cells.BMC research notes · 2016Article
- MPV17 Loss Causes Deoxynucleotide Insufficiency and Slow DNA Replication in Mitochondria.PLoS genetics · 2016Article
- Increased Expression of Mitochondrial Inner-Membrane Protein Mpv17 After Intracerebral Hemorrhage in Adult Rats.Neurochemical research · 2015Article
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Authors and funding
12 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The majority of multispanning inner mitochondrial membrane proteins utilize internal targeting signals, which direct them to the carrier translocase (TIM22 complex), for their import. MPV17 and its Saccharomyces cerevisiae orthologue Sym1 are multispanning inner membrane proteins of unknown function with an amino-terminal presequence that suggests they may be targeted to the mitochondria. Mutations affecting MPV17 are associated with mitochondrial DNA depletion syndrome (MDDS). Reconstitution of purified Sym1 into planar lipid bilayers and electrophysiological measurements have demonstrated that Sym1 forms a membrane pore. To address the biogenesis of Sym1, which oligomerizes in the inner mitochondrial membrane, we studied its import and assembly pathway. Sym1 forms a transport intermediate at the translocase of the outer membrane (TOM) complex. Surprisingly, Sym1 was not transported into mitochondria by an amino-terminal signal, and in contrast to what has been observed in carrier proteins, Sym1 transport and assembly into the inner membrane were independent of small translocase of mitochondrial inner membrane (TIM) and TIM22 complexes. Instead, Sym1 required the presequence of translocase for its biogenesis. Our analyses have revealed a novel transport mechanism for a polytopic membrane protein in which internal signals direct the precursor into the inner membrane via the TIM23 complex, indicating a presequence-independent function of this translocase.
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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.