Affinage

SLC25A4

ADP/ATP translocase 1 · UniProt P12235

Length
298 aa
Mass
33.1 kDa
Annotated
2026-04-28
100 papers in source corpus 23 papers cited in narrative 23 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

SLC25A4 (ANT1) is the principal heart- and skeletal-muscle isoform of the mitochondrial ADP/ATP carrier that catalyzes strict 1:1 exchange of cytosolic ADP for matrix ATP across the inner mitochondrial membrane, with substrate specificity restricted to ADP and ATP (PMID:23173940, PMID:2167309). Beyond nucleotide exchange, ANT1 functions as a voltage-sensor of the mitochondrial permeability transition pore, mediates basal proton leak in mitochondria, and transports fatty acid anions via an R79-dependent sliding mechanism along the protein–lipid interface (PMID:27221760, PMID:16831128, PMID:37762012). Dominant missense mutations cause protein misfolding that disrupts inner-membrane respiratory chain complex assembly and destabilizes mtDNA, leading to autosomal dominant progressive external ophthalmoplegia (adPEO), mitochondrial myopathy, and cardiomyopathy (PMID:27693233, PMID:25833713, PMID:15016764). ANT1 also participates in apoptosis signaling by recruiting NF-κB into mitochondria and is post-translationally regulated by SHP2-mediated dephosphorylation and GSNOR-mediated denitrosylation at C160, both of which modulate mitochondrial membrane potential and downstream stress responses (PMID:15231833, PMID:29255148, PMID:37377022).

Mechanistic history

Synthesis pass · year-by-year structured walk · 19 steps
  1. 1990 High

    Establishing that the AAC1/ANT1 protein possesses intrinsic ADP/ATP exchange activity when reconstituted in proteoliposomes defined SLC25A4 as a bona fide nucleotide transporter, albeit with lower activity than the AAC2 isoform.

    Evidence Reconstitution of yeast AAC1 and AAC2 in proteoliposomes with quantitative transport assay

    PMID:2167309

    Open questions at the time
    • Human protein not yet reconstituted at this point
    • No disease-linked mutations tested
    • Structural basis of substrate selectivity unknown
  2. 1996 Medium

    Demonstrating that yeast AAC1 transcription is induced ~8-fold by oxygen in a heme-independent manner established a link between aerobic metabolism and carrier expression.

    Evidence AAC1-lacZ reporter and Northern blot under aerobic vs. anaerobic growth in S. cerevisiae

    PMID:8774724

    Open questions at the time
    • Transcription factor(s) mediating oxygen response not identified
    • Relevance to mammalian ANT1 regulation not tested
    • Single-lab finding
  3. 2004 High

    Introducing human adPEO-equivalent mutations into yeast AAC2 revealed that pathogenic variants impair ADP/ATP transport selectivity and dominantly destabilize mtDNA, linking carrier dysfunction to mitochondrial genome maintenance.

    Evidence Site-directed mutagenesis in yeast, reconstituted transport, and mtDNA stability assays

    PMID:15016764

    Open questions at the time
    • Whether misfolding or transport defect is the primary pathogenic driver was unresolved
    • Human tissue validation not yet performed
  4. 2004 High

    Showing that ANT1 overexpression induces apoptosis by recruiting NF-κB into mitochondria and activating the intrinsic caspase cascade established a pro-apoptotic role distinct from its transport function.

    Evidence Overexpression in cancer cells with cytochrome c release, caspase activity, NF-κB EMSA, and mitochondrial membrane potential assays

    PMID:15231833

    Open questions at the time
    • Whether this apoptotic role is physiological or an overexpression artifact
    • Direct binding partner mediating NF-κB recruitment not identified
    • In vivo relevance in non-cancer tissue unclear
  5. 2005 Medium

    Ant1 knockout mice revealed tissue-specific compensation: skeletal and cardiac muscle developed myopathy while extraocular muscles were spared due to higher Ant2 expression, clarifying isoform redundancy.

    Evidence Ant1 KO mouse histology, contractility, and ANT isoform mRNA quantification

    PMID:16303948

    Open questions at the time
    • Compensation mechanism inferred from mRNA levels without direct Ant2 rescue
    • Brain phenotype not characterized in this study
  6. 2006 High

    Pharmacological inhibition in UCP1-null brown-fat mitochondria demonstrated that ANT1 mediates a significant fraction of basal proton leak, establishing a second bioenergetic function beyond nucleotide exchange.

    Evidence Carboxyatractyloside sensitivity of respiration in isolated brown-fat mitochondria from UCP1−/− mice

    PMID:16831128

    Open questions at the time
    • Structural determinants of proton leak through ANT1 unknown
    • Quantitative contribution in heart/skeletal muscle not determined
  7. 2010 High

    Identifying MeCP2-YY1 cooperative repression of ANT1 transcription, with ANT1 upregulation in MeCP2-null mice and Rett patient cells, linked ANT1 regulation to neurodevelopmental disease.

    Evidence Co-IP of MeCP2-YY1, ChIP at ANT1 promoter, mRNA/protein analysis in Mecp2-null brain and Rett fibroblasts

    PMID:20504995

    Open questions at the time
    • Functional consequence of ANT1 upregulation in Rett neurons not directly tested
    • Whether ANT1 derepression contributes to Rett pathology is unclear
  8. 2010 High

    Epistasis experiments showed ANT1 acts downstream of PGC-1α to mediate ischemia-reperfusion injury in cardiac cells, positioning ANT1 as a key mediator of oxidative stress vulnerability.

    Evidence siRNA knockdown of ANT1 in PGC-1α-overexpressing H9c2 cardiomyocytes subjected to anoxia-reoxygenation

    PMID:20600099

    Open questions at the time
    • Direct transcriptional regulation by PGC-1α on ANT1 promoter not demonstrated by ChIP
    • In vivo cardiac-specific ANT1 manipulation not performed
  9. 2011 High

    Expressing human adPEO mutant ANT1 in differentiated myotubes established that mutations cause dominant transport impairment (not simple haploinsufficiency), distinguishing gain-of-function pathogenesis.

    Evidence Adenoviral expression in C2C12 myotubes with ADP-ATP exchange and membrane potential measurements vs. siRNA knockdown

    PMID:21586654

    Open questions at the time
    • Molecular mechanism of dominant-negative action not resolved
    • Whether misfolding contributes was not addressed in this system
  10. 2012 High

    Reconstitution of human AAC1 in two heterologous systems confirmed that the carrier has very narrow substrate specificity limited to ADP and ATP, excluding other nucleotides.

    Evidence Functional reconstitution in E. coli and L. lactis with radiolabeled competition assays and MD simulations

    PMID:23173940

    Open questions at the time
    • Structural basis of discrimination against GTP/GMP not experimentally confirmed beyond simulations
  11. 2015 High

    Demonstrating that adPEO mutations cause ANT1 misfolding and disrupt assembly of multiple inner membrane complexes revealed proteostatic toxicity as a distinct pathogenic mechanism beyond transport impairment.

    Evidence Yeast Aac2 mutagenesis with blue native PAGE for complex integrity and aggregate visualization

    PMID:25833713

    Open questions at the time
    • Whether misfolding occurs in human patient tissue not directly shown
    • Chaperone or quality-control pathways involved not identified
  12. 2016 High

    Identifying ANT1 as the voltage-sensor of the mitochondrial permeability transition pore established a structural role in mPT regulation independent of its transport activity.

    Evidence mPT assays in ANT1-deficient patient fibroblasts and siRNA-treated C2C12 myotubes with mitochondrial volume, Ca2+ biosensor, and ADP-ATP exchange measurements

    PMID:27221760

    Open questions at the time
    • How ANT1 confers voltage sensitivity structurally is unresolved
    • Relationship to cyclophilin D in mPT opening not clarified
  13. 2016 High

    Functional reconstitution of de novo dominant SLC25A4 mutations (R80H, R235G) confirmed severe transport impairment and associated mtDNA depletion, expanding the clinical phenotype to neonatal-onset mitochondrial disease.

    Evidence Whole-exome sequencing, recombinant protein transport assay, patient muscle biopsy biochemistry

    PMID:27693233

    Open questions at the time
    • Mechanism linking transport impairment to mtDNA copy number loss not defined
  14. 2017 High

    Demonstrating that NF-κB directly binds the ANT1 promoter and represses transcription upon TNFα stimulation established a feedback loop: NF-κB suppresses ANT1, impairing ATP exchange and reducing mPTP opening while increasing ROS.

    Evidence ChIP and EMSA for NF-κB at ANT1 promoter, TNFα treatment in T98G and rat cortical neurons with ATP/ADP exchange and ROS measurements

    PMID:28317877

    Open questions at the time
    • Whether this NF-κB–ANT1 axis operates in cardiac tissue is unknown
    • Relative contribution of the two NF-κB binding elements not dissected
  15. 2017 High

    Identifying SHP2 as a phosphatase that translocates to mitochondria and dephosphorylates ANT1 to prevent membrane potential collapse linked ANT1 phosphorylation status to NLRP3 inflammasome regulation.

    Evidence Reciprocal co-IP, subcellular fractionation, SHP2 ablation/inhibition in macrophages with mitochondrial membrane potential and cytokine assays

    PMID:29255148

    Open questions at the time
    • Phosphorylation site(s) on ANT1 not mapped
    • Whether dephosphorylation affects transport activity directly is unknown
  16. 2018 High

    Brain-specific Ant1 heterozygous KO mice linked ANT1 mitochondrial dysfunction to serotonergic neurotransmission: dorsal raphe neuron hyperexcitability, enhanced serotonin turnover, and impulsivity-related behavior.

    Evidence Conditional brain Ant1 KO mice with electrophysiology, HPLC serotonin measurement, behavioral testing, and COX histochemistry

    PMID:29892051

    Open questions at the time
    • Whether the serotonergic effect is cell-autonomous in raphe neurons is unclear
    • Relevance to human psychiatric phenotypes not established
  17. 2023 High

    Identifying C160 as the S-nitrosylation site on ANT1 and GSNOR as its denitrosylase established a redox regulatory mechanism: nitrosylation impairs mitochondrial function, while denitrosylation restores membrane potential and promotes mitophagy in heart failure.

    Evidence Biotin-switch/LC-MS/MS for site identification, C160A mutagenesis rescue, AAV9-GSNOR overexpression and cardiac GSNOR KO mice

    PMID:37377022

    Open questions at the time
    • Whether C160 nitrosylation affects ADP/ATP transport directly is not measured
    • Interplay with SHP2-mediated phosphorylation regulation unknown
  18. 2023 High

    Reconstitution and mutagenesis defined an R79-dependent fatty acid anion sliding mechanism, establishing that ANT1 transports fatty acids through a protein–lipid interface pathway that shares a critical arginine with nucleotide binding.

    Evidence Planar lipid bilayer reconstitution, R79 mutagenesis, molecular dynamics simulations

    PMID:37762012

    Open questions at the time
    • Physiological relevance of FA transport by ANT1 vs. UCP1 in thermogenesis not quantified
    • Whether other ANT isoforms share the same FA transport mechanism
  19. 2023 High

    ANT1 loss in lung epithelial cells induced senescence and worsened lung fibrosis in vivo, extending ANT1's pathophysiological roles beyond muscle to lung aging and fibrotic disease.

    Evidence siRNA in lung cells, Ant1 global KO mice with bleomycin/asbestos fibrosis, senescence markers, NAD+/NADH ratio

    PMID:37487137

    Open questions at the time
    • Cell-type-specific conditional KO in lung not performed
    • Whether ANT2 compensation operates in lung tissue not tested

Open questions

Synthesis pass · forward-looking unresolved questions
  • Key unresolved questions include: the high-resolution structural basis for ANT1's voltage-sensing in the mPT pore, the identity of the ANT1 phosphorylation site(s) regulated by SHP2, and whether ANT1 misfolding and transport deficiency represent separable or synergistic mechanisms in human adPEO pathogenesis.
  • No high-resolution cryo-EM structure of human ANT1 in mPT pore context
  • SHP2-targeted phosphosite on ANT1 unmapped
  • Relative contribution of misfolding vs. transport loss to disease not dissected in patient tissue

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0005215 transporter activity 7 GO:0140104 molecular carrier activity 1
Localization
GO:0005739 mitochondrion 6
Pathway
R-HSA-382551 Transport of small molecules 6 R-HSA-1430728 Metabolism 4 R-HSA-1643685 Disease 4 R-HSA-5357801 Programmed Cell Death 2

Evidence

Reading pass · 23 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2016 De novo dominant SLC25A4 mutations (p.Arg80His and p.Arg235Gly) produce recombinant AAC1 proteins severely impaired in ADP/ATP transport, causing marked loss of mitochondrial DNA copy number and combined respiratory chain deficiencies in skeletal muscle; loss of AAC1 protein and respiratory chain complexes containing mtDNA-encoded subunits was demonstrated. Whole-exome sequencing of patients, recombinant protein expression and transport assay, muscle biopsy immunoblot and histochemistry American journal of human genetics High 27693233
2012 Human AAC1 (SLC25A4) expressed in E. coli or Lactococcus lactis transports only ADP and ATP (not GMP, AMP, or other nucleotides), demonstrating a very narrow substrate specificity; molecular dynamics simulations showed the guanine base moiety has low probability of binding at the central cavity. Functional reconstitution in E. coli and L. lactis membranes, radiolabeled transport competition assays, molecular dynamics simulations Molecular membrane biology High 23173940
2004 adPEO-equivalent mutations introduced into yeast AAC2 (the orthologue of human ANT1) impair ADP versus ATP transport selectivity and dominant traits of reduced cytochrome content and increased mtDNA instability, establishing that the mutations affect substrate binding and transport mechanics of the carrier. Site-directed mutagenesis of yeast AAC2, growth assays on non-fermentable carbon sources, mitochondrial cytochrome measurements, reconstitution in proteoliposomes, mtDNA stability assays Human molecular genetics High 15016764
2011 Mutant human ANT1 (carrying adPEO mutations) expressed in differentiated mouse myotubes causes dominant mitochondrial defects: decreased ADP-ATP exchange function and abnormal translocator reversal potential, establishing that the mutations impair carrier function rather than causing simple loss of function. Adenoviral expression in differentiated C2C12 myotubes, ADP-ATP exchange measurements, mitochondrial membrane potential assays, ANT1 siRNA knockdown controls Human molecular genetics High 21586654
2018 Novel de novo dominant SLC25A4 variant (p.Lys33Gln) expressed in Lactococcus lactis shows significantly impaired ADP/ATP transport activity, causing mild childhood-onset mitochondrial myopathy with COX-deficient fibers and decreased complex I, III, and IV protein levels. Functional expression in L. lactis, radiolabeled transport assay, muscle biopsy immunohistochemistry and immunoblot Neurology. Genetics High 30046662
2006 ANT1 is responsible for a significant portion of the basal proton leak (high basal respiration) in brown-fat mitochondria, as demonstrated by carboxyatractyloside (CAtr) sensitivity, whereas ANT2 mediates fatty-acid-induced uncoupling; brown adipose tissue uniquely co-expresses Ant1 and Ant2 mRNA at equal levels while liver expresses only Ant2. Inhibitor studies with CAtr in isolated brown-fat and liver mitochondria from UCP1-/- and wild-type mice, respiration measurements, mRNA analysis The Biochemical journal High 16831128
2004 ANT1 overexpression in cancer cells induces apoptosis by disrupting mitochondrial membrane potential, releasing cytochrome c, and activating caspases-9 and -3; it also recruits the IκBα-NF-κB complex into mitochondria, decreasing nuclear NF-κB DNA-binding activity and downregulating anti-apoptotic genes (Bcl-XL, MnSOD2, c-IAP2). ANT1 overexpression in cultured cells, mitochondrial membrane potential assay, cytochrome c release, caspase activity assays, NF-κB reporter/EMSA, co-expression rescue experiments The Journal of biological chemistry High 15231833
2008 ANT1 overexpression induces apoptosis accompanied by NF-κB inactivation and increased Bax expression, and suppresses tumor growth in vivo; pro-apoptotic effects involve disruption of mitochondrial membrane potential and caspase-9/caspase-3 activation. Transfection of ANT1 into MDA-MB-231 cells, NF-κB activity assay, Bax expression, caspase assays, xenograft nude mouse model BMC cancer Medium 18522758
2010 ANT1 upregulated by PGC-1α mediates increased susceptibility to ischemia-reperfusion injury in cardiac cells; siRNA knockdown of ANT1 abolishes the detrimental effect of PGC-1α overexpression, preserving mitochondrial membrane potential under oxidative stress. Adenoviral PGC-1α overexpression in H9c2 cells, Affymetrix gene array, siRNA knockdown of ANT1, anoxia-reoxygenation cell death assay, mitochondrial membrane potential measurement Journal of molecular and cellular cardiology High 20600099
2016 ANT1 (SLC25A4) confers sensitivity of the mitochondrial permeability transition (mPT) pore to the proton electrochemical gradient (voltage-sensing); cells lacking ANT1 are resistant to calcimycin- and H2O2-induced mitochondrial swelling and show altered voltage-thresholds of mPT opening. mPT assays in human fibroblasts with partial or complete ANT1 loss and ANT1-knockdown C2C12 myotubes; mitochondrial volume ('thinness ratio' and cobalt-calcein), matrix Ca2+ biosensor, ADP-ATP exchange measurement Scientific reports High 27221760
2017 SHP2 translocates to mitochondria upon NLRP3 inflammasome stimulation, interacts with ANT1 by co-immunoprecipitation, and dephosphorylates ANT1, thereby preventing mitochondrial membrane potential collapse and the downstream release of mitochondrial DNA and ROS that would hyperactivate NLRP3. Co-immunoprecipitation, subcellular fractionation, SHP2 ablation/inhibition in macrophages, mitochondrial membrane potential assay, cytokine measurements Nature communications High 29255148
2023 Mitochondrial GSNOR denitrosylates ANT1 at cysteine 160 (C160); S-nitrosylation of ANT1-C160 impairs mitochondrial function and membrane potential, while overexpression of GSNOR or the non-nitrosylatable ANT1-C160A mutant restores mitochondrial function and upregulates mitophagy in heart failure. Cellular fractionation and immunofluorescence for GSNOR localization, biotin-switch and LC-MS/MS for S-nitrosylation site identification, mitochondria-targeting GSNOR overexpression via AAV9, cardiac-specific GSNOR KO mice, mitochondrial membrane potential and mitophagy assays Circulation research High 37377022
2017 NF-κB binds two NF-κB responsive elements in the ANT1 promoter (+1 to +20 bp and +41 to +61 bp) and represses ANT1 transcription; TNFα-activated NF-κB suppresses ANT1 mRNA and protein levels, impairing ATP/ADP exchange, decreasing ATP production, reducing calcium-induced mPTP opening, elevating mitochondrial potential, and increasing ROS production. ChIP/EMSA for NF-κB binding to ANT1 promoter, TNFα treatment of T98G cells and rat cortical neurons, ATP/ADP exchange assay, ROS measurement, mitochondrial membrane potential assay Scientific reports High 28317877
2010 MeCP2 interacts with YY1 in vitro and in vivo, and MeCP2-YY1 cooperates to repress ANT1 gene transcription; in MeCP2-null mice and Rett patient fibroblasts, ANT1 mRNA and protein are upregulated. Co-immunoprecipitation of MeCP2 and YY1, ChIP, reporter assays, RT-PCR and western blot in Mecp2-null mouse brain and Rett fibroblasts Human molecular genetics High 20504995
2008 In FSHD myoblasts, reduced D4Z4 repeat number is associated with a global change in 4q35 three-dimensional chromatin structure; 4qA/B directly interacts with the ANT1 gene promoter (by chromosome conformation capture), along with a newly identified transcriptional enhancer within 4qA, providing a mechanism for ANT1 derepression in FSHD. Chromosome conformation capture (3C) analysis, enhancer identification PloS one Medium 18852887
1990 In yeast, the AAC1 protein (ortholog of human ANT1/SLC25A4) reconstituted in proteoliposomes has ADP/ATP transport activity approximately 40% that of AAC2; deletion of AAC2 but not AAC1 causes major reduction in mitochondrial cytochrome content and respiration, indicating functional non-equivalence of the two isoforms. Reconstitution in proteoliposomes, nucleotide transport assay, mitochondrial cytochrome measurement, respiration measurement The Journal of biological chemistry High 2167309
2015 adPEO-equivalent mutations in yeast Aac2 (ANT1 orthologue) cause protein misfolding, disrupt assembly and stability of multiple mitochondrial inner membrane protein complexes, and impair cell growth; adPEO-type mutations form large aggregates whereas cardiomyopathy/myopathy-type mutations cause similar proteostatic damage without aggregation. Yeast Aac2 mutagenesis, blue native PAGE for complex assembly, protein stability assays, aggregate visualization Molecular biology of the cell High 25833713
2014 The Drosophila ANT isoform (sesB, ortholog of human ANT1/SLC25A4) mutant exhibits decreased respiratory control ratio and downregulation of cytochrome oxidase; mutant adults show ATP depletion, lactate accumulation, and metabolic shift toward glycolysis; female sterility is substantially rescued by somatic expression of alternative oxidase (AOX), while developmental delay was alleviated by altered mtDNA background. Bioenergetic measurements, ATP/lactate assays, transcriptomics, genetic rescue with AOX transgene and mtDNA background alteration Disease models & mechanisms High 24812436
2023 ANT1 transports fatty acid anions by a 'FA sliding' mechanism: FA anions are attracted by positively charged residues (arginines/lysines) on the matrix side of ANT1, slide along the protein-lipid interface to R79 where they are protonated; R79 is also critical for competitive binding of ADP/ATP substrates and inhibitors (carboxyatractyloside and bongkrekic acid). Planar lipid bilayer reconstitution of ANT1, site-directed mutagenesis of R79, molecular dynamics simulations, current measurements International journal of molecular sciences High 37762012
1996 AAC1 gene transcription in Saccharomyces cerevisiae (ortholog of human SLC25A4) is regulated by oxygen in a heme-independent manner: AAC1 expression is repressed ~8-fold under anaerobic conditions but is constitutive under all aerobic conditions tested, regardless of carbon source. AAC1-lacZ fusion reporter assay, mRNA Northern blot, anaerobic vs. aerobic growth conditions European journal of biochemistry Medium 8774724
2023 Loss of ANT1 (SLC25A4) in alveolar type II cells and lung epithelial cells causes mitochondrial dysfunction, increased senescence markers (β-galactosidase, p21), and reduced NAD+/NADH ratio; global Ant1 knockout mice develop worse lung fibrosis and increased senescence in bleomycin and asbestos models. siRNA knockdown of ANT1 in lung epithelial cells, senescence marker assays, NAD+/NADH measurement, alveolar organoid growth assay, global Ant1 KO mice with bleomycin/asbestos fibrosis models American journal of respiratory cell and molecular biology High 37487137
2018 Brain-specific heterozygous Ant1 conditional knockout mice show hyperexcitability of dorsal raphe neurons and enhanced serotonin turnover in nucleus accumbens, along with upregulation of Maob and accumulation of COX-negative cells in dorsal raphe; behavioral analysis showed diminished delay discounting (increased impulsivity-related behavior), linking ANT1 mitochondrial dysfunction to serotonergic neurotransmission. Conditional brain-specific Ant1 KO mice, IntelliCage behavioral assay, 5-choice serial reaction time test, neuronal electrophysiology, HPLC serotonin turnover, COX histochemistry Molecular psychiatry High 29892051
2005 Ant1 knockout mice develop mitochondrial myopathy of skeletal and heart muscle with increased mitochondrial size and OXPHOS staining, but extraocular muscles (EOMs) are spared from functional defects, likely because EOMs normally express higher levels of Ant2 mRNA that compensates for Ant1 loss. Ant1 KO mouse histology and COX/SDH staining, ocular motility testing, isolated EOM contractility, ANT isoform mRNA expression Investigative ophthalmology & visual science Medium 16303948

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2001 The 7SK small nuclear RNA inhibits the CDK9/cyclin T1 kinase to control transcription. Nature 565 11713532
2003 Truncated TrkB-T1 mediates neurotrophin-evoked calcium signalling in glia cells. Nature 299 14603320
2018 Native T1 and Extracellular Volume in Transthyretin Amyloidosis. JACC. Cardiovascular imaging 225 29550324
1997 Signal transduction mediated by the truncated trkB receptor isoforms, trkB.T1 and trkB.T2. The Journal of neuroscience : the official journal of the Society for Neuroscience 196 9092589
2000 CCR2 expression determines T1 versus T2 polarization during pulmonary Cryptococcus neoformans infection. Journal of immunology (Baltimore, Md. : 1950) 183 10657654
1998 p53 and RB expression predict progression in T1 bladder cancer. Clinical cancer research : an official journal of the American Association for Cancer Research 149 9563875
2019 Astrocyte morphogenesis is dependent on BDNF signaling via astrocytic TrkB.T1. eLife 145 31433295
1989 Chronically obstructed sinonasal secretions: observations on T1 and T2 shortening. Radiology 133 2748834
2017 Tyrosine phosphatase SHP2 negatively regulates NLRP3 inflammasome activation via ANT1-dependent mitochondrial homeostasis. Nature communications 127 29255148
2003 Mutations of ANT1, Twinkle, and POLG1 in sporadic progressive external ophthalmoplegia (PEO). Neurology 123 12707443
1998 Upregulation of cyclin T1/CDK9 complexes during T cell activation. Oncogene 114 9872325
2009 Endogenous truncated TrkB.T1 receptor regulates neuronal complexity and TrkB kinase receptor function in vivo. The Journal of neuroscience : the official journal of the Society for Neuroscience 113 19158294
2001 Truncated trkB.T1 is dominant negative inhibitor of trkB.TK+-mediated cell survival. Biochemical and biophysical research communications 113 11162678
2020 Identification of Differential Tumor Subtypes of T1 Bladder Cancer. European urology 100 32684305
2006 In vivo restoration of physiological levels of truncated TrkB.T1 receptor rescues neuronal cell death in a trisomic mouse model. Neuron 99 16815329
2001 ANT1, an aromatic and neutral amino acid transporter in Arabidopsis. Plant physiology 90 11299361
2000 The TRK-T1 fusion protein induces neoplastic transformation of thyroid epithelium. Oncogene 90 11126359
2016 Recurrent De Novo Dominant Mutations in SLC25A4 Cause Severe Early-Onset Mitochondrial Disease and Loss of Mitochondrial DNA Copy Number. American journal of human genetics 89 27693233
2017 Truncated TrkB.T1-Mediated Astrocyte Dysfunction Contributes to Impaired Motor Function and Neuropathic Pain after Spinal Cord Injury. The Journal of neuroscience : the official journal of the Society for Neuroscience 85 28270575
2014 Characterization of FLOWERING LOCUS T1 (FT1) gene in Brachypodium and wheat. PloS one 85 24718312
2001 Interaction between cyclin T1 and SCF(SKP2) targets CDK9 for ubiquitination and degradation by the proteasome. Molecular and cellular biology 84 11689688
1999 Cyclin T1 domains involved in complex formation with Tat and TAR RNA are critical for tat-activation. Journal of molecular biology 83 10329125
2004 Recruitment of NF-kappaB into mitochondria is involved in adenine nucleotide translocase 1 (ANT1)-induced apoptosis. The Journal of biological chemistry 81 15231833
2012 Imbalance of neurotrophin receptor isoforms TrkB-FL/TrkB-T1 induces neuronal death in excitotoxicity. Cell death & disease 80 22258407
2004 The genome and proteome of coliphage T1. Virology 78 14972552
1990 Structure-function studies of adenine nucleotide transport in mitochondria. II. Biochemical analysis of distinct AAC1 and AAC2 proteins in yeast. The Journal of biological chemistry 78 2167309
2015 BDNF modulates heart contraction force and long-term homeostasis through truncated TrkB.T1 receptor activation. The Journal of cell biology 77 26347138
2006 Carboxyatractyloside effects on brown-fat mitochondria imply that the adenine nucleotide translocator isoforms ANT1 and ANT2 may be responsible for basal and fatty-acid-induced uncoupling respectively. The Biochemical journal 77 16831128
2020 Function and Mechanisms of Truncated BDNF Receptor TrkB.T1 in Neuropathic Pain. Cells 75 32403409
2022 OCA-T1 and OCA-T2 are coactivators of POU2F3 in the tuft cell lineage. Nature 74 35576971
1997 Mapping AAC1, AAC2 and AACP, the genes for arylamine N-acetyltransferases, carcinogen metabolising enzymes on human chromosome 8p22, a region frequently deleted in tumours. Cytogenetics and cell genetics 72 9284941
2000 HIV-1 TAR RNA enhances the interaction between Tat and cyclin T1. The Journal of biological chemistry 71 10944537
2003 The growth factor granulin interacts with cyclin T1 and modulates P-TEFb-dependent transcription. Molecular and cellular biology 70 12588988
2004 Mutations in AAC2, equivalent to human adPEO-associated ANT1 mutations, lead to defective oxidative phosphorylation in Saccharomyces cerevisiae and affect mitochondrial DNA stability. Human molecular genetics 69 15016764
2009 Acetylation of cyclin T1 regulates the equilibrium between active and inactive P-TEFb in cells. The EMBO journal 61 19387490
2006 TrkB-T1 regulates the RhoA signaling and actin cytoskeleton in glioma cells. Biochemical and biophysical research communications 57 16500620
2008 Over-expression of adenine nucleotide translocase 1 (ANT1) induces apoptosis and tumor regression in vivo. BMC cancer 55 18522758
2005 A novel ANT1 gene mutation with probable germline mosaicism in autosomal dominant progressive external ophthalmoplegia. Neuromuscular disorders : NMD 55 15792871
1994 Expression of p53 in early (T1) gastric carcinoma and precancerous adjacent mucosa. Gut 55 7829004
2012 The substrate specificity of the human ADP/ATP carrier AAC1. Molecular membrane biology 51 23173940
2010 Neurotrophin receptors TrkB.T1 and p75NTR cooperate in modulating both functional and structural plasticity in mature hippocampal neurons. The European journal of neuroscience 51 20955473
2011 HIV-1 replication and latency are regulated by translational control of cyclin T1. Journal of molecular biology 50 21763496
2000 T1-deficient and T1-Fc-transgenic mice develop a normal protective Th2-type immune response following infection with Nippostrongylus brasiliensis. European journal of immunology 49 10940882
2013 Gadolinium complex and phosphorescent probe-modified NaDyF4 nanorods for T1- and T2-weighted MRI/CT/phosphorescence multimodality imaging. Biomaterials 47 24119502
2010 The MeCP2/YY1 interaction regulates ANT1 expression at 4q35: novel hints for Rett syndrome pathogenesis. Human molecular genetics 47 20504995
2023 Mitochondrial GSNOR Alleviates Cardiac Dysfunction via ANT1 Denitrosylation. Circulation research 45 37377022
2010 In glaucoma the upregulated truncated TrkC.T1 receptor isoform in glia causes increased TNF-alpha production, leading to retinal ganglion cell death. Investigative ophthalmology & visual science 45 20574020
1992 1/T1 rho and low-field 1/T1 of tissue water protons arise from magnetization transfer to macromolecular solid-state broadened lines. Magnetic resonance in medicine 45 1331697
2005 The adenine nucleotide translocase type 1 (ANT1): a new factor in mitochondrial disease. IUBMB life 41 16203679
2001 Radiotherapy for T1 glottic carcinoma: impact of anterior commissure involvement. The Journal of laryngology and otology 41 11276333
1976 The structure and function of ribonuclease T1. XXI. Modification of histidine residues in ribonuclease T1 with iodoacetamide. Journal of biochemistry 41 14120
2015 Isolation of candidate genes for the barley Ant1 and wheat Rc genes controlling anthocyanin pigmentation in different vegetative tissues. Molecular genetics and genomics : MGG 39 25585663
2004 High level expression of thermostable lipase from Geobacillus sp. strain T1. Bioscience, biotechnology, and biochemistry 39 14745170
1995 The isolation of Ant1, a transposable element from Aspergillus niger. Molecular & general genetics : MGG 37 8552048
2018 Ant1 mutant mice bridge the mitochondrial and serotonergic dysfunctions in bipolar disorder. Molecular psychiatry 35 29892051
2008 A functional role for 4qA/B in the structural rearrangement of the 4q35 region and in the regulation of FRG1 and ANT1 in facioscapulohumeral dystrophy. PloS one 35 18852887
1991 Studies on RNase T1 mutants affecting enzyme catalysis. European journal of biochemistry 35 1901790
2023 Generating active T1 transitions through mechanochemical feedback. eLife 34 37039463
1983 Crystallographic study of mechanism of ribonuclease T1-catalysed specific RNA hydrolysis. Journal of biomolecular structure & dynamics 34 6086061
2011 adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria. Human molecular genetics 32 21586654
2006 Magnetic resonance imaging with T1 dispersion contrast. Magnetic resonance in medicine 32 16673360
2019 Clinical management for T1 and T2 external auditory canal cancer. Auris, nasus, larynx 31 30799138
2010 Transient upregulation of PGC-1alpha diminishes cardiac ischemia tolerance via upregulation of ANT1. Journal of molecular and cellular cardiology 31 20600099
2010 Cyclin T1 overexpression induces malignant transformation and tumor growth. Cell cycle (Georgetown, Tex.) 31 20714219
2017 Transcription factor NF-kappa B represses ANT1 transcription and leads to mitochondrial dysfunctions. Scientific reports 30 28317877
2014 Phenotypic rescue of a Drosophila model of mitochondrial ANT1 disease. Disease models & mechanisms 29 24812436
2006 Cyclin D3 expression in primary Ta/T1 bladder cancer. The Journal of pathology 29 16482499
2016 Alterations in voltage-sensing of the mitochondrial permeability transition pore in ANT1-deficient cells. Scientific reports 28 27221760
2023 Loss of ANT1 Increases Fibrosis and Epithelial Cell Senescence in Idiopathic Pulmonary Fibrosis. American journal of respiratory cell and molecular biology 27 37487137
2021 Inhibiting BDNF/TrkB.T1 receptor improves resiniferatoxin-induced postherpetic neuralgia through decreasing ASIC3 signaling in dorsal root ganglia. Journal of neuroinflammation 27 33874962
2011 The magnetic field dependence of water T1 in tissues. Magnetic resonance in medicine 27 22144333
2015 Two Novel Mutations in the SLC25A4 Gene in a Patient with Mitochondrial Myopathy. JIMD reports 26 25732997
2009 Specificity of Hexim1 and Hexim2 complex formation with cyclin T1/T2, importin alpha and 7SK snRNA. Journal of molecular biology 26 19883659
2013 Renal cell recurrence for T1 tumors after laparoscopic partial nephrectomy. Journal of endourology 25 24074156
2004 Proton T1 and T2 relaxivities of serum proteins. Magnetic resonance imaging 24 15172062
2020 Maize ANT1 modulates vascular development, chloroplast development, photosynthesis, and plant growth. Proceedings of the National Academy of Sciences of the United States of America 23 32817425
2007 The expression of Troponin T1 gene is induced by ketamine in adult mouse brain. Brain research 23 17850769
2000 TTIP is a novel protein that interacts with the truncated T1 TrkB neurotrophin receptor. Biochemical and biophysical research communications 23 11162451
1997 NTRS, a new family of highly repetitive DNAs specific for the T1 chromosome of tobacco. Chromosoma 23 9362545
2013 Associations between fibrocytes and postcontrast myocardial T1 times in hypertrophic cardiomyopathy. Journal of the American Heart Association 22 24125844
2010 Retinal function and structure in Ant1-deficient mice. Investigative ophthalmology & visual science 22 20671283
2008 Clinical significance of osteopontin expression in T1 and T2 tongue cancers. Head & neck 22 18228527
2006 POLG1, C10ORF2, and ANT1 mutations are uncommon in sporadic progressive external ophthalmoplegia with multiple mitochondrial DNA deletions. Neurology 22 16682683
1996 Characterization of the VIP receptor from SUP T1 lymphoblasts. Advances in neuroimmunology 22 8790781
2015 Misfolding of mutant adenine nucleotide translocase in yeast supports a novel mechanism of Ant1-induced muscle diseases. Molecular biology of the cell 21 25833713
1996 Transcription of the AAC1 gene encoding an isoform of mitochondrial ADP/ATP carrier in Saccharomyces cerevisiae is regulated by oxygen in a heme-independent manner. European journal of biochemistry 21 8774724
2021 GLABRA2-based selection efficiently enriches Cas9-generated nonchimeric mutants in the T1 generation. Plant physiology 20 34608972
2020 Evidence for Expanding Invasive Mediastinal Staging for Peripheral T1 Lung Tumors. Chest 20 32599066
2018 Expanding the phenotype of de novo SLC25A4-linked mitochondrial disease to include mild myopathy. Neurology. Genetics 20 30046662
1996 Catalyzed and assisted protein folding of ribonuclease T1. Biological chemistry 20 8922275
1999 Management of N0 neck in T1-T2 unilateral supraglottic cancer. The Annals of otology, rhinology, and laryngology 19 10526856
2020 Evaluation of the Diagnostic and Predictive Value of Serum Levels of ANT1, ATG5, and Parkin in Multiple Sclerosis. Clinical neurology and neurosurgery 18 32890892
2012 A study of PKM2, PFK-1, and ANT1 expressions in cervical biopsy tissues in China. Medical oncology (Northwood, London, England) 18 22227854
2005 Eliminating the Ant1 isoform produces a mouse with CPEO pathology but normal ocular motility. Investigative ophthalmology & visual science 18 16303948
1993 Ploidy status and the response of T1 glottic carcinoma to radiotherapy. Clinical otolaryngology and allied sciences 18 8508549
2023 FA Sliding as the Mechanism for the ANT1-Mediated Fatty Acid Anion Transport in Lipid Bilayers. International journal of molecular sciences 17 37762012
2014 The interplay of T1- and T2-relaxation on T1-weighted MRI of hMSCs induced by Gd-DOTA-peptides. Biomaterials 17 24560458
1990 A mitochondrial protein fraction catalyzing transport of the K+ analog T1+. FEBS letters 17 1699808
1987 Identification, purification, and characterization of Escherichia coli virus T1 DNA methyltransferase. The Journal of biological chemistry 17 3312202
2017 Two new cases of mitochondrial myopathy with exercise intolerance, hyperlactatemia and cardiomyopathy, caused by recessive SLC25A4 mutations. Mitochondrion 16 28823815