Affinage

OXCT1

Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial · UniProt P55809

Length
520 aa
Mass
56.2 kDa
Annotated
2026-04-29
100 papers in source corpus 22 papers cited in narrative 22 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

OXCT1 encodes the mitochondrial succinyl-CoA:3-oxoacid CoA transferase (SCOT) that catalyzes the rate-limiting step of extrahepatic ketolysis by transferring CoA from succinyl-CoA to acetoacetate, and loss-of-function mutations cause hereditary SCOT deficiency with episodic ketoacidosis (PMID:8751852, PMID:10964512). Beyond its canonical ketolytic activity, OXCT1 functions as a lysine succinyltransferase—dependent on residue G424—that succinylates substrates including LACTB (inhibiting its protease activity to enhance mitochondrial respiration) and PGK1 (stabilizing the protein to promote glycolysis), linking OXCT1 to hepatocellular carcinoma and breast cancer progression (PMID:38176415, PMID:40634657). OXCT1 catalytic activity is itself regulated by SUCLA2-mediated succinylation at K421 downstream of IGF1/ERK2/PIN1 signaling, by frataxin-dependent suppression of proteasomal degradation, and by inhibitory peroxynitrite-mediated nitration at Y4 and Y76 (PMID:39862868, PMID:36016708, PMID:20527992). In macrophages, OXCT1-driven ketolysis generates succinate that increases H3K4me3 at the Arg1 promoter to promote protumor polarization, while in neurons OXCT1 overexpression confers neuroprotection via the SIRT3-SOD2 and Akt/GSK-3β/β-catenin pathways (PMID:38759889, PMID:36921750, PMID:38199308).

Mechanistic history

Synthesis pass · year-by-year structured walk · 13 steps
  1. 1996 High

    Establishing OXCT1 as the gene encoding SCOT and linking its loss to hereditary ketoacidosis answered the fundamental question of what enzyme catalyzes the rate-limiting step of ketolysis in extrahepatic tissues.

    Evidence cDNA cloning, chromosomal mapping to 5p13, and identification of homozygous S283X nonsense mutation in SCOT-deficient patient fibroblasts

    PMID:8751852

    Open questions at the time
    • enzymatic mechanism and active-site architecture not yet resolved
    • tissue-specific expression pattern not systematically characterized
  2. 1998 High

    Functional expression of patient-derived missense alleles in SCOT-deficient cells established the principle that individual residues (V133, C456) are essential for catalytic activity while others (T58) are neutral, enabling genotype-phenotype correlation.

    Evidence Transient expression of V133E, C456F, and T58M mutant cDNAs in immortalized SCOT-deficient fibroblasts with activity assay

    PMID:9671268

    Open questions at the time
    • no crystal structure to rationalize why specific residues are essential
    • incomplete allelic series
  3. 2000 High

    Determining the full genomic structure (17 exons, >100 kb) and using homology modeling to predict dimerization and active-site residues—validated by mutagenesis—provided the first structural framework for understanding SCOT function.

    Evidence Gene cloning, homology modeling against A. fermentans glutaconate CoA transferase, transient expression of G219E, V221M, G324E mutants

    PMID:10964512

    Open questions at the time
    • homology model not experimentally validated by crystallography
    • dimerization interface not confirmed by biophysical methods
  4. 2004 High

    Discovery that the T435N and later R268H mutations are temperature-sensitive explained the clinical observation that febrile illness triggers ketoacidotic crises in patients with residual SCOT activity, revealing protein stability as a disease-modifying factor.

    Evidence Transient expression at 30°C, 37°C, and 40°C with heat-treatment stability assays; 3D structural analysis of the R268-D52 salt bridge

    PMID:15496607 PMID:17706444

    Open questions at the time
    • no in vivo confirmation of temperature sensitivity in animal models
    • folding intermediates and chaperone dependencies not characterized
  5. 2010 High

    Identification of Y4 and Y76 as peroxynitrite-nitration sites that causally inhibit SCOT activity in diabetic heart mitochondria established a post-translational mechanism for OXCT1 inactivation in metabolic disease.

    Evidence Recombinant SCOT treated with peroxynitrite, LC-ESI-MS/MS site identification, Y4F/Y76F mutagenesis protecting activity

    PMID:20527992

    Open questions at the time
    • in vivo relevance of nitration in diabetic cardiomyopathy not directly tested
    • whether nitration is reversible or irreversible in cells is unknown
  6. 2010 High

    OXCT1 knockdown in beta-cells demonstrated that SCOT-dependent ketone body metabolism is required for normal glucose- and metabolite-stimulated insulin secretion, extending its role beyond simple fuel oxidation.

    Evidence shRNA knockdown in INS-1 832/13 insulinoma cells with insulin secretion assay

    PMID:20460097

    Open questions at the time
    • mechanism linking OXCT1 to insulin granule exocytosis not defined
    • not confirmed in primary islets or in vivo
  7. 2013 High

    The crystal structure of human SCOT enabled structure-based classification of all known pathogenic alleles, resolving whether mutations affect stability, dimerization, or catalysis.

    Evidence X-ray crystallography of human SCOT with mapping of disease mutations

    PMID:23420214

    Open questions at the time
    • no co-crystal with substrate or CoA intermediate
    • conformational dynamics during catalysis not captured
  8. 2022 High

    Discovery that frataxin physically interacts with OXCT1 and protects it from ubiquitin-proteasome degradation revealed a new layer of OXCT1 regulation and connected Friedreich's ataxia pathophysiology to impaired ketolysis.

    Evidence Reciprocal co-IP in vivo and in vitro, frataxin OE/KD across human fibroblasts and mouse models, proteasome inhibitor rescue, ketone body metabolite quantification

    PMID:36016708

    Open questions at the time
    • E3 ubiquitin ligase responsible for OXCT1 degradation not identified
    • structural basis of frataxin-OXCT1 interaction unknown
  9. 2023 Medium

    OXCT1 overexpression after traumatic brain injury showed that OXCT1-dependent ketone metabolism activates the SIRT3-SOD2 axis to reduce ROS and neuronal death, positioning OXCT1 as a neuroprotective factor.

    Evidence AAV-mediated OXCT1 overexpression in mouse TBI model, immunoblot for SIRT3/acetyl-SOD2, ROS quantification, cognitive behavioral tests

    PMID:36921750

    Open questions at the time
    • whether SIRT3 activation is a direct or indirect consequence of enhanced ketolysis is unclear
    • loss-of-function approach not performed in this system
  10. 2024 High

    The revelation that OXCT1 possesses intrinsic lysine succinyltransferase activity (dependent on G424) that succinylates LACTB to inhibit its protease function fundamentally expanded the enzyme's functional repertoire beyond ketolysis.

    Evidence In vitro succinyltransferase assay, G424 mutagenesis, LACTB K284 succinylation mapping by MS and Co-IP, mitochondrial respiration measurements

    PMID:38176415

    Open questions at the time
    • full substrate scope of OXCT1 succinyltransferase activity unknown
    • structural basis for dual catalytic activities not resolved
  11. 2024 High

    Mapping the IGF1→ERK2→SUCLA2(S124 phosphorylation)→PIN1→SUCLA2-OXCT1 interaction→K421 succinylation cascade showed how growth factor signaling activates OXCT1 ketolytic activity to fuel tumor growth.

    Evidence Co-IP of OXCT1-SUCLA2, phospho- and isomerization-deficient mutants, K421 mutagenesis, in vitro ketolysis assay, mouse tumor models

    PMID:39862868

    Open questions at the time
    • whether K421 succinylation also affects succinyltransferase activity is untested
    • desuccinylase(s) reversing K421 modification not identified
  12. 2024 High

    Macrophage-specific OXCT1 knockout demonstrated that OXCT1-driven succinate accumulation epigenetically activates Arg1 via H3K4me3, promoting protumor macrophage polarization and CD8+ T-cell exhaustion.

    Evidence LysMcre-OXCT1f/f conditional KO mice, ChIP-seq for H3K4me3 at Arg1 promoter, succinate metabolomics, CD8+ T-cell functional assays

    PMID:38759889

    Open questions at the time
    • which histone methyltransferase is recruited by succinate accumulation not identified
    • generalizability beyond liver cancer TAMs not established
  13. 2025 Medium

    Identification of PGK1 K146 as a second OXCT1 succinyltransferase substrate that stabilizes PGK1 protein and promotes glycolysis/PD-L1-mediated immune evasion broadened the oncogenic scope of OXCT1's non-canonical activity.

    Evidence Succinylation site mapping on PGK1, ubiquitination assay, KMT5A ChIP for H4K20me1 at OXCT1 promoter, T-cell killing assay in TNBC cells and PDOs

    PMID:40634657

    Open questions at the time
    • whether PGK1 succinylation occurs in non-cancer contexts is unknown
    • OXCT1 succinyltransferase activity has not been reconstituted with purified components for PGK1

Open questions

Synthesis pass · forward-looking unresolved questions
  • Key unresolved questions include the full substrate repertoire of OXCT1's succinyltransferase activity, the structural basis for its dual enzymatic functions, the identity of the E3 ligase mediating OXCT1 proteasomal turnover, and whether the non-canonical activities operate in normal physiology or are cancer-specific.
  • no structural model of succinyltransferase active site or dual-function mechanism
  • E3 ubiquitin ligase for OXCT1 degradation not identified
  • succinyltransferase substrate scope not systematically mapped

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016740 transferase activity 8 GO:0140096 catalytic activity, acting on a protein 2
Localization
GO:0005739 mitochondrion 4
Pathway
R-HSA-1430728 Metabolism 5 R-HSA-1643685 Disease 5 R-HSA-392499 Metabolism of proteins 3 R-HSA-168256 Immune System 1
Partners
ERK2FXNLACTBPGK1PIN1SUCLA2
Complex memberships
SCOT homodimer

Evidence

Reading pass · 22 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1996 Human OXCT1 (SCOT) encodes a succinyl-CoA:3-oxoacid CoA transferase that catalyzes the rate-determining step of ketolysis (esterification of acetoacetate to CoA) in extrahepatic tissues; a homozygous nonsense mutation (S283X) abolishes enzyme function and causes hereditary SCOT deficiency with episodic ketoacidosis. The gene was mapped to chromosome 5p13 by in situ hybridization. cDNA cloning, in situ hybridization, mutation analysis in patient fibroblasts American journal of human genetics High 8751852
2000 The human OXCT1 gene spans >100 kb with 17 exons on chromosome 5p13. Homology modeling based on Acidaminococcus fermentans glutaconate CoA transferase predicted that V221 and G219 lie on the dimerizing surface while G324 is near the active site; transient expression of missense mutant cDNAs in SCOT-deficient fibroblasts confirmed that G219E and G324E abolish activity while V221M retains ~10% activity, consistent with the structural model. Gene cloning, homology structural modeling, transient expression assay in patient fibroblasts Genomics High 10964512
1997 OXCT1 protein (SCOT) is detectable by immunoblot in control fibroblasts and lymphocytes but absent in fibroblasts and lymphocytes from SCOT-deficient patients, establishing immunoblot as a diagnostic tool and confirming complete loss of protein in null-mutation patients. Recombinant human SCOT expression in bacteria, antibody development, immunoblot analysis of patient and control cells Biochimica et biophysica acta Medium 9128180
1998 OXCT1 missense mutations V133E and C456F each abolish SCOT enzymatic activity when expressed in SCOT-deficient fibroblasts, whereas the co-occurring T58M substitution is functionally neutral, demonstrating allele-specific pathogenicity through transient expression. Transient expression of mutant cDNAs in immortalized SCOT-deficient fibroblasts, SCOT activity assay Human mutation High 9671268
2002 A testis-specific paralog of OXCT1, human Scot-t (OXCT2), is encoded by an intronless gene located within an intron of BMP8 on chromosome 1p34.1-35.3, and its protein localizes to the mitochondria-rich midpiece of ejaculated spermatozoa, suggesting a role in ketone body-based energy metabolism in sperm. cDNA cloning, PCR-based genomic structure analysis, subcellular localization by immunostaining of spermatozoa Molecular human reproduction Medium 11756565
2003 In mouse testis, the somatic SCOT isoform (scot-s/OXCT1) is expressed exclusively in Leydig and Sertoli cells, whereas the germline isoform (scot-t) is expressed in germ cells; SCOT enzymatic activity in sperm (from scot-t) is 2.5-fold higher than in Leydig cells, indicating cell-type-specific isoform utilization for ketone body metabolism. Differential RT-PCR, enzymatic activity assay in Leydig cell and sperm fractions International journal of andrology Medium 12534938
2004 The T435N mutation in OXCT1 retains significant residual SCOT activity (~20-50% depending on temperature) and is temperature-sensitive, explaining why patients homozygous for T435N develop ketoacidotic crises during febrile illness but lack permanent ketosis. Residual activity is more vulnerable to heat treatment than wild-type. Transient expression of mutant cDNA in SCOT-deficient fibroblasts, SCOT activity assay at multiple temperatures, heat-treatment stability assay Pediatric research High 15496607
2007 The R268H OXCT1 mutation produces a temperature-sensitive protein: residual SCOT activity is 59.7%, 34%, and 4% when expressed at 30°C, 37°C, and 40°C, respectively. Three-dimensional structural analysis indicates R268 forms a conserved salt bridge with D52; disruption of this bridge destabilizes the protein in a temperature-dependent manner. Transient expression at multiple temperatures, heat-treatment stability assay, 3D structural analysis Molecular genetics and metabolism High 17706444
2010 In diabetic db/db mouse heart mitochondria, SCOT (OXCT1) is nitrated at Tyr4 and Tyr76 by peroxynitrite. Site-directed mutagenesis of either tyrosine protects SCOT from peroxynitrite modification and prevents loss of enzymatic activity, establishing these residues as the priority nitration sites that causally inhibit SCOT catalysis. Recombinant SCOT incubation with peroxynitrite, LC-ESI-MS/MS identification of nitrated residues, site-directed mutagenesis, activity assay Journal of proteome research High 20527992
2010 OXCT1 (SCOT) knockdown by shRNA in INS-1 832/13 pancreatic beta-cells reduces glucose- or methyl succinate+β-hydroxybutyrate-stimulated insulin release by >70%, demonstrating that OXCT1-dependent mitochondrial acetoacetate export is required for normal insulin secretion. shRNA knockdown in insulinoma cell line, enzymatic activity confirmation, insulin secretion assay Archives of biochemistry and biophysics High 20460097
2011 Multiple OXCT1 missense mutations (L327P, R468C, A215V, S226N) exhibit residual activity or temperature-sensitive stability; structural prediction indicates main effects are destabilization of the SCOT dimer or disruption of catalytic activity. Expression at 30°C vs. 37°C rescues some mutant protein levels, confirming temperature-sensitive character. Transient expression of mutant cDNAs, immunoblot, SCOT activity assay at 30°C and 37°C Biochimica et biophysica acta High 21296660
2013 Crystal structure of human SCOT (OXCT1) was determined, providing molecular understanding of disease-causing mutations: mutations cluster in regions affecting protein stability or active-site geometry, enabling structure-based classification of all reported pathogenic OXCT1 alleles. X-ray crystallography of human SCOT protein Journal of inherited metabolic disease High 23420214
2013 A splice-site mutation (c.1248+5g>a) in OXCT1 causes simultaneous skipping of exons 12 and 13 through a 'splicing paralysis' mechanism: the mutation retains intron 13, which in turn prevents removal of introns 12 and 11; the entire intron11-exon12-intron12-exon13-mutant intron13 unit is then skipped, producing a frame-retaining mRNA that escapes nonsense-mediated decay. RT-PCR of heteronuclear RNA intermediates from patient vs. control fibroblasts, comparison of intron removal order Human mutation Medium 23281106
2022 Frataxin physically interacts with OXCT1 both in vivo and in vitro, and frataxin overexpression increases OXCT1 protein levels while frataxin deficiency decreases OXCT1 across multiple cell types. Frataxin suppresses ubiquitin-proteasome system (UPS)-dependent degradation of OXCT1, thereby regulating ketone body metabolism; frataxin-deficient cells fail to convert ketone bodies to acetyl-CoA. Co-immunoprecipitation in vivo and in vitro, frataxin overexpression/knockdown in human fibroblasts and knock-in/knockout mice, UPS inhibitor rescue, ketone body metabolite quantification PNAS nexus High 36016708
2024 OXCT1 functions as a lysine succinyltransferase in addition to its ketolytic role: residue G424 is essential for succinyltransferase activity. OXCT1 directly succinylates LACTB at K284, which inhibits LACTB proteolytic activity, leading to increased mitochondrial membrane potential and respiration and promoting HCC progression. In vitro succinyltransferase assay, site-directed mutagenesis (G424), identification of LACTB as substrate by MS and Co-IP, LACTB K284 succinylation functional assay, mitochondrial membrane potential and respiration measurements Molecular cell High 38176415
2024 In HCC cells, IGF1 stimulation triggers ERK2-mediated phosphorylation of SUCLA2 at S124, followed by PIN1-mediated cis-trans isomerization of SUCLA2, enabling SUCLA2 to interact with OXCT1. SUCLA2-associated OXCT1 is then succinylated at K421, which activates OXCT1 enzymatic activity, substantially enhancing ketolysis and tumor growth. Co-IP of OXCT1-SUCLA2 complex, phospho-mutant and isomerization-deficient constructs, K421 succinylation site mutagenesis, in vitro ketolysis assay, mouse tumor models, acetohydroxamic acid inhibitor treatment Molecular cell High 39862868
2024 OXCT1 deficiency in tumor-associated macrophages (TAMs) suppresses tumor growth by reducing succinate accumulation; high OXCT1-driven ketolysis generates succinate that increases H3K4me3 at the Arg1 promoter, promoting Arg1 transcription, TAM polarization to a protumor phenotype, and CD8+ T-cell exhaustion. LysMcre-OXCT1f/f conditional knockout mice, multiplex immunohistochemistry, ChIP-seq/H3K4me3 measurement at Arg1 promoter, succinate metabolite quantification, CD8+ T-cell functional assays Journal of hepatology High 38759889
2023 After traumatic brain injury (TBI), OXCT1 expression decreases in hippocampal neurons; adeno-associated virus-mediated OXCT1 overexpression increases SIRT3 expression and reduces acetylated SOD2, thereby decreasing reactive oxygen species production in injured hippocampal neurons, reducing neuronal death, and improving cognitive function. AAV-mediated OXCT1 overexpression in mice, TBI weight-drop model, immunoblot for SIRT3 and acetyl-SOD2, DHE staining for ROS, Nissl staining for neuronal death, cognitive behavioral tests Brain research Medium 36921750
2024 OXCT1 overexpression in hippocampal neurons after subarachnoid hemorrhage activates the Akt/GSK-3β/β-catenin signaling pathway to promote adult hippocampal neurogenesis; this effect is abolished by the PI3K inhibitor LY294002, placing OXCT1-dependent ketone metabolism upstream of Akt signaling in neurogenesis. AAV-mediated OXCT1 overexpression in SAH mouse model, LY294002 pharmacological inhibition, doublecortin/EdU dual staining for neurogenesis, immunofluorescence and immunoblot for Akt/GSK-3β/β-catenin, Morris water maze and Y-maze Brain research Medium 38199308
2025 OXCT1 succinylates PGK1 at K146, increasing PGK1 protein stability (reducing its ubiquitination without affecting mRNA), thereby enhancing aerobic glycolysis and PD-L1 expression to promote immune escape in triple-negative breast cancer. KMT5A methyltransferase increases H4K20me1 at the OXCT1 promoter to drive OXCT1 expression upstream. OXCT1 overexpression/knockdown in TNBC cells and patient-derived organoids, succinylation site mapping (PGK1 K146), ubiquitination assay, KMT5A ChIP for H4K20me1 at OXCT1 promoter, T-cell killing assay Communications biology Medium 40634657
2019 OXCT1 knockdown in ovine preadipocytes promotes lipid accumulation, while overexpression reduces it, demonstrating that OXCT1 negatively regulates adipogenesis/lipid deposition in adipocytes. OXCT1 siRNA knockdown and overexpression in ovine adipocytes, lipid accumulation assay Biochemical and biophysical research communications Medium 30928098
2021 OXCT1 overexpression in PDAC cells promotes gemcitabine resistance by activating the NF-κB signaling pathway; an NF-κB inhibitor reverses OXCT1-mediated gemcitabine resistance both in vitro and in mouse tumor models. OXCT1 overexpression/knockdown in PDAC cell lines, NF-κB inhibitor rescue, GSEA pathway analysis, mouse xenograft tumor models, apoptosis assay Frontiers in oncology Medium 34804914

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2016 Immunosuppressive Medications and Squamous Cell Skin Carcinoma: Nested Case-Control Study Within the Skin Cancer after Organ Transplant (SCOT) Cohort. American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 99 26824445
2022 SCOT: Single-Cell Multi-Omics Alignment with Optimal Transport. Journal of computational biology : a journal of computational molecular cell biology 75 35050714
2016 Diagnostic Strategies for the Evaluation of Chest Pain: Clinical Implications From SCOT-HEART and PROMISE. Journal of the American College of Cardiology 74 26892420
2024 OXCT1 functions as a succinyltransferase, contributing to hepatocellular carcinoma via succinylating LACTB. Molecular cell 65 38176415
2012 Role of multidetector computed tomography in the diagnosis and management of patients attending the rapid access chest pain clinic, The Scottish computed tomography of the heart (SCOT-HEART) trial: study protocol for randomized controlled trial. Trials 60 23036114
2017 The role of OXCT1 in the pathogenesis of cancer as a rate-limiting enzyme of ketone body metabolism. Life sciences 55 28684065
2013 Gastric antral vascular ectasia and its clinical correlates in patients with early diffuse systemic sclerosis in the SCOT trial. The Journal of rheumatology 54 23418384
1996 Succinyl CoA: 3-oxoacid CoA transferase (SCOT): human cDNA cloning, human chromosomal mapping to 5p13, and mutation detection in a SCOT-deficient patient. American journal of human genetics 51 8751852
2000 Succinyl-CoA:3-ketoacid CoA transferase (SCOT): cloning of the human SCOT gene, tertiary structural modeling of the human SCOT monomer, and characterization of three pathogenic mutations. Genomics 47 10964512
2024 Targeting OXCT1-mediated ketone metabolism reprograms macrophages to promote antitumor immunity via CD8+ T cells in hepatocellular carcinoma. Journal of hepatology 39 38759889
2013 Start Codon Targeted (SCoT) marker reveals genetic diversity of Dendrobium nobile Lindl., an endangered medicinal orchid species. Gene 37 23939470
2020 Circ-OXCT1 Suppresses Gastric Cancer EMT and Metastasis by Attenuating TGF-β Pathway Through the Circ-OXCT1/miR-136/SMAD4 Axis. OncoTargets and therapy 34 32523351
2018 Double-blind, randomized, multicenter phase 2 study of SC411 in children with sickle cell disease (SCOT trial). Blood advances 34 30097463
2023 Start codon targeted (SCoT) polymorphism marker in plant genome analysis: current status and prospects. Planta 30 36622439
2011 Clinical and molecular characterization of five patients with succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency. Biochimica et biophysica acta 28 21296660
1991 Partial or global rat brain ischemia: the SCOT model. Brain research bulletin 28 2049602
2020 Efficiency of RAPD, ISSR, iPBS, SCoT and phytochemical markers in the genetic relationship study of five native and economical important bamboos of North-East India. Phytochemistry 27 32146386
2004 Patients homozygous for the T435N mutation of succinyl-CoA:3-ketoacid CoA Transferase (SCOT) do not show permanent ketosis. Pediatric research 27 15496607
2022 Genetic and Morphological Diversity Assessment of Five Kalanchoe Genotypes by SCoT, ISSR and RAPD-PCR Markers. Plants (Basel, Switzerland) 26 35807674
2015 Genetic diversity analysis among male and female Jojoba genotypes employing gene targeted molecular markers, start codon targeted (SCoT) polymorphism and CAAT box-derived polymorphism (CBDP) markers. Meta gene 26 26110116
2015 Characterization of genetic diversity in chickpea using SSR markers, Start Codon Targeted Polymorphism (SCoT) and Conserved DNA-Derived Polymorphism (CDDP). Physiology and molecular biology of plants : an international journal of functional plant biology 26 26261401
2013 Comparative assessment of ISSR, DAMD and SCoT markers for evaluation of genetic diversity and conservation of landrace chickpea (Cicer arietinum L.) genotypes collected from north-west of Iran. Physiology and molecular biology of plants : an international journal of functional plant biology 26 24431526
2010 The nitrated proteome in heart mitochondria of the db/db mouse model: characterization of nitrated tyrosine residues in SCOT. Journal of proteome research 26 20527992
2015 Potential of Start Codon Targeted (SCoT) markers to estimate genetic diversity and relationships among Chinese Elymus sibiricus accessions. Molecules (Basel, Switzerland) 25 25853316
2014 Potential of Start Codon Targeted (SCoT) markers for DNA fingerprinting of newly synthesized tritordeums and their respective parents. Journal of applied genetics 25 24733248
2013 A structural mapping of mutations causing succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency. Journal of inherited metabolic disease 25 23420214
2022 Frataxin controls ketone body metabolism through regulation of OXCT1. PNAS nexus 24 36016708
2017 Assessment of genetic diversity in Vigna unguiculata L. (Walp) accessions using inter-simple sequence repeat (ISSR) and start codon targeted (SCoT) polymorphic markers. BMC genetics 24 29149837
2013 Molecular basis of two-exon skipping (exons 12 and 13) by c.1248+5g>a in OXCT1 gene: study on intermediates of OXCT1 transcripts in fibroblasts. Human mutation 24 23281106
2008 Type II thioesterase ScoT, associated with Streptomyces coelicolor A3(2) modular polyketide synthase Cpk, hydrolyzes acyl residues and has a preference for propionate. Applied and environmental microbiology 23 19074611
2002 Cloning and characterization of a human orthologue of testis-specific succinyl CoA: 3-oxo acid CoA transferase (Scot-t) cDNA. Molecular human reproduction 22 11756565
2010 Lower succinyl-CoA:3-ketoacid-CoA transferase (SCOT) and ATP citrate lyase in pancreatic islets of a rat model of type 2 diabetes: knockdown of SCOT inhibits insulin release in rat insulinoma cells. Archives of biochemistry and biophysics 21 20460097
2004 Succinyl-CoA:3-ketoacid transferase (SCOT) deficiency in a new patient homozygous for an R217X mutation. Journal of inherited metabolic disease 21 15669687
1997 Succinyl-CoA:3-ketoacid coenzyme A transferase (SCOT): development of an antibody to human SCOT and diagnostic use in hereditary SCOT deficiency. Biochimica et biophysica acta 21 9128180
2006 Single-base substitution at the last nucleotide of exon 6 (c.671G>A), resulting in the skipping of exon 6, and exons 6 and 7 in human succinyl-CoA:3-ketoacid CoA transferase (SCOT) gene. Molecular genetics and metabolism 20 17169596
2025 OXCT1 succinylation and activation by SUCLA2 promotes ketolysis and liver tumor growth. Molecular cell 19 39862868
2014 Genetic variability and structure of Quercus brantii assessed by ISSR, IRAP and SCoT markers. Gene 19 25241382
2004 Growth-inhibitory effects of the ketone body, monoacetoacetin, on human gastric cancer cells with succinyl-CoA: 3-oxoacid CoA-transferase (SCOT) deficiency. Anticancer research 19 15330163
1998 Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency: two pathogenic mutations, V133E and C456F, in Japanese siblings. Human mutation 19 9671268
2018 Development of Species-Specific SCAR Markers, Based on a SCoT Analysis, to Authenticate Physalis (Solanaceae) Species. Frontiers in genetics 18 29910824
2021 Succinyl-CoA:3-oxoacid coenzyme A transferase (SCOT) deficiency: A rare and potentially fatal metabolic disease. Biochimie 17 33596448
2019 Indirect Regeneration and Assessment of Genetic Fidelity of Acclimated Plantlets by SCoT, ISSR, and RAPD Markers in Rauwolfia tetraphylla L.: An Endangered Medicinal Plant. BioMed research international 17 31111050
2016 Molecular evolution and phylogenetic analysis of biocontrol genes acquired from SCoT polymorphism of mycoparasitic Trichoderma koningii inhibiting phytopathogen Rhizoctonia solani Kuhn. Infection, genetics and evolution : journal of molecular epidemiology and evolutionary genetics in infectious diseases 17 27720889
2003 Differential expression of succinyl CoA transferase (SCOT) genes in somatic and germline cells of the mouse testis. International journal of andrology 17 12534938
2021 RAPD, ISSR, and SCoT markers based genetic stability assessment of micropropagated Dendrobium fimbriatum Lindl. var. oculatum Hk. f.- an important endangered orchid. Physiology and molecular biology of plants : an international journal of functional plant biology 16 33707873
2021 Molecular diversity analysis in hexaploid wheat (Triticum aestivum L.) and two Aegilops species (Aegilops crassa and Aegilops cylindrica) using CBDP and SCoT markers. Journal, genetic engineering & biotechnology 15 33852105
2016 Genetic Homogeneity Revealed Using SCoT, ISSR and RAPD Markers in Micropropagated Pittosporum eriocarpum Royle- An Endemic and Endangered Medicinal Plant. PloS one 15 27434060
2022 Analysis of genetic diversity among common bean germplasm by start codon targeted (SCoT) markers. Molecular biology reports 14 35301653
2021 Assessment of genetic diversity among Iranian Aegilops triuncialis accessions using ISSR, SCoT, and CBDP markers. Journal, genetic engineering & biotechnology 14 33428012
2015 Start codon targeted (SCoT) polymorphism reveals genetic diversity in wild and domesticated populations of ramie (Boehmeria nivea L. Gaudich.), a premium textile fiber producing species. Meta gene 14 25750860
2023 CircRNA OXCT1 promotes the malignant progression and glutamine metabolism of non-small cell lung cancer by absorbing miR-516b-5p and upregulating SLC1A5. Cell cycle (Georgetown, Tex.) 13 35482822
2023 Genetic diversity analysis in wheat cultivars using SCoT and ISSR markers, chloroplast DNA barcoding and grain SEM. BMC plant biology 13 37041463
2022 Start Codon Targeted (SCoT) markers for the assessment of genetic diversity in yeast isolated from Turkish sourdough. Food microbiology 12 35953177
2021 lncRNA OXCT1-AS1 Promotes Metastasis in Non-Small-Cell Lung Cancer by Stabilizing LEF1, In Vitro and In Vivo. BioMed research international 12 34337014
2014 Type II thioesterase ScoT is required for coelimycin production by the modular polyketide synthase Cpk of Streptomyces coelicolor A3(2). Acta biochimica Polonica 12 24660171
2007 Identification and characterization of a temperature-sensitive R268H mutation in the human succinyl-CoA:3-ketoacid CoA transferase (SCOT) gene. Molecular genetics and metabolism 12 17706444
2019 Genetic homogeneity revealed in micropropagated Bauhinia racemosa Lam. using gene targeted markers CBDP and SCoT. Physiology and molecular biology of plants : an international journal of functional plant biology 11 30956438
2015 Genetic relationship and diversity among coconut (Cocos nucifera L.) accessions revealed through SCoT analysis. 3 Biotech 11 28324407
2010 A neonatal-onset succinyl-CoA:3-ketoacid CoA transferase (SCOT)-deficient patient with T435N and c.658-666dupAACGTGATT p.N220_I222dup mutations in the OXCT1 gene. Journal of inherited metabolic disease 11 20652411
2018 Development of SCoT-Based SCAR Marker for Rapid Authentication of Taxus Media. Biochemical genetics 10 29388069
2016 Genetic variation, population structure and linkage disequilibrium in Switchgrass with ISSR, SCoT and EST-SSR markers. Hereditas 10 28096766
2024 Applicability of SCoT markers in unraveling genetic variation and population structure among sugar beet (Beta vulgaris L.) germplasm. Molecular biology reports 9 38683231
2023 Neuroprotective mechanisms of OXCT1 via the SIRT3-SOD2 pathway after traumatic brain injury. Brain research 9 36921750
2021 Genetic relationships and diversity analysis in Turkish laurel (Laurus nobilis L.) germplasm using ISSR and SCoT markers. Molecular biology reports 9 34148209
2021 OXCT1 Enhances Gemcitabine Resistance Through NF-κB Pathway in Pancreatic Ductal Adenocarcinoma. Frontiers in oncology 9 34804914
2020 Droplet-vitrification of Aranda Broga Blue orchid: Role of ascorbic acid on the antioxidant system and genetic fidelity assessments via RAPD and SCoT markers. Biotechnology reports (Amsterdam, Netherlands) 9 32368510
2019 Assessment of genetic diversity, population structure and sex identification in dioecious crop, Trichosanthes dioica employing ISSR, SCoT and SRAP markers. Heliyon 9 30923770
2013 RpoS and oxidative stress conditions regulate succinyl-CoA: 3-ketoacid-coenzyme A transferase (SCOT) expression in Burkholderia pseudomallei. Microbiology and immunology 9 23808410
2023 Efficiency of RAPD and SCoT Markers in the Genetic Diversity Assessment of the Common Bean. Plants (Basel, Switzerland) 8 37570917
2022 Flow cytometry and start codon targeted (SCoT) genetic fidelity assessment of regenerated plantlets in Tylophora indica (Burm.f.) Merrill. Plant cell, tissue and organ culture 8 35250130
2021 DNA Fingerprinting and Genetic Relationships Similarities Among the Accessions/Species of Ocimum Using SCoT and ISSR Markers System. Molecular biotechnology 8 33754283
2019 Role of OXCT1 in ovine adipose and preadipocyte differentiation. Biochemical and biophysical research communications 8 30928098
2017 Manufacture of Autologous CD34+ Selected Grafts in the NIAID-Sponsored HALT-MS and SCOT Multicenter Clinical Trials for Autoimmune Diseases. Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation 8 28602891
2016 Potential Start Codon Targeted (SCoT) and Inter-retrotransposon Amplified Polymorphism (IRAP) Markers for Evaluation of Genetic Diversity and Conservation of Wild Pistacia Species Population. Biochemical genetics 8 27056191
2025 OXCT1 promotes triple negative breast cancer immune escape via modulating succinylation modification of PGK1. Communications biology 6 40634657
2023 A Novel Mutation in the OXCT1 Gene Causing Succinyl-CoA:3-Ketoacid CoA Transferase (SCOT) Deficiency Starting with Neurologic Manifestations. Iranian journal of child neurology 6 37091464
2023 Deciphering the level of genetic diversity in some aegilops species using CAAT box-derived polymorphism (CBDP) and start codon target polymorphism (SCoT) markers. Molecular biology reports 6 37219668
2022 MicroRNA-886 suppresses osteosarcoma cell proliferation and its maturation is suppressed by long non-coding RNA OXCT1-AS1. Bioengineered 6 35191809
2022 LncRNA OXCT1-AS1 promotes the proliferation of non-small cell lung cancer cells by targeting the miR-195/CCNE1 axis. Translational cancer research 6 35706791
2021 Genetic diversity analysis in a mini core collection of Damask rose (Rosa damascena Mill.) germplasm from Iran using URP and SCoT markers. Journal, genetic engineering & biotechnology 6 34591207
2021 Clinical variability and outcome of succinyl-CoA:3-ketoacid CoA transferase deficiency caused by a single OXCT1 mutation: Report of 17 cases. JIMD reports 6 34765403
2020 SCOT: Rethinking the classification of secondary structure elements. Bioinformatics (Oxford, England) 6 31742326
2006 Use of the SCOT solution in kidney transplantation: preliminary report. Transplantation proceedings 6 16980064
2024 OXCT1 regulates hippocampal neurogenesis and alleviates cognitive impairment via the Akt/GSK-3β/β-catenin pathway after subarachnoid hemorrhage. Brain research 5 38199308
2024 Evaluation of genetic diversity and population structure of Annamocarya sinensis using SCoT markers. PloS one 5 39231174
2022 Ploidy Status, Nuclear DNA Content and Start Codon Targeted (SCoT) Genetic Homogeneity Assessment in Digitalis purpurea L., Regenerated In Vitro. Genes 5 36553602
2019 The study of inter-specific relationships of Bromus genus based on SCoT and ISSR molecular markers. Molecular biology reports 5 31313131
2018 Exploring genetic variability in Prosopis cineraria using two gene targeted CAAT box-derived polymorphism (CBDP) and start codon targeted (SCoT) polymorphism markers. Molecular biology reports 5 30255277
2012 Identification of ORF sequences and exercise-induced expression change in thoroughbred horse OXCT1 gene. Gene 5 22301269
2024 Induced genetic diversity through mutagenesis in wheat gene pool and significant use of SCoT markers to underpin key agronomic traits. BMC plant biology 4 39004709
2022 Single-Cell Multiomics Integration by SCOT. Journal of computational biology : a journal of computational molecular cell biology 4 34985990
2019 A Rare Cause of Life-Threatening Ketoacidosis: Novel Compound Heterozygous OXCT1 Mutations Causing Succinyl-CoA:3-Ketoacid CoA Transferase Deficiency. Yonsei medical journal 4 30799594
2017 Antioxidant System Response and cDNA-SCoT Marker Profiling in Phoenix dactylifera L. Plant under Salinity Stress. International journal of genomics 4 28702461
2025 Unveiling genetic diversity and population structure in lentil (Lens culinaris) germplasm through scot markers. Molecular biology reports 3 40731106
2024 Assessment of genetic homogeneity of in-vitro propagated apple root stock MM 104 using ISSR and SCoT primers. BMC plant biology 3 38570817
2023 Impacts of ZnO as a nanofertilizer on fenugreek: some biochemical parameters and SCoT analysis. Journal, genetic engineering & biotechnology 3 37126122
2022 Molecular Characterization of Tinospora cordifolia (Willd.) Miers Using Novel g-SSR Markers and Their Comparison with EST-SSR and SCoT Markers for Genetic Diversity Study. Genes 3 36360279
2017 A Case of Succinyl-CoA:3-Oxoacid CoA Transferase Deficiency Presenting with Severe Acidosis in a 14-Month-Old Female: Evidence for Pathogenicity of a Point Mutation in the OXCT1 Gene. Journal of pediatric intensive care 3 31073471
2015 Genetic structure and genetic diversity of single-variety Lonicera macranthoides populations in China, as indicated by SCoT markers. Genetics and molecular research : GMR 3 26214488
2024 Protective Effect of Long Noncoding RNA OXCT1-AS1 on Doxorubicin-Induced Apoptosis of Human Myocardial Cells by the Competitive Endogenous RNA Pattern. Arquivos brasileiros de cardiologia 2 38958296