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Showing OXA1L — OXA1 is a alias.

OXA1L

Mitochondrial inner membrane protein OXA1L · UniProt Q15070

Length
435 aa
Mass
48.5 kDa
Annotated
2026-06-10
82 papers in source corpus 25 papers cited in narrative 24 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 6/6 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

OXA1L is a conserved integral protein of the mitochondrial inner membrane that functions as an insertase/translocase mediating membrane insertion and assembly of respiratory chain complexes, a role established by the ability of human OXA1L to complement a respiratory-deficient yeast oxa1 null mutant (PMID:8196054, PMID:7991568). It is required for the correct assembly of cytochrome c oxidase and the F1Fo-ATP synthase (PMID:8612730), and in human cells its depletion reduces complex V and complex I (PMID:17936786) while biallelic patient variants produce combined deficiency of complexes I, IV, and V that is rescued by wild-type protein (PMID:30201738). The protein adopts a five-transmembrane topology with N terminus and loop L2 facing the intermembrane space and the C terminus and loops L1/L3 in the matrix, with a conserved GLPWWG hexapeptide near TM1 governing topogenesis (PMID:19349278); its reconstituted hydrophobic core forms a cation-selective, substrate-responsive membrane pore wide enough to translocate polypeptides (PMID:22829595). The matrix-exposed C-terminal tail directly and cooperatively binds the large subunit of the mitochondrial ribosome — in mammals contacting MRPL13, MRPL20, MRPL28, MRPL48, MRPL49, and MRPL51 (PMID:20601428) and in yeast docking at the polypeptide exit site near Mrp20/MrpL40 (PMID:14657017, PMID:14657018, PMID:19783770) — thereby tethering the translating ribosome to the insertase for co-translational insertion of mtDNA-encoded subunits (PMID:14657018, PMID:14657017); close ribosome proximity is further required to coordinate downstream assembly (PMID:22904327). OXA1L additionally inserts nuclear-encoded inner-membrane carrier proteins such as the ADP/ATP carrier (PMID:22846909) and cooperates with TMEM126A in quality-controlled insertion, with stalled cargo cleared by the iAAA protease (PMID:38199007, PMID:10816574). Biallelic OXA1L mutations cause a human combined OXPHOS deficiency disorder presenting as mitochondrial myopathy/encephalopathy (PMID:30201738), and OXA1L loss in muscle models drives OXPHOS impairment, elevated ROS, NF-κB activation, and apoptosis (PMID:40551575).

Mechanistic history

Synthesis pass · year-by-year structured walk · 14 steps
  1. 1994 High

    Established that OXA1 is required for cytochrome oxidase biogenesis and that this function is evolutionarily conserved to humans, defining OXA1L as the human ortholog.

    Evidence Yeast oxa1 null/disruption mutants with cytochrome spectroscopy and functional complementation by human OXA1L cDNA

    PMID:7991568 PMID:8196054

    Open questions at the time
    • Did not define whether the role was in insertion, translocation, or assembly
    • No biochemical mechanism for how the human protein substitutes
  2. 1996 High

    Broadened the substrate scope beyond complex IV by showing Oxa1 is also required for assembly of the F1Fo-ATP synthase.

    Evidence Yeast deletion mutant analyzed by cytochrome spectroscopy, ATPase activity, and blue native PAGE

    PMID:8612730

    Open questions at the time
    • Did not distinguish a direct insertase role from an indirect assembly defect
  3. 2002 Medium

    Defined the physical core of the translocase by showing Oxa1 forms a homo-oligomeric complex whose loss depletes complex I and IV subunits.

    Evidence Neurospora crassa depletion with native gel electrophoresis and mass estimation

    PMID:11823466

    Open questions at the time
    • Oligomeric state inferred from native mass estimation only
    • No structural validation of the homotetramer
  4. 2003 High

    Identified the mechanism coupling insertion to translation: the matrix C-terminal domain binds the large ribosomal subunit at the peptide exit tunnel to enable co-translational insertion.

    Evidence Ribosome-binding assays, crosslinking to Mrp20/L23 homolog, truncation analysis, and in organello translation in yeast

    PMID:14657017 PMID:14657018

    Open questions at the time
    • Did not resolve the structure of the ribosome-insertase interface
    • Substrate handoff from tunnel to membrane pore not visualized
  5. 2003 Medium

    Connected Oxa1 substrate dependence to a physical property by showing translocation requirement correlates with the net negative charge of the IMS-exposed domain.

    Evidence Yeast fusion-protein constructs in a mitochondrial genome deletion mutant with in organello translation and alkaline extraction

    PMID:14593095

    Open questions at the time
    • Charge-recognition mechanism at the pore not defined
    • Single-substrate-family scope
  6. 2005 Medium

    Placed Oxa1 membrane integration downstream of the TIM23 import pathway, separating its insertion step from matrix import.

    Evidence In vitro import assays, crosslinking to Tim23, and mtHsp70 mutant analysis

    PMID:16253275

    Open questions at the time
    • How mature Oxa1 transfers from Tim23 to the membrane not defined
    • In vitro reconstitution from one lab
  7. 2007 High

    Demonstrated the human ortholog's relevance by showing OXA1L knockdown preferentially impairs human ATP synthase and complex I, and revealed post-translational substrate engagement with Atp9.

    Evidence shRNA knockdown in HEK293 with BN-PAGE and activity assays; co-IP of newly synthesized Atp9 in yeast with deletion analysis

    PMID:17344477 PMID:17936786

    Open questions at the time
    • Human substrate spectrum incompletely mapped
    • Reason for differing complex sensitivities between human and yeast unclear
  8. 2009 High

    Provided structural and topological frameworks: Oxa1 forms ribosome-stabilized dimers above the exit tunnel, and mammalian OXA1L has a defined five-TM topology requiring a conserved GLPWWG motif.

    Evidence Cryo-EM of ribosome-nascent-chain complexes with cysteine crosslinking; systematic mutagenesis and protease-protection topology mapping in human cells; additional crosslinking to MrpL40

    PMID:19349278 PMID:19450532 PMID:19783770

    Open questions at the time
    • Pore-lining residues and gating not resolved structurally
    • No high-resolution structure of the mammalian protein
  9. 2010 High

    Quantified the mammalian OXA1L C-terminal-tail/ribosome interaction and revealed a distinct ribosomal interface (MRPL13/20/28/48/49/51) different from yeast.

    Evidence Isothermal titration calorimetry, crosslinking with mass spectrometry, and biophysical anisotropy/FCS analysis of the CTT

    PMID:20601428 PMID:20739282

    Open questions at the time
    • Functional consequence of distinct mammalian interface not tested by mutagenesis
    • Structure of the bound CTT not solved
  10. 2012 High

    Established the pore function biophysically and tied ribosome proximity, substrate availability, and submembrane localization to insertase activity and carrier import.

    Evidence Reconstituted electrophysiology of purified Oxa1; linker-insertion genetics; STED imaging of IBM/CM distribution; temperature-sensitive import assays for carrier proteins

    PMID:22513091 PMID:22829595 PMID:22846909 PMID:22904327

    Open questions at the time
    • Direct visualization of a polypeptide traversing the pore lacking
    • Mechanism of carrier-protein recognition by Oxa1 undefined
  11. 2018 High

    Established OXA1L as a human disease gene, linking biallelic variants to combined complex I/IV/V deficiency rescuable by wild-type protein.

    Evidence Whole exome sequencing, fibroblast complementation, co-IP of OXA1L with mtDNA-encoded subunits, BN-PAGE, and Drosophila RNAi

    PMID:30201738

    Open questions at the time
    • Genotype-phenotype correlation across patients limited
    • Tissue-specific vulnerability not explained
  12. 2023 Medium

    Refined the ribosome-binding region of the yeast CTD at residue resolution, identifying conserved contact stretches that undergo conformational transition on binding.

    Evidence Solution NMR of the yeast Oxa1 CTD bound to E. coli 70S ribosomes

    PMID:37834108

    Open questions at the time
    • Used heterologous bacterial ribosome rather than mitoribosome
    • Identified contact sites not validated by mutagenesis
  13. 2024 High

    Identified TMEM126A as a quality-control co-factor, integrating OXA1L insertion with iAAA-protease-mediated clearance of stalled cargo.

    Evidence Affinity purification, mitochondrial ribosome profiling, proteomics, and iAAA protease epistasis with TMEM126A knockdown

    PMID:38199007

    Open questions at the time
    • Structural basis of OXA1L-TMEM126A cooperation unknown
    • How blocked cargo is sensed and routed to the iAAA protease undefined
  14. 2025 Medium

    Modeled the disease mechanism in muscle, connecting OXA1L loss to OXPHOS failure, ROS, NF-κB signaling, and apoptosis, and confirmed rescue requires the intact protein.

    Evidence hiPSC-derived myotubes, CRISPR knockout muscle cells, conditional knockout mice, OXPHOS/ROS assays, and NF-κB pathway analysis

    PMID:40551575

    Open questions at the time
    • NF-κB placement is correlative rather than direct epistasis
    • Whether ROS-NF-κB axis is the primary cause of muscle pathology untested

Open questions

Synthesis pass · forward-looking unresolved questions
  • A high-resolution structure of the active mammalian OXA1L insertase engaged with the mitoribosome and a translocating substrate, and the molecular basis of substrate selection at the pore, remain unresolved.
  • No atomic structure of the mammalian OXA1L pore in action
  • Rules governing which nuclear- and mtDNA-encoded substrates require OXA1L not fully defined
  • Mechanistic link from OXA1L loss to tissue-specific human disease incompletely mapped

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0060090 molecular adaptor activity 3 GO:0140096 catalytic activity, acting on a protein 3 GO:0005215 transporter activity 2 GO:0140104 molecular carrier activity 2
Localization
GO:0005739 mitochondrion 2
Pathway
R-HSA-1852241 Organelle biogenesis and maintenance 3 R-HSA-392499 Metabolism of proteins 3 R-HSA-1430728 Metabolism 2
Partners
MRPL13MRPL20MRPL28MRPL48MRPL49MRPL51TIMM23TMEM126A
Complex memberships
OXA1/OXA insertase complexmitochondrial large ribosomal subunit (associated)

Evidence

Reading pass · 24 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1994 OXA1 (yeast) is required at a post-translational step in cytochrome oxidase biogenesis, likely at the level of assembly; null mutation causes complete respiratory deficiency and absence of cytochrome aa3. The human homolog (OXA1L) complements the yeast oxa1 mutant, restoring cytochrome oxidase assembly and oxygen consumption, indicating conserved function. Yeast genetics (oxa1 null mutant and disruption allele), cytochrome spectroscopy, functional complementation with human cDNA Journal of molecular biology / Proceedings of the National Academy of Sciences High 7991568 8196054
1996 Yeast Oxa1p is necessary for the correct assembly of both cytochrome c oxidase (complex IV) and the F1Fo-ATP synthase (complex V), as shown by absence of heme aa3 and dramatic decrease in oligomycin-sensitive ATPase activity in oxa1 deletion strains; analysis on non-denaturing gels confirmed failure to assemble both complexes. Yeast deletion mutant, cytochrome spectroscopy, enzymatic activity measurements, blue native PAGE FEBS letters High 8612730
2003 The matrix-exposed C-terminal region of yeast Oxa1 forms an alpha-helical domain that physically binds mitochondrial ribosomes (large subunit), and deletion of this domain strongly diminishes co-translational membrane insertion of the mitochondrially encoded Cox2 subunit. Biochemical ribosome-binding assay, deletion mutant analysis, in organello translation, mitochondria isolation The EMBO journal High 14657017 14657018
2003 Yeast Oxa1 physically associates with the large mitochondrial ribosomal subunit; the C-terminal region of Oxa1 supports this interaction. Oxa1 cross-links to Mrp20, a large ribosomal subunit protein homologous to bacterial L23 located adjacent to the peptide exit tunnel. Co-immunoprecipitation, chemical cross-linking, sucrose gradient sedimentation, truncation analysis The EMBO journal High 14657017 14657018
2002 Oxa1 from Neurospora crassa forms a homo-oligomeric complex of ~170–180 kDa (likely a homotetramer of 43-kDa monomers), representing the core of the OXA translocase; cells with severely reduced Oxa1 are deficient in complex I and complex IV subunits. Neurospora crassa genetic depletion, native gel electrophoresis, molecular weight estimation of isolated complex The Journal of biological chemistry Medium 11823466
2007 Human OXA1L localizes exclusively to mitochondria as an integral inner membrane protein of ~42 kDa, present in a 600–700 kDa complex. shRNA-mediated knockdown in HEK293 cells markedly decreases steady-state levels and activity of F1Fo-ATP synthase and moderately reduces NADH:ubiquinone oxidoreductase (complex I), but does not significantly affect cytochrome c oxidase or the bc1 complex. shRNA knockdown in HEK293 cells, blue native PAGE, enzymatic activity assays, mitochondrial fractionation, immunoblotting Journal of molecular biology High 17936786
2007 Yeast Oxa1 directly and physically interacts with newly synthesized mitochondrially encoded Atp9 protein in a post-translational manner (unlike the co-translational interaction with COX subunits), and this interaction does not require the C-terminal matrix-localized region of Oxa1. In the absence of Oxa1, Atp9 assembles into an intermediate complex with F1 subunits but fails to associate with Atp6 of the Fo sector. Co-immunoprecipitation, in organello radiolabeling, blue native PAGE, deletion mutant analysis Molecular biology of the cell High 17344477
2009 Cryo-EM structures of both E. coli YidC and S. cerevisiae Oxa1 bound to ribosome nascent chain complexes show that YidC and Oxa1 form dimers localized above the ribosomal tunnel exit, with the ribosome specifically stabilizing the dimeric state. Conserved transmembrane helices of Oxa1 localize to the dimer interface (by cysteine crosslinking), and both Oxa1 and YidC dimers contact the ribosome at ribosomal protein L23 and conserved rRNA helices 59 and 24. Cryo-electron microscopy, cysteine crosslinking Molecular cell High 19450532
2009 Chemical crosslinking maps the Saccharomyces cerevisiae Oxa1-ribosome interface and identifies MrpL40 (the yeast L24 homolog) as a ribosomal protein in close physical proximity to Oxa1 at the polypeptide exit site of the large ribosomal subunit. Chemical crosslinking, co-immunoprecipitation, mass spectrometry-based identification Eukaryotic cell Medium 19783770
2009 Mammalian OXA1L has a five-transmembrane topology in the mitochondrial inner membrane with N terminus and loop L2 exposed to the intermembrane space, and C-terminal region and loops L1/L3 exposed to the matrix. A conserved GLPWWG hexapeptide near the N terminus of TM1 is essential for correct topogenesis (export of N-terminal segment and L2 into the IMS). All five TMs cooperate for efficient membrane integration. Systematic deletion and mutation constructs in HeLa cells, protease protection assays, fluorescence microscopy, mitochondrial fractionation The Journal of biological chemistry High 19349278
2010 The C-terminal tail of human OXA1L (Oxa1L-CTT, ~100 aa) binds mammalian mitochondrial ribosomes with Kd of 0.3–0.8 μM and a stoichiometry of 2; this binding is enthalpy-driven. Oxa1L-CTT cross-links to mammalian mitochondrial ribosomal proteins MRPL13, MRPL20, MRPL28, MRPL48, MRPL49, and MRPL51, but does NOT cross-link to proteins decorating the canonical exit tunnel (L22, L23, L24, L29), indicating a distinct ribosome-binding interface compared to yeast Oxa1. Isothermal titration calorimetry, chemical crosslinking, mass spectrometry identification of crosslinked partners The Journal of biological chemistry High 20601428
2010 Oxa1L-CTT forms oligomers cooperatively (submicromolar Kd); a region near position 39 (within a long helix) mediates contacts during oligomer formation. Fluorescence correlation spectroscopy and anisotropy show that regions around positions 39, 67, and 94 of the CTT make extensive contacts with the ribosome, and the CTT undergoes conformational changes and induced oligomerization upon ribosome binding. Fluorescence anisotropy, fluorescence lifetime measurements, fluorescence correlation spectroscopy, cysteine-specific fluorescent labeling The Journal of biological chemistry Medium 20739282
2012 Purified and reconstituted yeast Oxa1 forms a cation-selective membrane pore that specifically responds to mitochondrial export signals, with a restriction zone diameter of 0.6–2 nm sufficient for polypeptide translocation. Single-channel analyses suggest four cooperative hydrophilic pores per active unit; pore properties are regulated by membrane potential and substrate. Protein purification, lipid bilayer reconstitution, electrophysiology (single-channel analysis) The Journal of biological chemistry High 22829595
2012 Insertion of flexible linkers (100 or 200 aa) between the Oxa1 insertase domain and ribosome-binding region causes length-dependent decrease in respiratory activity and decreased cytochrome c oxidase levels, specifically impairing assembly (rather than membrane insertion per se) of COX subunits. This implicates the close proximity of Oxa1 to ribosomes in coordinating assembly factor access during co-translational biogenesis. Yeast genetic engineering, mitochondria isolation, in organello translation, blue native PAGE, respiratory growth assays The Journal of biological chemistry Medium 22904327
2012 The inner-membrane distribution of Oxa1 between the inner boundary membrane (IBM) and cristae membrane (CM) is dynamic: under fermentable conditions Oxa1 is enriched in the IBM; under respiratory conditions it shifts to the CM. Enrichment in the CM requires both mitochondrial translation and the ribosome-binding domain of Oxa1. Overexpression of a nuclear-encoded Oxa1 substrate (Mdl1) shifts Oxa1 toward the IBM. Deletion of the ribosome receptor domain prevents CM enrichment. Stimulated emission depletion (STED) super-resolution microscopy, subcellular fractionation, yeast genetic mutants Molecular biology of the cell High 22513091
2012 Oxa1 is required for efficient import of mitochondrial carrier proteins (e.g., Aac2/ATP-ADP carrier) into the inner membrane; temperature-sensitive oxa1 mutant mitochondria show severely reduced import rates of Aac2 and other carrier family members, while matrix protein import is unaffected. This extends the Oxa1 substrate spectrum beyond bacterial-homolog mitochondrial proteins to eukaryote-specific carriers. Temperature-sensitive oxa1 mutant, in vitro mitochondrial import assay, enzymatic activity measurement, immunoblotting Journal of molecular biology Medium 22846909
2018 In human patients with biallelic OXA1L variants (c.500_507dup and c.620G>T), there is deficiency of oxidative phosphorylation complexes I, IV, and V. Expression of wild-type human OXA1L in patient fibroblasts rescued the complex IV and V defects. Immunoprecipitation of OXA1L revealed enrichment of mtDNA-encoded subunits of complexes I, IV, and V as interacting partners. OXA1L depletion in Drosophila recapitulated complex I, IV, and V defects. Whole exome sequencing, complementation in patient fibroblasts, co-immunoprecipitation of OXA1L with respiratory complex subunits, BN-PAGE, Drosophila RNAi model EMBO molecular medicine High 30201738
2024 TMEM126A is identified as an OXA1L-interacting protein; it associates with mitochondrial ribosomes and translation products. Loss of TMEM126A destabilizes mitochondrial translation products, triggering iAAA protease-dependent degradation at the inner membrane. Upon TMEM126A loss, OXA1L insertase complexes with blocked cargo undergo proteolytic clearance by the iAAA protease, revealing TMEM126A as a quality control co-factor that cooperates with OXA1L in co-translational protein insertion. Co-immunoprecipitation/affinity purification, mitochondrial ribosome profiling, proteomics, iAAA protease inhibitor/mutant analysis, TMEM126A knockdown Molecular cell High 38199007
2023 NMR characterization of yeast Oxa1 C-terminal domain (CTD) reveals it forms a transient α-helical structure within residues P342–Q385. Two conserved ribosome contact sites are identified: a very N-terminal site (V321–I327) and a central stretch (I348–Q370) that undergoes transient-to-stable conformational transition upon ribosome binding. NMR spectroscopy (solution NMR), binding to E. coli 70S ribosomes International journal of molecular sciences Medium 37834108
2025 OXA1L deficiency in patient-specific hiPSC-derived myotubes and OXA1L-knockout immortalized skeletal muscle cells causes combined mitochondrial respiratory chain defects and OXPHOS impairment. Rescue with wild-type OXA1L (but not truncated mutant) restores respiratory chain function. Skeletal muscle conditional Oxa1l-knockout mice exhibit OXPHOS deficiencies and muscle morphofunctional abnormalities. Mechanistically, OXPHOS impairment leads to elevated ROS, which activates NF-κB signaling, triggering apoptosis. hiPSC differentiation to myotubes, CRISPR/Cas9 knockout in IHSMC, conditional mouse knockout, OXPHOS activity assays, ROS measurement, NF-κB pathway analysis Clinical and translational medicine Medium 40551575
2005 Oxa1 biogenesis involves initial accumulation at Tim23 (the inner membrane protein translocase) immediately after mitochondrial import; presequence cleavage requires mtHsp70, but subsequent membrane insertion of mature Oxa1 is essentially mtHsp70-independent, indicating that membrane integration occurs downstream of Tim23 docking and is distinct from matrix import. In vitro mitochondrial import assay, crosslinking to Tim23, mtHsp70 mutant analysis, protease protection Journal of molecular biology Medium 16253275
2000 In yeast, Fo-ATPase subunits (Atp4p, Atp6p, Atp17p) are specifically degraded in the absence of Oxa1p by the iAAA protease Yme1p; deletion of YME1 in oxa1Δ cells stabilizes these subunits and restores oligomycin-sensitive ATPase activity, demonstrating that Yme1p-mediated proteolysis is responsible for F0 subunit degradation when Oxa1p is absent and that Oxa1p is not strictly essential for export of these ATPase subunits. Yeast double-mutant analysis, enzymatic assays, cytochrome spectroscopy, immunoblotting The Journal of biological chemistry Medium 10816574
2003 The dependence of intermembrane space domain translocation on Oxa1 correlates with the net charge of the translocated domain (negatively charged domains require Oxa1), not with its position within the protein sequence, as demonstrated using fusion proteins in a yeast mitochondrial genome deletion mutant. Yeast genetic analysis, fusion protein constructs, in organello translation, alkaline extraction The Journal of biological chemistry Medium 14593095
1997 The human OXA1L gene spans 5 kb, is composed of 10 exons and 9 introns, and encodes a protein with a 24-amino acid N-terminal mitochondrial presequence, confirming its mitochondrial targeting. Long PCR amplification, genomic sequencing, exon-intron structure determination Biochimica et biophysica acta Medium 9247084

Source papers

Stage 0 corpus · 82 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2001 Phylogenetic and structural analyses of the oxa1 family of protein translocases. FEMS microbiology letters 276 11731127
2003 Ribosome binding to the Oxa1 complex facilitates co-translational protein insertion in mitochondria. The EMBO journal 208 14657018
1987 Precise insertion of antibiotic resistance determinants into Tn21-like transposons: nucleotide sequence of the OXA-1 beta-lactamase gene. Proceedings of the National Academy of Sciences of the United States of America 203 2823258
1994 OXA1, a Saccharomyces cerevisiae nuclear gene whose sequence is conserved from prokaryotes to eukaryotes controls cytochrome oxidase biogenesis. Journal of molecular biology 184 8196054
2003 Yeast Oxa1 interacts with mitochondrial ribosomes: the importance of the C-terminal region of Oxa1. The EMBO journal 179 14657017
1996 The Saccharomyces cerevisiae OXA1 gene is required for the correct assembly of cytochrome c oxidase and oligomycin-sensitive ATP synthase. FEBS letters 123 8612730
2017 Identification of Oxa1 Homologs Operating in the Eukaryotic Endoplasmic Reticulum. Cell reports 114 29281821
1994 Emergence of clinical isolates of Escherichia coli producing TEM-1 derivatives or an OXA-1 beta-lactamase conferring resistance to beta-lactamase inhibitors. Antimicrobial agents and chemotherapy 114 8067742
2009 YidC and Oxa1 form dimeric insertion pores on the translating ribosome. Molecular cell 102 19450532
2003 The Alb3/Oxa1/YidC protein family: membrane-localized chaperones facilitating membrane protein insertion? Trends in cell biology 101 14507478
1994 Cloning of a human gene involved in cytochrome oxidase assembly by functional complementation of an oxa1- mutation in Saccharomyces cerevisiae. Proceedings of the National Academy of Sciences of the United States of America 94 7991568
2015 YidC/Alb3/Oxa1 Family of Insertases. The Journal of biological chemistry 93 25947384
2002 Insertion of proteins into the inner membrane of mitochondria: the role of the Oxa1 complex. Biochimica et biophysica acta 88 12191770
2010 Inserting membrane proteins: the YidC/Oxa1/Alb3 machinery in bacteria, mitochondria, and chloroplasts. Biochimica et biophysica acta 84 20800571
2007 Oxa1 directly interacts with Atp9 and mediates its assembly into the mitochondrial F1Fo-ATP synthase complex. Molecular biology of the cell 84 17344477
2007 Knockdown of human Oxa1l impairs the biogenesis of F1Fo-ATP synthase and NADH:ubiquinone oxidoreductase. Journal of molecular biology 79 17936786
2002 The Oxa1 protein forms a homooligomeric complex and is an essential part of the mitochondrial export translocase in Neurospora crassa. The Journal of biological chemistry 72 11823466
2008 Roles of Oxa1-related inner-membrane translocases in assembly of respiratory chain complexes. Biochimica et biophysica acta 70 18522806
2000 Absence of the mitochondrial AAA protease Yme1p restores F0-ATPase subunit accumulation in an oxa1 deletion mutant of Saccharomyces cerevisiae. The Journal of biological chemistry 68 10816574
2018 OXA1L mutations cause mitochondrial encephalopathy and a combined oxidative phosphorylation defect. EMBO molecular medicine 63 30201738
2003 Complementary impact of paralogous Oxa1-like proteins of Bacillus subtilis on post-translocational stages in protein secretion. The Journal of biological chemistry 62 12586834
2004 The Oxa2 protein of Neurospora crassa plays a critical role in the biogenesis of cytochrome oxidase and defines a ubiquitous subbranch of the Oxa1/YidC/Alb3 protein family. Molecular biology of the cell 61 14767059
2005 Oxa1/Alb3/YidC system for insertion of membrane proteins in mitochondria, chloroplasts and bacteria (review). Molecular membrane biology 53 16092528
2014 Detection of OXA-1 β-lactamase gene of Klebsiella pneumoniae from blood stream infections (BSI) by conventional PCR and in-silico analysis to understand the mechanism of OXA mediated resistance. PloS one 51 24647004
1992 Iron uptake by plants from microbial siderophores : a study with 7-nitrobenz-2 oxa-1,3-diazole-desferrioxamine as fluorescent ferrioxamine B analog. Plant physiology 51 16669040
2021 Structural and molecular mechanisms for membrane protein biogenesis by the Oxa1 superfamily. Nature structural & molecular biology 49 33664512
2011 Evolution of YidC/Oxa1/Alb3 insertases: three independent gene duplications followed by functional specialization in bacteria, mitochondria and chloroplasts. Biological chemistry 47 21194367
2006 The synechocystis sp PCC 6803 oxa1 homolog is essential for membrane integration of reaction center precursor protein pD1. The Plant cell 46 16905652
2009 Mutation of the active site carboxy-lysine (K70) of OXA-1 beta-lactamase results in a deacylation-deficient enzyme. Biochemistry 43 19485421
2009 Mapping of the Saccharomyces cerevisiae Oxa1-mitochondrial ribosome interface and identification of MrpL40, a ribosomal protein in close proximity to Oxa1 and critical for oxidative phosphorylation complex assembly. Eukaryotic cell 42 19783770
2010 Properties of the C-terminal tail of human mitochondrial inner membrane protein Oxa1L and its interactions with mammalian mitochondrial ribosomes. The Journal of biological chemistry 41 20601428
2012 The mitochondrial oxidase assembly protein1 (Oxa1) insertase forms a membrane pore in lipid bilayers. The Journal of biological chemistry 39 22829595
2011 False extended-spectrum {beta}-lactamase phenotype in clinical isolates of Escherichia coli associated with increased expression of OXA-1 or TEM-1 penicillinases and loss of porins. The Journal of antimicrobial chemotherapy 35 21742679
2004 A yeast mitochondrial membrane methyltransferase-like protein can compensate for oxa1 mutations. The Journal of biological chemistry 35 15355998
2009 Translocation and assembly of mitochondrially coded Saccharomyces cerevisiae cytochrome c oxidase subunit Cox2 by Oxa1 and Yme1 in the absence of Cox18. Genetics 33 19307606
2003 Protein export across the inner membrane of mitochondria: the nature of translocated domains determines the dependence on the Oxa1 translocase. The Journal of biological chemistry 33 14593095
2000 The respiratory gene OXA1 has two fission yeast orthologues which together encode a function essential for cellular viability. Molecular microbiology 31 10712694
2012 The membrane insertase Oxa1 is required for efficient import of carrier proteins into mitochondria. Journal of molecular biology 30 22846909
2012 Oxa1-ribosome complexes coordinate the assembly of cytochrome C oxidase in mitochondria. The Journal of biological chemistry 30 22904327
2010 Identification of protein-protein and protein-ribosome interacting regions of the C-terminal tail of human mitochondrial inner membrane protein Oxa1L. The Journal of biological chemistry 30 20739282
2012 The inner-mitochondrial distribution of Oxa1 depends on the growth conditions and on the availability of substrates. Molecular biology of the cell 29 22513091
2012 The YidC/Oxa1/Alb3 protein family: common principles and distinct features. Biological chemistry 29 23111630
2008 Inhibition of OXA-1 beta-lactamase by penems. Antimicrobial agents and chemotherapy 25 18559643
2004 Interaction between the oxa1 and rmp1 genes modulates respiratory complex assembly and life span in Podospora anserina. Genetics 25 15545650
2022 The ER membrane complex (EMC) can functionally replace the Oxa1 insertase in mitochondria. PLoS biology 24 35231030
2022 Detection of carbapenemases blaOXA48-blaKPC-blaNDM-blaVIM and extended-spectrum-β-lactamase blaOXA1-blaSHV-blaTEM genes in Gram-negative bacterial isolates from ICU burns patients. Annals of clinical microbiology and antimicrobials 24 35590320
2014 The different inhibition mechanisms of OXA-1 and OXA-24 β-lactamases are determined by the stability of active site carboxylated lysine. The Journal of biological chemistry 24 24443569
2010 Detection of unrelated Escherichia coli strains harboring genes of CTX-M-15, OXA-1, and AAC(6')-Ib-cr enzymes in a Tunisian hospital and characterization of their integrons and virulence factors. Journal of chemotherapy (Florence, Italy) 22 21123154
2024 Identification of TMEM126A as OXA1L-interacting protein reveals cotranslational quality control in mitochondria. Molecular cell 21 38199007
2005 Conserved mechanism of Oxa1 insertion into the mitochondrial inner membrane. Journal of molecular biology 20 16253275
1986 Analysis by using DNA probes of the OXA-1 beta-lactamase gene and its transposon. Antimicrobial agents and chemotherapy 20 3019237
2015 Reduced Susceptibility to Cefepime in Clinical Isolates of Enterobacteriaceae Producing OXA-1 Beta-Lactamase. Microbial drug resistance (Larchmont, N.Y.) 18 26295796
2012 Site-saturation mutagenesis of position V117 in OXA-1 β-lactamase: effect of side chain polarity on enzyme carboxylation and substrate turnover. Biochemistry 16 22429123
2009 Topogenesis of mammalian Oxa1, a component of the mitochondrial inner membrane protein export machinery. The Journal of biological chemistry 16 19349278
2018 Oxa1 Superfamily: New Members Found in the ER. Trends in biochemical sciences 14 29310909
2018 Draft genome sequence of an NDM-5, CTX-M-15 and OXA-1 co-producing Escherichia coli ST167 clinical strain isolated from a urine sample. Journal of global antimicrobial resistance 13 30103014
1985 Genesis of a complex transposon encoding the OXA-1 (type II) beta-lactamase gene. Antimicrobial agents and chemotherapy 13 3010839
2005 Mutation detection in four candidate genes (OXA1L, MRS2L, YME1L and MIPEP) for combined deficiencies in the oxidative phosphorylation system. Journal of inherited metabolic disease 12 16435202
2018 Polymorphisms in the DAD1 and OXA1L genes are associated with asthma and atopy in a South American population. Molecular immunology 11 30032071
2011 Characterization of a novel extended-spectrum β-lactamase phenotype from OXA-1 expression in Salmonella Typhimurium strains from Africa and Ireland. Diagnostic microbiology and infectious disease 9 21767713
2022 Evaluation of Piperacillin-Tazobactam ETEST for the Detection of OXA-1 Resistance Mechanism among Escherichia coli and Klebsiella pneumoniae. Journal of clinical microbiology 8 36416539
1997 Sequence and structure of the human OXA1L gene and its upstream elements. Biochimica et biophysica acta 8 9247084
2021 Genomic characterisation of Escherichia coli isolates co-producing NDM-5 and OXA-1 from hospitalised patients with invasive infections. Journal of global antimicrobial resistance 7 34965471
2020 ZapE/Afg1 interacts with Oxa1 and its depletion causes a multifaceted phenotype. PloS one 7 32579605
2020 β-lactamases (bla ) and class C β-lactamases gene frequency in Pseudomonas aeruginosa isolated from various clinical specimens in Khartoum State, Sudan: a cross sectional study. F1000Research 7 33363717
2009 Mammalian Oxa1 protein is useful for assessment of submitochondrial protein localization and mitochondrial membrane integrity. Analytical biochemistry 7 19854151
2005 The transcriptional activator HAP4 is a high copy suppressor of an oxa1 yeast mutation. Gene 7 15908145
2001 Crystallization and preliminary X-ray study of OXA-1, a class D beta-lactamase. Acta crystallographica. Section D, Biological crystallography 7 11717515
2013 Clonal spread of Klebsiella pneumoniae producing OXA-1 betalactamase in a Spanish hospital. International microbiology : the official journal of the Spanish Society for Microbiology 6 25102723
2025 OXA1L deficiency causes mitochondrial myopathy via reactive oxygen species regulated nuclear factor kappa B signalling pathway. Clinical and translational medicine 5 40551575
2013 The SFT-1 and OXA-1 respiratory chain complex assembly factors influence lifespan by distinct mechanisms in C. elegans. Longevity & healthspan 5 24472117
2009 A mutational analysis reveals new functional interactions between domains of the Oxa1 protein in Saccharomyces cerevisiae. Molecular microbiology 5 20025673
1984 Different mechanisms of TEM-1 and Oxa-1 mediated resistance to piperacillin in E. coli. Zentralblatt fur Bakteriologie, Mikrobiologie, und Hygiene. Series A, Medical microbiology, infectious diseases, virology, parasitology 5 6397946
2021 Purification of Mitochondrial Ribosomes with the Translocase Oxa1L from HEK Cells. Bio-protocol 3 34458404
2013 Partial suppression of Oxa1 mutants by mitochondria-targeted signal recognition particle provides insights into the evolution of the cotranslational insertion systems. The FEBS journal 3 23198851
2024 Palladium-Catalyzed [7 + 5] and Higher-Order Annulations of Oxa-1,n-dipoles: Synthesis of 12- to 15-Membered Lactones. Organic letters 2 39347609
2024 New variants of the DAD1 and OXA1L genes are associated with asthma and atopy in an adult population. Gene 2 39615807
2026 Correlation of OXA-1 and TEM-1 genes with antibiotic resistance to piperacillin/tazobactam in ESBL-producing Enterobacterales: insights from a multi-center analysis. Frontiers in cellular and infection microbiology 1 41602100
2025 Identification of an IncHI5-like plasmid co-harboring bla NDM-1 and bla OXA-1 in mcr-8.1-positive Klebsiella pneumoniae isolate. Frontiers in microbiology 1 40497048
1999 Expression of the divergent transcription unit containing the yeast PET122 and OXA1 genes. Biochemistry and molecular biology international 1 10410243
2023 A Correlation between 3'-UTR of OXA1 Gene and Yeast Mitochondrial Translation. Journal of fungi (Basel, Switzerland) 0 37108900
2023 NMR-Based Characterization of the Interaction between Yeast Oxa1-CTD and Ribosomes. International journal of molecular sciences 0 37834108

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