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Showing ATP5F1A — ATP5A1 is a alias.

ATP5F1A

ATP synthase F(1) complex subunit alpha, mitochondrial · UniProt P25705

Length
553 aa
Mass
59.8 kDa
Annotated
2026-06-09
86 papers in source corpus 26 papers cited in narrative 26 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

ATP5F1A encodes the catalytic α-subunit of mitochondrial complex V (ATP synthase F1), where its interaction with the β-subunit forms the catalytic core required for complex V stability, assembly, and ATP synthesis (PMID:23599390, PMID:39540264). Heterozygous and recurrent de novo missense variants disrupt the α–β subunit interface adjacent to the active site, destabilizing complex V and causing complex V deficiency with a dominant-negative, uncoupled oxidative-phosphorylation phenotype underlying neonatal mitochondrial encephalopathy, developmental delay, and movement disorders (PMID:23599390, PMID:34483339, PMID:40672495). The α-subunit is a hub for post-translational regulation that tunes complex V activity: TNK2/ACK1-mediated phosphorylation at Tyr243/Tyr246 stabilizes the enzyme and blocks binding of its inhibitor ATP5IF1 to sustain energy output (PMID:35895804); SIRT3-mediated deacetylation at K498 (and additional lysines) and H2S-dependent S-sulfhydration at Cys244 protect mitochondrial function, mitophagy, and resistance to ferroptosis and apoptosis (PMID:29486221, PMID:41655128); whereas CPT1A-driven K531 succinylation impedes assembly and impairs activity, and calpain-1 cleavage degrades the protein, impairing mitochondrial function and triggering NLRP3 inflammasome activation (PMID:35997820, PMID:41879856). Its abundance is further controlled by ubiquitin-dependent degradation, which is enhanced by C9ORF72-derived poly(GR) and by methamphetamine (at D376), and is counteracted by stabilizing partners such as HSPD1 (PMID:31086314, PMID:39430254, PMID:41132843). Loss of ATP5F1A compromises mitochondrial integrity in diverse cell types, impairing steroidogenesis, motor-neuron axon development, and autophagy flux, while its restoration rescues neurotoxicity and cardiac dysfunction in disease models (PMID:34894202, PMID:41053757, PMID:38910562). ATP5F1A additionally functions at the cell surface as a binding partner for extracellular ligands including LPS and bacterial adhesins (PMID:37526573, PMID:34098024).

Mechanistic history

Synthesis pass · year-by-year structured walk · 9 steps
  1. 2007 Medium

    Established that Atp5a1 is essential in vivo and can modify a tumor phenotype, the first evidence linking this subunit to organismal viability and disease beyond bioenergetics catalogues.

    Evidence Linkage analysis and congenic mouse intercross of an exon 3 duplication allele as the Mom2 modifier of ApcMin tumorigenesis

    PMID:17387143

    Open questions at the time
    • Molecular mechanism of tumor modification not resolved
    • No human disease link established at this stage
  2. 2013 High

    Defined the disease mechanism by which ATP5F1A mutations cause complex V deficiency: a disturbed α–β subunit interaction destabilizing assembly, with functional rescue confirming causality.

    Evidence Whole exome sequencing, complex V assembly immunoblotting, oxygen consumption, and complementation rescue in patient fibroblasts plus structural modelling

    PMID:23599390

    Open questions at the time
    • Single mutation; allelic spectrum unknown
    • Structural prediction not experimentally resolved
  3. 2018 Medium

    Revealed that the α-subunit is regulated by reversible post-translational modifications — S-sulfhydration at Cys244 and SIRT3-mediated deacetylation — that protect mitochondrial function and cell survival.

    Evidence Biotin-switch/maleimide MS for sulfhydration (CBS+/- mice, C244 mutant, H2S donor rescue) and Co-IP/MS for GRA8–SIRT3-driven deacetylation at K506/K531 in a sepsis model

    PMID:29486221 PMID:29869623

    Open questions at the time
    • How each PTM mechanistically alters catalysis or assembly not detailed
    • Crosstalk between modifications unaddressed
  4. 2019 High

    Connected ATP5F1A abundance to neurodegeneration, showing C9ORF72-derived poly(GR) drives its ubiquitination and degradation as a cause of mitochondrial dysfunction.

    Evidence Co-IP/binding assays in mouse neurons and patient brains, ubiquitination assay, and in vivo rescue by ectopic Atp5a1 expression

    PMID:31086314

    Open questions at the time
    • E3 ligase mediating poly(GR)-induced ubiquitination not identified
    • Ubiquitination site not mapped
  5. 2021 Medium

    Extended ATP5F1A function beyond the matrix, identifying a cell-surface/extracellular role as an LPS-binding protein and establishing requirement for mitochondrial integrity in steroidogenesis.

    Evidence Thermal shift and ELISA binding with intracerebroventricular rATP5A1 in neuroinflammation; siRNA knockdown in Leydig cells with steroidogenic enzyme readouts

    PMID:34098024 PMID:34483339 PMID:34894202

    Open questions at the time
    • Mechanism of surface localization unexplained
    • Overexpression/knockdown phenotypes may be indirect
  6. 2022 High

    Mapped activating phosphorylation and degradative cleavage as opposing controls of complex V output: TNK2/ACK1 phosphorylation at Tyr243/246 blocks ATP5IF1 inhibition, while calpain-1 cleavage degrades the subunit and triggers NLRP3 inflammasome activation.

    Evidence In vitro kinase assay, Y243/246A mutagenesis, Co-IP with ATP5IF1, TNK2 transgenic mice; calpain-1 inhibition/siRNA with NLRP3/pyroptosis readouts and calpastatin transgenic mice

    PMID:35895804 PMID:35997820

    Open questions at the time
    • Calpain-1 cleavage site not defined
    • Stoichiometry of phospho-ATP5F1A in vivo unknown
  7. 2024 Medium

    Identified protein and RNA partners that stabilize ATP5F1A — HSPD1 reducing K48 ubiquitination and a chimeric lncRNA enhancing TNK2 phosphorylation — coupling its abundance to growth signaling and cancer progression.

    Evidence Co-IP/MS and ubiquitination blots (HSPD1/osteosarcoma); RNA pulldown, Co-IP, and ATP measurement (SFT2D2-TBX19/prostate cancer)

    PMID:39430254 PMID:39540264

    Open questions at the time
    • Direct vs indirect stabilization not fully separated
    • Single-lab findings without reciprocal validation across systems
  8. 2025 High

    Consolidated the dominant-negative disease mechanism in humans and identified ATP5F1A as a druggable node, with cannabidiol preventing METH-induced ubiquitination at D376 to restore ATP synthase assembly.

    Evidence C. elegans dominant-negative modelling plus proteomics and mitochondrial physiology in patient cells (preprint); activity-based protein profiling, D376 mutagenesis, and in vivo VTA siRNA with behavioral readouts for CBD

    PMID:40672495 PMID:41132843

    Open questions at the time
    • Disease genetics from a preprint awaiting peer review
    • E3 ligase acting at D376 not identified
  9. 2026 High

    Resolved competing lysine modifications controlling assembly and stress survival: SIRT3 deacetylation at K498 enables protective mitophagy, while CPT1A-driven K531 succinylation impedes assembly, with site-mutant rescue protecting against cardiac ischemic injury.

    Evidence Site-specific acetylation analysis with SIRT3 KO/activator and mitophagy assays (ALI model); succinylation proteomics, K531R mutagenesis, AAV9 cardiac delivery, and activity assays (ischemia models)

    PMID:41655128 PMID:41879856 PMID:42172898

    Open questions at the time
    • Interplay between K498/K506/K531 modifications not integrated
    • Quantitative contribution of each PTM to assembly unresolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • How the diverse post-translational modifications, partner interactions, and reported extracellular functions are coordinately integrated to set ATP5F1A levels and complex V activity in a given tissue remains unresolved.
  • No unified model of competing PTMs and degradation pathways
  • Mechanism of cell-surface localization unexplained
  • E3 ligases for ubiquitin-dependent turnover unidentified

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140657 ATP-dependent activity 3 GO:0005198 structural molecule activity 2
Localization
GO:0005739 mitochondrion 4 GO:0005886 plasma membrane 2
Pathway
R-HSA-1643685 Disease 4 R-HSA-1430728 Metabolism 3 R-HSA-9612973 Autophagy 3
Partners
AHSA1ATP5F1BATP5IF1CAPN1CPT1AHSPD1SIRT3TNK2
Complex memberships
mitochondrial ATP synthase (complex V) F1

Evidence

Reading pass · 26 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2013 Heterozygous mutation in ATP5A1 (coding for complex V subunit α) causes a disturbed interaction of the α-subunit with the β-subunit of complex V, interfering with complex V stability and assembly, resulting in neonatal complex V deficiency. Complementation with wild-type ATP5A1 restored complex V in patient fibroblasts, confirming pathogenicity. Whole exome sequencing, immunoblotting for complex V assembly, oxygen consumption rate measurement, enzyme analysis in fibroblasts, complementation assay, 3D structural modelling Brain : a journal of neurology High 23599390
2019 Poly(GR) (encoded by C9ORF72 repeat expansion) binds preferentially to the mitochondrial complex V component ATP5A1, enhances its ubiquitination and degradation, leading to reduced ATP5A1 protein levels and mitochondrial dysfunction. Ectopic Atp5a1 expression in poly(GR)-expressing neurons rescued poly(GR)-induced neurotoxicity. Co-immunoprecipitation/binding assay in mouse neurons and patient brains, ubiquitination assay, rescue experiment with ectopic Atp5a1 expression in inducible mouse model Nature neuroscience High 31086314
2022 Mitochondrial calpain-1 cleaves ATP5A1 after CVB3 infection; downregulating ATP5A1 via siRNA impairs mitochondrial function, decreases cell viability, and induces NLRP3 inflammasome activation and pyroptosis, establishing ATP5A1 as the proteolytic target of calpain-1 in the pathway linking viral infection to NLRP3 inflammasome activation. Calpain-1 activation/inhibition experiments in vitro, ATP5A1 siRNA knockdown, NLRP3 inflammasome activation assays, transgenic calpastatin overexpression mice, mitochondria-targeted antioxidant rescue Basic research in cardiology High 35997820
2022 TNK2/ACK1 tyrosine kinase phosphorylates ATP5F1A at Tyr243 and Tyr246 (Tyr200 and Tyr203 in the mature protein), increasing complex V stability and mitochondrial energy output. Phospho-ATP5F1A prevents binding to its physiological inhibitor ATP5IF1, causing sustained mitochondrial activity. Y243/246A mutant ATP5F1A abolished these effects. In vitro kinase assay, phospho-specific antibody detection, co-immunoprecipitation with ATP5IF1, site-directed mutagenesis (Y243A/Y246A), TNK2 transgenic mice, mitophagy assays Autophagy High 35895804
2018 T. gondii GRA8 interacts with the nucleotide-binding domain of ATP5A1 in mitochondria; GRA8 also interacts with SIRT3, facilitating ATP5A1 deacetylation at K506 and K531, thereby promoting ATP production and anti-septic activity in vivo. Co-immunoprecipitation, mass spectrometry, site-directed deacetylation analysis (K506, K531), PKCα phosphorylation assay, in vivo sepsis model Experimental & molecular medicine Medium 29869623
2018 Endogenous H2S (produced by CBS) S-sulfhydrates ATP5A1 at cysteine 244. Reduced H2S in CBS+/- or LPS-treated mice decreases S-sulfhydrated ATP5A1 levels, leading to mitochondrial-mediated apoptosis and adrenal insufficiency. Overexpression of a C244 mutant ATP5A1 caused loss of LPS-induced apoptosis and restored ACTH responsiveness. Maleimide assay combined with mass spectrometry, modified biotin switch assay, CBS+/- mice, site-directed mutagenesis (C244), GYY4137 H2S donor rescue, ACTH response assay Molecular and cellular endocrinology High 29486221
2007 A 4-nucleotide duplication in exon 3 of Atp5a1 (encoding ATP synthase α-subunit) causes a recessive embryonic lethal phenotype in mice and acts as a modifier of ApcMin-induced intestinal tumorigenesis (Mom2 locus), with adenomas in carriers tending to progress to carcinomas in the absence of APC LOH. Linkage analysis, exome sequencing, expression analysis, tumor analysis, congenic mouse intercross Genome research Medium 17387143
2021 Atp5a1 knockdown in Leydig cells via siRNA decreased StAR, CYP11A1, and 17β-HSD expression by damaging mitochondrial structure, establishing Atp5a1 as required for mitochondrial integrity and steroidogenesis in response to chronic stress. 2-DE and MALDI-TOF-MS protein identification, Atp5a1 siRNA transfection in TM3 cells, mitochondrial structure analysis Journal of cellular and molecular medicine Medium 34894202
2021 Recurrent de novo ATP5F1A substitution c.620G>A [p.(Arg207His)] causes multiple deficits in complex V function and expression in patient-derived fibroblasts. Structural modelling predicts this creates an abnormal region of negative charge on ATP5F1A's β-subunit-interacting surface adjacent to the active site. Exome sequencing, functional assays in patient fibroblasts (complex V activity and expression), structural modelling European journal of human genetics : EJHG Medium 34483339
2021 ATP5A1 overexpression in HeLa cells significantly promotes cellular apoptosis (but not proliferation) and modulates expression of genes associated with innate immune response, angiogenesis, and MMP2/MMP19, as well as altering alternative splicing of hundreds of genes including those in HIF-1 signaling and glucose homeostasis pathways. Plasmid overexpression, RNA-seq transcriptomics, flow cytometry apoptosis assay, RT-qPCR validation Technology in cancer research & treatment Low 34520292
2021 Repeated aconitine treatment upregulates ATP5A1 via the AMPK-OPA1-ATP5A1 pathway, increasing mitochondrial fusion, biogenesis, and cellular ATP content in cardiomyocytes; AMPK inhibitor compound C reversed the increases in phospho-AMPK, OPA1, and ATP5A1. Pharmacological AMPK inhibition (compound C), Western blot, mitochondrial morphology analysis, ATP content measurement in NRVMs Frontiers in pharmacology Low 34177570
2023 ATP synthase subunit alpha (ATPase α / ATP5A1) serves as a cell-surface ligand for Salmonella Enteritidis Peg fimbriae adhesion protein PegD; siRNA knockdown of Atp5a1 in LMH cells significantly reduced S. Enteritidis adhesion. Ligand blotting, mass spectrometry, in vitro binding assay with purified PegD, siRNA knockdown with adhesion rate quantification Avian pathology : journal of the W.V.P.A Medium 37526573
2024 HSPD1 (HSP60) interacts with ATP5A1 (identified by Co-IP/MS) and reduces K48-linked ubiquitination and degradation of ATP5A1, thereby stabilizing it and activating AKT/mTOR signaling to promote osteosarcoma progression. Immunoprecipitation followed by mass spectrometry, Western blot for ubiquitination, in vitro and in vivo proliferation/apoptosis assays International journal of biological sciences Medium 39430254
2021 Extracellular recombinant ATP5A1 binds LPS directly (demonstrated by cellular thermal shift assay and ELISA-based binding assay), and intracerebroventricular injection of rATP5A1 suppresses LPS-induced neuroinflammation, microglial/astrocyte activation, IL-1β accumulation, and tau phosphorylation in mice. Cellular thermal shift assay, ELISA-based binding assay, intracerebroventricular injection in mouse model, behavioral assays Neuroscience letters Medium 34098024
2022 Exogenous Otx2 recombinant protein interacts with ATP5a1 in midbrain dopaminergic neurons and promotes ATP synthesis, protecting these neurons against MPP+-induced neurotoxicity. Co-immunoprecipitation, ATP synthesis assay, primary neuron culture with MPP+ treatment Neurotoxicology Low 35644505
2024 Chimeric SFT2D2-TBX19 (as lncRNA) interacts with ATP5F1A, increasing its TNK2/ACK1-mediated phosphorylation and stabilizing the interaction between ATP5F1A and ATP5F1B, thereby enhancing mitochondrial ATP synthase activity and ATP production in prostate cancer cells. RNA pulldown, Co-immunoprecipitation, phosphorylation assay, ATP production measurement, functional domain mapping Advanced science (Weinheim, Baden-Wurttemberg, Germany) Medium 39540264
2025 Cannabidiol (CBD) directly targets ATP5A1 and prevents METH-induced ubiquitination of ATP5A1 at residue D376, reversing METH-induced reduction of ATP5A1 protein and promoting ATP synthase assembly. ATP5A1 knockdown in the ventral tegmental area reversed CBD's therapeutic efficacy against METH addiction behaviors. Activity-based protein profiling (target fishing), ubiquitination assay, site-specific mutagenesis (D376), siRNA knockdown in VTA in vivo, behavioral assays (locomotor sensitization, CPP) Acta pharmaceutica Sinica. B High 41132843
2025 AHA1 (AHSA1) translocates to mitochondria under ischemia/reperfusion stress and directly interacts with ATP5A1, disrupting the cellular ATP/AMP ratio and increasing ROS production, leading to AMPK/mTOR/ULK1-mediated excessive mitophagy. AHA1 silencing suppressed pathological mitophagy and reduced infarct volume. siRNA knockdown, co-immunoprecipitation, MCAO/R mouse model and OGD/R cell model, AMPK/mTOR pathway analysis, behavioral tests Redox biology Medium 41297454
2026 SIRT3 deacetylates ATP5A1 at lysine 498; reduced SIRT3 in LPS-induced acute lung injury leads to acetylation of ATP5F1A/K498, impairing mitophagy and promoting endothelial ferroptosis. Downregulating ATP5F1A acetylation prevents SIRT3 inhibition from blocking SIGMAR1-mediated mitophagy and ferroptosis protection. Site-specific acetylation analysis (K498), SIRT3 knockout/activator, siRNA, PINK1/Parkin mitophagy assays, ALI mouse model Autophagy High 41655128
2026 Succinylation of ATP5A1 at K531 (by CPT1A as trans-succinylase, reversed by SIRT5 as desuccinylase) impedes ATP synthase assembly and impairs its activity. ATP5A1-K531R mutant (abolishing succinylation) delivered by AAV9 protected against ischemia-induced cardiomyocyte death and heart failure development. Succinylation proteomic analysis, site-directed mutagenesis (K531R), AAV9 cardiac-specific delivery, ATP synthase activity assay, OGD cardiomyocyte model, LAD ligation mouse model Basic research in cardiology High 41879856
2026 SIRT3 deacetylates ATP5A1, improving mitochondrial membrane potential, decreasing ROS, enhancing mitochondrial respiration and inhibiting cardiomyocyte apoptosis in ischemia-reperfusion injury. SIRT3 overexpression significantly reduced ATP5A1 acetylation; SIRT3 inhibition exacerbated mitochondrial damage. Co-immunoprecipitation, point mutation analysis, SIRT3 overexpression/inhibitor in MIRI mouse model and H/R HL-1 cells, mitochondrial membrane potential, ROS, respiration assays Biochemical and biophysical research communications Medium 42172898
2025 De novo heterozygous missense ATP5F1A variants cause dominant negative disruption of complex V, leading to markedly reduced complex V abundance and activity in proband-derived blood cells and fibroblasts, increased oxygen consumption yet decreased mitochondrial membrane potential and ATP levels (uncoupled oxidative phosphorylation), associated with developmental delay and movement disorders. Functional evaluation in C. elegans (dominant negative mechanism), biochemical assays in patient blood cells/fibroblasts, proteomics for complex V abundance, mitochondrial physiology in fibroblasts medRxiv : the preprint server for health sciencespreprint High 40672495
2024 BRG1 promotes ATP5A1 synthesis in liver cancer via TOMM40; BRG1 knockdown decreased ATP5A1 expression, impaired mitochondrial function (reduced MMP, increased mPTP opening), and promoted apoptosis in HCC cells. siRNA knockdown, plasmid overexpression, ICC/IF staining, Western blot, mitochondrial membrane potential and mPTP assays Cancer biology & therapy Low 38978225
2025 In zebrafish, atp5fa1 knockdown causes motor dysfunction, impaired motor neuron axon development, and growth retardation; rescue with human wild-type ATP5F1A mRNA partially restored motor neuron morphology. Knockdown increased P62 and decreased Lc3b-II, indicating inhibition of autophagy flux. Morpholino knockdown in zebrafish, mRNA rescue experiment, Western blot (P62, LC3B-II), motor neuron imaging in Tg(mnx1:eGFP) zebrafish, RNA-seq Journal of translational medicine Medium 41053757
2025 Dexamethasone inhibits osteogenic differentiation in BMSCs by modulating the CAST-CAPN1 axis to suppress ATP5A1 expression and reduce ATP activity; CAST overexpression partially mitigated these effects while CAPN1 overexpression exacerbated them, placing ATP5A1 downstream of calpain regulation. CAST/CAPN1 overexpression plasmids in rat BMSCs, Western blot, qRT-PCR, Alizarin Red S staining, ELISA for ATP activity Discovery medicine Low 40116104
2024 Overexpression of ATP5F1A in cardiomyocytes via cardiomyocyte-specific AAV9 improved heart function and morphology, and reduced fibrosis and cardiomyocyte size in transverse aortic constriction and dilated cardiomyopathy mouse heart failure models, identifying ATP5F1A as a mediator of cardiac reverse remodeling. Single-nucleus RNA sequencing of human CRR/non-CRR hearts, AAV9-mediated cardiomyocyte-specific overexpression in mouse HF models, echocardiography, pathological staining Circulation. Heart failure Medium 38910562

Source papers

Stage 0 corpus · 86 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2010 Orm family proteins mediate sphingolipid homeostasis. Nature 491 20182505
2019 C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo. Nature neuroscience 177 31086314
2022 Mitochondrial calpain-1 activates NLRP3 inflammasome by cleaving ATP5A1 and inducing mitochondrial ROS in CVB3-induced myocarditis. Basic research in cardiology 122 35997820
2012 Orm protein phosphoregulation mediates transient sphingolipid biosynthesis response to heat stress via the Pkh-Ypk and Cdc55-PP2A pathways. Molecular biology of the cell 118 22535525
2015 Aberrant ORM (yeast)-like protein isoform 3 (ORMDL3) expression dysregulates ceramide homeostasis in cells and ceramide exacerbates allergic asthma in mice. The Journal of allergy and clinical immunology 109 25842287
2019 The ORMDL/Orm-serine palmitoyltransferase (SPT) complex is directly regulated by ceramide: Reconstitution of SPT regulation in isolated membranes. The Journal of biological chemistry 92 30700557
2013 TORC1-regulated protein kinase Npr1 phosphorylates Orm to stimulate complex sphingolipid synthesis. Molecular biology of the cell 86 23363605
2013 A complex V ATP5A1 defect causes fatal neonatal mitochondrial encephalopathy. Brain : a journal of neurology 81 23599390
2018 Orm/ORMDL proteins: Gate guardians and master regulators. Advances in biological regulation 69 30193828
2013 Orm proteins integrate multiple signals to maintain sphingolipid homeostasis. The Journal of biological chemistry 62 23737533
2015 ATP5A1 and ATP5B are highly expressed in glioblastoma tumor cells and endothelial cells of microvascular proliferation. Journal of neuro-oncology 53 26526033
1987 Orosomucoid (ORM) typing by isoelectric focusing: evidence for gene duplication of ORM1 and genetic polymorphism of ORM2. Human genetics 50 3679210
1983 delta-Aminolevulinatedehydrase: synteny with ABO-AK1-ORM (and assignment to chromosome 9). Clinical genetics 45 6839527
2000 Male-biased mutation rates revealed from Z and W chromosome-linked ATP synthase alpha-subunit (ATP5A1) sequences in birds. Journal of molecular evolution 38 10824087
2022 TNK2/ACK1-mediated phosphorylation of ATP5F1A (ATP synthase F1 subunit alpha) selectively augments survival of prostate cancer while engendering mitochondrial vulnerability. Autophagy 37 35895804
2019 Role of acute-phase protein ORM in a mice model of ischemic stroke. Journal of cellular physiology 36 31026065
1987 Three new orosomucoid (ORM) variants revealed by isoelectric focusing and print immunofixation. Human genetics 35 3679213
2016 ORM Expression Alters Sphingolipid Homeostasis and Differentially Affects Ceramide Synthase Activity. Plant physiology 33 27506241
2007 The modifier of Min 2 (Mom2) locus: embryonic lethality of a mutation in the Atp5a1 gene suggests a novel mechanism of polyp suppression. Genome research 33 17387143
2013 Pharmacological characterisation of a structurally novel α2C-adrenoceptor antagonist ORM-10921 and its effects in neuropsychiatric models. Basic & clinical pharmacology & toxicology 32 23718812
2022 Fucoidan from Fucus vesiculosus prevents the loss of dopaminergic neurons by alleviating mitochondrial dysfunction through targeting ATP5F1a. Carbohydrate polymers 31 36657849
2016 ORM Promotes Skeletal Muscle Glycogen Accumulation via CCR5-Activated AMPK Pathway in Mice. Frontiers in pharmacology 26 27679573
2018 Endogenous H2S resists mitochondria-mediated apoptosis in the adrenal glands via ATP5A1 S-sulfhydration in male mice. Molecular and cellular endocrinology 24 29486221
2018 Toxoplasma gondii GRA8 induces ATP5A1-SIRT3-mediated mitochondrial metabolic resuscitation: a potential therapy for sepsis. Experimental & molecular medicine 24 29869623
1988 Orosomucoid (ORM) typing by isoelectric focusing: evidence for an additional duplicated ORM1 locus haplotype and close linkage of two ORM loci. American journal of human genetics 24 3400642
2018 Estrogen weakens muscle endurance via estrogen receptor-p38 MAPK-mediated orosomucoid (ORM) suppression. Experimental & molecular medicine 23 29869624
2009 Pro-angiogenic properties of orosomucoid (ORM). Experimental cell research 23 19651122
2014 A PET Tracer for Brain α2C Adrenoceptors, (11)C-ORM-13070: Radiosynthesis and Preclinical Evaluation in Rats and Knockout Mice. Journal of nuclear medicine : official publication, Society of Nuclear Medicine 22 24799619
2018 Reduced Levels of ATP Synthase Subunit ATP5F1A Correlate with Earlier-Onset Prostate Cancer. Oxidative medicine and cellular longevity 20 30538797
2014 Test-retest reliability of (11)C-ORM-13070 in PET imaging of α2C-adrenoceptors in vivo in the human brain. European journal of nuclear medicine and molecular imaging 19 25201008
2021 Chronic stress inhibits testosterone synthesis in Leydig cells through mitochondrial damage via Atp5a1. Journal of cellular and molecular medicine 18 34894202
2014 ¹¹C-ORM-13070, a novel PET ligand for brain α₂C-adrenoceptors: radiometabolism, plasma pharmacokinetics, whole-body distribution and radiation dosimetry in healthy men. European journal of nuclear medicine and molecular imaging 17 24838249
2014 Efficacy of selective NCX inhibition by ORM-10103 during simulated ischemia/reperfusion. European journal of pharmacology 17 24975099
1990 Orosomucoid (ORM) typing by isoelectric focusing: evidence for several new variants including ORM1 and ORM2 silent alleles. Vox sanguinis 16 2339522
2021 Repeated Aconitine Treatment Induced the Remodeling of Mitochondrial Function via AMPK-OPA1-ATP5A1 Pathway. Frontiers in pharmacology 15 34177570
2021 ATP5A1 Participates in Transcriptional and Posttranscriptional Regulation of Cancer-Associated Genes by Modulating Their Expression and Alternative Splicing Profiles in HeLa Cells. Technology in cancer research & treatment 15 34520292
2020 Blockade of sodium‑calcium exchanger via ORM-10962 attenuates cardiac alternans. Journal of molecular and cellular cardiology 15 33383036
2014 Amphetamine decreases α2C-adrenoceptor binding of [11C]ORM-13070: a PET study in the primate brain. The international journal of neuropsychopharmacology 15 25522417
2022 ORM 1 as a biomarker of increased vascular invasion and decreased sorafenib sensitivity in hepatocellular carcinoma. Bosnian journal of basic medical sciences 14 35765957
2015 Validation of [(11) C]ORM-13070 as a PET tracer for alpha2c -adrenoceptors in the human brain. Synapse (New York, N.Y.) 14 25530024
1992 Comparative mapping of mouse chromosome 4 and human chromosome 9: Lv, Orm, and Hxb are closely linked on mouse chromosome 4. Mammalian genome : official journal of the International Mammalian Genome Society 12 1379092
2018 Validation of an LC-MS/MS method for simultaneous quantitation of enzalutamide, N-desmethylenzalutamide, apalutamide, darolutamide and ORM-15341 in mice plasma and its application to a mice pharmacokinetic study. Journal of pharmaceutical and biomedical analysis 11 29709784
2024 HSPD1 Supports Osteosarcoma Progression through Stabilizing ATP5A1 and thus Activation of AKT/mTOR Signaling. International journal of biological sciences 10 39430254
2001 Loss of the gene for the alpha subunit of ATP synthase (ATP5A1) from the W chromosome in the African grey parrot (Psittacus erithacus). Journal of molecular evolution 10 11479684
2021 A recurrent de novo ATP5F1A substitution associated with neonatal complex V deficiency. European journal of human genetics : EJHG 9 34483339
2015 Detecting a dexmedetomidine-evoked reduction of noradrenaline release in the human brain with the alpha2C-adrenoceptor PET ligand [11C]ORM-13070. Synapse (New York, N.Y.) 9 26562363
1988 AIDS: no association with the genetic systems GC (D-binding protein), ORM (orosomucoid = alpha-1-acid glycoprotein), and A2HS (alpha-2-HS-glycoprotein). Infection 9 3162895
2024 Chimeric SFT2D2-TBX19 Promotes Prostate Cancer Progression by Encoding TBX19-202 Protein and Stabilizing Mitochondrial ATP Synthase through ATP5F1A Phosphorylation. Advanced science (Weinheim, Baden-Wurttemberg, Germany) 8 39540264
2021 Dissecting the regulatory roles of ORM proteins in the sphingolipid pathway of plants. PLoS computational biology 8 33507896
2017 A sandwich ELISA for porcine alpha-1 acid glycoprotein (pAGP, ORM-1) and further demonstration of its use to evaluate growth potential in newborn pigs. Domestic animal endocrinology 8 28551395
2024 BRG1 promotes liver cancer cell proliferation and metastasis by enhancing mitochondrial function and ATP5A1 synthesis through TOMM40. Cancer biology & therapy 7 38978225
2021 Interaction between extracellular ATP5A1 and LPS alleviates LPS-induced neuroinflammation in mice. Neuroscience letters 7 34098024
2013 Real-time monitoring of cell viability and cell density on the basis of a three dimensional optical reflectance method (3D-ORM): investigation of the effect of sub-lethal and lethal injuries. Journal of industrial microbiology & biotechnology 7 23604555
2024 The structure of the Orm2-containing serine palmitoyltransferase complex reveals distinct inhibitory potentials of yeast Orm proteins. Cell reports 6 39167489
2016 ORM-3819 promotes cardiac contractility through Ca(2+) sensitization in combination with selective PDE III inhibition, a novel approach to inotropy. European journal of pharmacology 6 26872993
1994 Serum protein polymorphism in Chuetas (Majorcan Jews)--GC, A2HS, ORM, ITI and HP. Gene geography : a computerized bulletin on human gene frequencies 6 7547602
2024 Integration of fungal transcriptomics and metabolomics provides insights into the early interaction between the ORM fungus Tulasnella sp. and the orchid Serapias vomeracea seeds. IMA fungus 5 39456087
2020 Application of the PET ligand [11C]ORM-13070 to examine receptor occupancy by the α2C-adrenoceptor antagonist ORM-12741: translational validation of target engagement in rat and human brain. EJNMMI research 5 33296042
2025 Cannabidiol alleviates methamphetamine addiction via targeting ATP5A1 and modulating the ATP-ADO-A1R signaling pathway. Acta pharmaceutica Sinica. B 4 41132843
2024 Overexpression of ATP5F1A in Cardiomyocytes Promotes Cardiac Reverse Remodeling. Circulation. Heart failure 4 38910562
2024 Orm proteins control ceramide synthesis and endocytosis via LCB-mediated Ypk1 regulation. Journal of lipid research 4 39490931
2022 Exogenous Otx2 protects midbrain dopaminergic neurons from MPP+ by interacting with ATP5a1 and promoting ATP synthesis. Neurotoxicology 4 35644505
2002 Identification and characterization of polymorphisms at the HAS alpha1-acid glycoprotein (ORM*) gene locus in Caucasians. Genetics and molecular research : GMR 4 14963818
1994 A linkage study of affective disorder with DNA markers for the ABO-AK1-ORM linkage group near the dopamine beta hydroxylase gene. Biological psychiatry 4 7811839
2025 Beyond molecular chaperoning: AHA1 reprograms autophagy flux through direct ATP5A1 interaction in ischemic neuronal injury. Redox biology 3 41297454
2024 Emodin Blocks mPTP Opening and Improves LPS-Induced HMEC-1 Cell Injury by Upregulation of ATP5A1. Chemistry & biodiversity 3 38511277
2022 High-intensity interval training along with spirulina algae consumption and caloric restriction ameliorated the Nrf1/Tfam/Mgmt and ATP5A1 pathway in the heart tissue of obese rats. Journal of food biochemistry 3 35037261
2025 ATP5F1A deficiency causes developmental delay and motor dysfunction in humans and zebrafish. Journal of translational medicine 2 41053757
2023 Synthesis and Antitumor Activity of Brominated-Ormeloxifene (Br-ORM) against Cervical Cancer. ACS omega 2 37901538
2022 Effects of methanol and formic acid on CRYB, ALDH2, and ATP5A1 of RGCs. Cutaneous and ocular toxicology 2 35696782
1991 Orosomucoid (ORM 1) subtyping and formal genetics. Human genetics 2 1879829
2025 Regulating Osteogenic Fate: How Dexamethasone Targets the CAST-CAPN1-ATP5A1 Axis in BMSCs. Discovery medicine 1 40116104
2024 Single-dose methamphetamine administration impairs ORM retrieval in mice via excessive DA-mediated inhibition of PrLGlu activity. Acta pharmacologica Sinica 1 38914676
2026 SIRT3-mediated mitophagy by deacetylating ATP5F1A involved in the protective effects of SIGMAR1/Sigma-1 receptor against ferroptosis and microvascular hyperpermeability in lipopolysaccharide-induced acute lung injury. Autophagy 0 41655128
2026 A New Case of a Neurodevelopmental Disorder and Myoclonic Dystonia Associated with the C.1404del Variant of the ATP5F1A Gene. International medical case reports journal 0 41743395
2026 ATP5A1 succinylation as a key driver for the transition of myocardial ischemia to development of heart failure. Basic research in cardiology 0 41879856
2026 Structural alterations in PPP1R13L, SAE1, ATP5A1 and PCK2 disrupt NF-κB signalling and mitochondrial metabolism in primary dermal fibroblasts in systemic sclerosis. Rheumatology (Oxford, England) 0 41926748
2026 Intersection of Sphingolipid and Sterol Metabolism at the Level of Orm Proteins in Yeast. Cells 0 42121915
2026 SIRT3-mediated deacetylation of ATP5A1 improves mitochondrial function to attenuate myocardial ischemia-reperfusion injury. Biochemical and biophysical research communications 0 42172898
2026 Expression and potential role of ATP5F1A in breast cancer through an integrated in silico and in vitro approach. In silico pharmacology 0 42211829
2025 Dominant negative ATP5F1A variants disrupt oxidative phosphorylation causing neurological disorders. medRxiv : the preprint server for health sciences 0 40672495
2024 Preclinical in vitro and in vivo evaluation of [11C]ORM-13070 as PET ligand for alpha-2C adrenergic receptor occupancy using PET imaging in non-human primates. Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism 0 39479946
2024 [Ginsenoside Rg_1 modulates ATP5A1 deacetylation via SIRT3 to attenuate hypoxia/reoxygenation injury in HL-1 cardiomyocytes]. Zhongguo Zhong yao za zhi = Zhongguo zhongyao zazhi = China journal of Chinese materia medica 0 39701765
2023 siRNA targeting Atp5a1 gene encoding ATPase α, the ligand of Peg fimbriae, reduced Salmonella Enteritidis adhesion. Avian pathology : journal of the W.V.P.A 0 37526573
2012 A genetic polymorphism in the sex-linked ATP5A1 gene is associated with individual fitness in Ovenbirds (Seiurus aurocapilla). Ecology and evolution 0 22833803
1981 [Plasma protein spectrum of dog blood during treatment of hypoxia with a Sever-OMR membrane oxygenator]. Biulleten' eksperimental'noi biologii i meditsiny 0 7248496

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