Affinage

OXCT1

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

Length
520 aa
Mass
56.2 kDa
Annotated
2026-06-10
100 papers in source corpus 22 papers cited in narrative 22 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/7 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

OXCT1 (SCOT) is a mitochondrial homodimeric CoA-transferase that catalyzes the rate-limiting, reversible step of ketolysis, transferring CoA between succinyl-CoA and acetoacetate to generate acetoacetyl-CoA for energy production; loss of this activity abolishes ketone-body utilization (PMID:8751852, PMID:36016708). The human crystal structure mapped ~20 disease-associated alleles and showed that pathogenic missense variants act predominantly by destabilizing the homodimer rather than by directly poisoning the active site (PMID:23420214, PMID:21296660), a principle anticipated by homology modeling that localized mutations to the dimer interface (V221, G219) versus the catalytic region (G324) (PMID:10964512). Several variants confer temperature-sensitive instability—retaining substantial activity at 30°C but losing it at febrile temperatures—explaining episodic ketoacidotic crises in SCOT deficiency, a Mendelian disorder caused by loss-of-function OXCT1 mutations (PMID:8751852, PMID:15496607, PMID:17706444). Multiple pathogenic alleles also disrupt splicing, producing exon-skipping transcripts subject to nonsense-mediated decay (PMID:17169596, PMID:23281106). Beyond catalysis, OXCT1 acts as a lysine succinyltransferase: residue G424 is required for transfer of succinyl groups to substrate proteins including LACTB (K284), inhibiting LACTB proteolysis to enhance mitochondrial respiration, and PGK1 (K146), stabilizing PGK1 to drive aerobic glycolysis and immune escape (PMID:38176415, PMID:40634657). OXCT1 enzymatic activity is itself controlled by post-translational modification: it is activated by SUCLA2-dependent succinylation at K421 downstream of IGF1/ERK2/PIN1 signaling (PMID:39862868), stabilized by frataxin through suppression of ubiquitin-proteasome–mediated degradation (PMID:36016708), and inhibited by peroxynitrite nitration at Tyr4/Tyr76 (PMID:20527992). Through its metabolic output, OXCT1 shapes tissue physiology and tumor biology: ketolysis-derived succinate accumulates in tumor-associated macrophages and epigenetically promotes Arg1 transcription via H3K4me3, driving protumor macrophage polarization and CD8+ T-cell exhaustion (PMID:38759889), while in pancreatic islets OXCT1 supports glucose- and ketone-stimulated insulin secretion (PMID:20460097).

Mechanistic history

Synthesis pass · year-by-year structured walk · 19 steps
  1. 1996 High

    Established OXCT1 as the gene whose loss causes SCOT deficiency, defining its essential role in ketolysis at the molecular-genetic level.

    Evidence cDNA cloning, chromosomal mapping to 5p13, and identification of the S283X nonsense mutation in a patient

    PMID:8751852

    Open questions at the time
    • Did not resolve enzyme structure or catalytic mechanism
    • No genotype–phenotype mechanism for missense alleles
  2. 2000 High

    Linked specific residues to dimerization versus catalysis, showing pathogenic mutations map either to the dimer interface or the active site and predict residual-activity severity.

    Evidence Genomic structure, homology modeling on glutaconate CoA transferase, and transient expression of G219E/G324E/V221M in SCOT-deficient fibroblasts

    PMID:10964512

    Open questions at the time
    • Structural assignments rested on a homology model, not an experimental human structure
    • Limited to three variants
  3. 1998 Medium

    Validated a transient-expression assay that distinguishes pathogenic from neutral variants and warned that homogenate activity can be artifactual.

    Evidence Transient expression of V133E, C456F, and T58M in SCOT-deficient fibroblasts with activity and immunoblot

    PMID:9671268

    Open questions at the time
    • Single lab
    • No structural basis for activity loss provided
  4. 2004 Medium

    Explained why patients tolerate normal periods but crash during fever, by showing certain mutants are temperature-sensitive.

    Evidence Transient expression of T435N at 30/37/39.5°C with heat-stability assays

    PMID:15496607

    Open questions at the time
    • Mechanism of thermal destabilization inferred, not structurally resolved at the time
    • Single allele
  5. 2007 Medium

    Provided a residue-level structural rationale for temperature sensitivity, tying instability to loss of a conserved salt bridge.

    Evidence Transient expression of R268H across a temperature series with predicted R268–D52 salt-bridge disruption

    PMID:17706444

    Open questions at the time
    • Salt-bridge effect predicted, not directly measured
    • Single patient
  6. 2006 Medium

    Showed that pathogenic OXCT1 alleles can act at the RNA level, producing exon-skipped transcripts cleared by nonsense-mediated decay.

    Evidence Nuclear/cytoplasmic RNA fractionation and sequencing of c.671G>A splice variants

    PMID:17169596

    Open questions at the time
    • Single mutation analyzed
    • Quantitative contribution to disease not established
  7. 2013 High

    Delivered the experimental human SCOT crystal structure and consolidated the unifying disease principle: pathogenic variants chiefly destabilize the homodimer.

    Evidence X-ray crystallography of human SCOT with structural mapping of ~20 disease alleles; complementary expression/structural modeling of additional temperature-sensitive variants

    PMID:21296660 PMID:23420214

    Open questions at the time
    • Did not capture post-translationally modified or substrate-bound states
    • Catalytic vs stability contributions per allele not all individually measured
  8. 2013 Medium

    Resolved an unusual splicing pathology in which a donor-site mutation paralyzes the normal intron-removal order, forcing two-exon skipping.

    Evidence RT-PCR of hnRNA intermediates comparing intron-removal order in patient and control fibroblasts for c.1248+5g>a

    PMID:23281106

    Open questions at the time
    • Single patient
    • Generalizability of the splicing-paralysis model unknown
  9. 2010 High

    Identified the first inhibitory post-translational regulation of OXCT1, with tyrosine nitration causally suppressing catalysis in diabetic heart.

    Evidence MS site identification of Tyr4/Tyr76 nitration, recombinant peroxynitrite treatment, and protective site-directed mutagenesis with activity assays

    PMID:20527992

    Open questions at the time
    • Physiological extent of nitration in vivo not quantified
    • Reversibility/repair not addressed
  10. 2010 High

    Demonstrated a tissue function beyond systemic ketolysis, placing OXCT1 in the insulin-secretion pathway of pancreatic beta cells.

    Evidence Reduced islet SCOT in GK rats and shRNA knockdown in INS-1 cells with insulin-release readouts

    PMID:20460097

    Open questions at the time
    • Molecular link between SCOT activity and secretory machinery not defined
    • Cell-line based knockdown
  11. 2022 High

    Established that OXCT1 protein abundance is controlled by frataxin, identifying a proteostatic regulator and a metabolic consequence of OXCT1 loss.

    Evidence Reciprocal Co-IP, frataxin gain/loss with proteasome inhibition, KIKO mouse, and ketone-body/succinyl-CoA metabolite measurements

    PMID:36016708

    Open questions at the time
    • Ubiquitin ligase mediating OXCT1 degradation not identified
    • Direct vs indirect frataxin–OXCT1 contact unresolved
  12. 2024 High

    Reclassified OXCT1 as a moonlighting lysine succinyltransferase, with substrate succinylation rewiring mitochondrial and tumor metabolism.

    Evidence In vitro succinyltransferase assay, G424 mutagenesis, MS mapping of LACTB K284, and LACTB protease/mitochondrial respiration readouts

    PMID:38176415

    Open questions at the time
    • Full substrate repertoire unknown
    • Structural basis of succinyltransferase activity vs CoA-transfer not defined
  13. 2024 High

    Connected OXCT1 metabolic output to the tumor immune microenvironment via succinate-driven epigenetic macrophage reprogramming.

    Evidence Macrophage-specific OXCT1 knockout mice, H3K4me3 ChIP at the Arg1 promoter, succinate measurement, and CD8+ T-cell assays

    PMID:38759889

    Open questions at the time
    • Enzyme(s) writing H3K4me3 in response to succinate not pinpointed
    • Relative contribution of macrophage vs tumor-cell OXCT1 unresolved
  14. 2025 High

    Showed OXCT1 activity is switched on by signaling-driven succinylation, integrating growth-factor input with ketolytic capacity.

    Evidence IGF1-induced OXCT1–SUCLA2 Co-IP, ERK2 kinase and PIN1 isomerization assays, MS mapping of OXCT1 K421 succinylation, activity assays, and tumor models

    PMID:39862868

    Open questions at the time
    • Whether K421 succinylation is enzymatic or non-enzymatic in this context not fully delineated
    • Reversal/desuccinylase not identified
  15. 2025 Medium

    Extended OXCT1 succinyltransferase function to PGK1, linking it to glycolysis, PD-L1, and immune escape, and identified a chromatin regulator of OXCT1 expression.

    Evidence MS mapping of PGK1 K146 succinylation, ubiquitination/stability assays, organoids, and KMT5A/H4K20me1 ChIP at the OXCT1 promoter

    PMID:40634657

    Open questions at the time
    • Single-lab succinylation mapping
    • Direct vs indirect KMT5A control of OXCT1 promoter not fully resolved
  16. 2023 Medium

    Implicated OXCT1 in neuronal stress resilience by linking it to the SIRT3–SOD2 antioxidant axis after brain injury.

    Evidence AAV OXCT1 overexpression in mouse hippocampus with SIRT3/acetyl-SOD2 immunoblots, ROS staining, and cognitive testing

    PMID:36921750

    Open questions at the time
    • Mechanism connecting OXCT1 to SIRT3 induction unknown
    • Single study, gain-of-function only
  17. 2024 Medium

    Associated neuronal OXCT1 with pro-neurogenic Akt/GSK-3β/β-catenin signaling and cognitive recovery after hemorrhage.

    Evidence AAV OXCT1 overexpression in SAH mice with LY294002 rescue, doublecortin/EdU staining, pathway immunoblots, and behavioral tests

    PMID:38199308

    Open questions at the time
    • How OXCT1 activates Akt signaling not defined
    • Single lab
  18. 2021 Medium

    Linked OXCT1 to chemoresistance through NF-κB signaling in pancreatic cancer.

    Evidence OXCT1 gain/loss in PDAC cells, apoptosis/colony assays, NF-κB inhibitor rescue, and mouse tumor models

    PMID:34804914

    Open questions at the time
    • Mechanism coupling OXCT1 to NF-κB not established
    • Single lab
  19. 2003 Medium

    Distinguished somatic OXCT1 expression in testicular Leydig/Sertoli cells from the germ-cell paralog SCOT-t, clarifying tissue-specific ketolytic capacity.

    Evidence In situ hybridization/RT-PCR for cell-type expression and SCOT activity assays in isolated cell fractions

    PMID:12534938

    Open questions at the time
    • Functional role of OXCT1 in somatic testis cells not tested
    • Activity attribution between paralogs partly inferential

Open questions

Synthesis pass · forward-looking unresolved questions
  • How OXCT1 toggles between CoA-transferase and succinyltransferase activities, what determines its full succinylation substrate repertoire, and the identity of the ligase/desuccinylase machinery regulating it remain unresolved.
  • No structure of the succinyltransferase-competent or substrate-bound state
  • Complete physiological substrate set undefined
  • Degradation and desuccinylation machinery not identified

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016740 transferase activity 4 GO:0140096 catalytic activity, acting on a protein 2 GO:0016787 hydrolase activity 1
Localization
GO:0005739 mitochondrion 3
Pathway
R-HSA-1430728 Metabolism 2 R-HSA-1643685 Disease 2 R-HSA-168256 Immune System 2 R-HSA-392499 Metabolism of proteins 2
Partners
FXNLACTBPGK1SUCLA2

Evidence

Reading pass · 22 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1996 Human SCOT (OXCT1) cDNA was cloned, revealing a 1,560-nt coding sequence. The gene was mapped to chromosome 5p13 by in situ hybridization. A homozygous C-to-G transversion at nt 848 (S283X nonsense mutation) was identified as the first pathogenic mutation in SCOT deficiency, establishing that loss of OXCT1 function abolishes ketolytic capacity. cDNA cloning, chromosomal mapping by in situ hybridization, mutation detection by RT-PCR and sequencing 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 the Acidaminococcus fermentans glutaconate CoA transferase crystal structure predicted that V221 and G219 are on the dimerizing surface while G324 is near the active site. Transient expression of G219E and G324E mutant cDNAs in SCOT-deficient fibroblasts produced no detectable activity, whereas V221M yielded ~10% of control and detectable activity, correlating with the mildest clinical course reported. Genomic cloning, tertiary structural homology modeling, transient expression assay in SCOT-deficient fibroblasts Genomics High 10964512
2013 Crystal structure of human SCOT was determined, providing a molecular map of disease-associated mutations in OXCT1. The structure revealed structural effects of ~20 disease-associated alleles, showing that pathogenic variants cluster in regions affecting protein stability rather than solely at the catalytic site. X-ray crystallography (crystal structure determination), structural mapping of mutations Journal of inherited metabolic disease High 23420214
2010 In the db/db diabetic mouse heart, SCOT (OXCT1) is nitrated by peroxynitrite at Tyr4 and Tyr76. Site-directed mutagenesis of these residues significantly protected recombinant SCOT from peroxynitrite modification and prevented loss of enzymatic activity, establishing that nitration of these two tyrosine residues causally inhibits SCOT catalysis. 2DE/Western blot/MS proteomics, recombinant protein incubation with peroxynitrite, LC-ESI-MS/MS site identification, site-directed mutagenesis with enzyme activity assay Journal of proteome research High 20527992
2010 SCOT (OXCT1) mRNA and enzyme activity are decreased >70% in pancreatic islets of the GK rat model of type 2 diabetes. shRNA-mediated knockdown of SCOT in INS-1 832/13 insulinoma cells (>70% reduction) caused >70% reduction in glucose- or methyl succinate-plus-β-hydroxybutyrate-stimulated insulin release, establishing a functional role for OXCT1 in insulin secretion. Quantitative RT-PCR, enzyme activity assay in rat islets, shRNA knockdown in INS-1 cells, insulin release assay Archives of biochemistry and biophysics High 20460097
2022 Frataxin physically interacts with OXCT1 both in vivo and in vitro. Frataxin overexpression increases OXCT1 protein levels while frataxin deficiency decreases OXCT1 in cerebellum and skeletal muscle. This regulation occurs via frataxin-dependent suppression of ubiquitin-proteasome system (UPS)-mediated OXCT1 degradation. Frataxin-deficient cells fail to metabolize ketone bodies to acetyl-CoA, accompanied by increased succinyl-CoA, confirming OXCT1 as the downstream effector. Co-immunoprecipitation (in vivo and in vitro), frataxin overexpression/knockdown, proteasome inhibition assay, metabolite measurement (plasma ketone bodies and succinyl-CoA), conditional knockout mouse (KIKO) PNAS nexus High 36016708
2024 OXCT1 functions as a lysine succinyltransferase in addition to its canonical ketolytic role. Residue G424 is essential for this succinyltransferase activity. LACTB was identified as a primary target of OXCT1-mediated succinylation; succinylation of LACTB at K284 inhibits LACTB's proteolytic activity, resulting in increased mitochondrial membrane potential and respiration, promoting hepatocellular carcinoma progression. In vitro succinyltransferase assay, site-directed mutagenesis (G424), mass spectrometry identification of succinylation site on LACTB K284, LACTB proteolytic activity assay, mitochondrial functional assays Molecular cell High 38176415
2025 OXCT1 interacts with SUCLA2 upon IGF1 stimulation in HCC cells, driven by ERK2-mediated SUCLA2 S124 phosphorylation and subsequent PIN1-mediated cis-trans isomerization of SUCLA2. SUCLA2-associated OXCT1 generates succinyl-CoA, which directly succinylates OXCT1 at K421, activating OXCT1 enzymatic activity and enhancing ketolysis and tumor growth. Co-immunoprecipitation, in vitro kinase assay (ERK2), PIN1 isomerization assay, succinylation site mapping by MS, OXCT1 activity assay, murine tumor models Molecular cell High 39862868
2024 OXCT1 promotes antitumor immunity suppression in hepatocellular carcinoma by causing accumulation of succinate (a byproduct of ketolysis) in tumor-associated macrophages, which epigenetically increases H3K4me3 levels at the Arg1 promoter, thereby promoting Arg1 transcription and macrophage polarization toward a protumor phenotype, leading to CD8+ T-cell exhaustion. Conditional macrophage-specific OXCT1 knockout mice (LysMcreOXCT1f/f), chromatin immunoprecipitation (H3K4me3 at Arg1 promoter), succinate metabolite measurement, CD8+ T-cell functional assays, multiplex immunohistochemistry Journal of hepatology High 38759889
2025 OXCT1 succinylates PGK1 at K146, increasing PGK1 protein stability (reducing ubiquitination) without affecting PGK1 mRNA, thereby promoting aerobic glycolysis and PD-L1 expression and immune escape in triple negative breast cancer. KMT5A promotes OXCT1 expression through H4K20me1 histone methylation at the OXCT1 promoter. Succinylation site mapping (MS), ubiquitination assay, OXCT1 overexpression/knockdown, patient-derived organoids, ChIP (H4K20me1 at OXCT1 promoter), T-cell killing assay Communications biology Medium 40634657
2023 OXCT1 overexpression in hippocampal neurons after traumatic brain injury increased SIRT3 expression and reduced acetylation of SOD2, decreasing reactive oxygen species production, reducing neuronal death, and improving cognitive function. This places OXCT1 upstream of the SIRT3-SOD2 antioxidant axis in neurons. AAV-mediated OXCT1 overexpression in mouse hippocampus, Western blotting (SIRT3, acetylated-SOD2), Dihydroethidium ROS staining, Nissl staining (neuronal death), Morris water maze and Y-maze (cognitive function) Brain research Medium 36921750
2024 OXCT1 overexpression in hippocampal neurons after subarachnoid hemorrhage activated Akt/GSK-3β/β-catenin signaling, promoted adult hippocampal neurogenesis (assessed by doublecortin/EdU staining), and improved cognitive function. Pharmacological inhibition of Akt (LY294002) reversed these effects, establishing OXCT1 upstream of this pro-neurogenic signaling cascade. AAV-mediated neuronal OXCT1 overexpression in SAH mouse model, pharmacological PI3K/Akt inhibition (LY294002), immunofluorescence (doublecortin/EdU), Western blotting (Akt/GSK-3β/β-catenin), Morris water maze/Y-maze Brain research Medium 38199308
1998 Using a transient expression system in SCOT-deficient fibroblasts, mutations V133E and C456F in OXCT1 were shown to produce no detectable SCOT enzymatic activity, while co-occurring T58M was functionally neutral. The system also revealed that apparent residual SCOT activity measured in cell homogenates may be artifactual due to other enzymes acting on the substrate acetoacetyl-CoA. Transient expression assay in immortalized SCOT-deficient fibroblasts, enzyme activity assay, immunoblotting Human mutation Medium 9671268
2004 Transient expression analysis showed that the OXCT1 T435N mutant retains significant residual SCOT activity (~25% at 37°C, ~50% at 30°C), and the mutant protein is temperature-sensitive and more vulnerable to heat treatment than wild-type. This temperature sensitivity explains why patients develop ketoacidotic crises during febrile illness despite lacking permanent ketosis. Transient expression assay in SCOT-deficient fibroblasts, enzyme activity at multiple temperatures (30°C, 37°C, 39.5°C), heat treatment stability assay Pediatric research Medium 15496607
2007 The R268H OXCT1 mutation causes temperature-sensitive SCOT deficiency: the mutant protein retains ~60% activity at 30°C but only ~4% at 40°C. Structural analysis predicted that R268H disrupts a conserved salt bridge between R268 and D52, destabilizing the protein in a temperature-dependent manner. Transient expression assay at multiple temperatures, heat-treatment stability assay, 3D structural analysis (salt bridge prediction) Molecular genetics and metabolism Medium 17706444
2011 Structural analysis of SCOT mutations revealed that missense mutations L327P, R468C, and A215V retain some residual activity but are temperature-sensitive (more stable at 30°C than 37°C). Main effects of pathogenic mutations are destabilization of the SCOT homodimer, with some mutations also directly affecting catalytic activity, as predicted from the tertiary structure. Transient expression assay (37°C and 30°C), immunoblot analysis, tertiary structural modeling of mutation effects Biochimica et biophysica acta Medium 21296660
2006 A point mutation at the last nucleotide of exon 6 (c.671G>A) of OXCT1 causes exon skipping. Nuclear RNA analysis showed exon 6 skipping as the predominant nuclear transcript, but in cytoplasmic RNA, exons 6+7 skipping was more abundant. This was explained by nonsense-mediated mRNA decay of the exon-6-skipped transcript in the cytoplasm, while the in-frame exons-6+7-skipped mRNA is stable. RT-PCR of nuclear and cytoplasmic RNA fractions, sequencing of splice variants Molecular genetics and metabolism Medium 17169596
2013 A splice-donor mutation c.1248+5g>a (IVS13) in OXCT1 causes predominantly two-exon skipping (exons 12 and 13) via a 'splicing paralysis' mechanism. Analysis of hnRNA intermediates showed that in controls, intron 11 is the last intron spliced and intron 12 removal is slow and follows intron 13 removal. The mutation causes intron 13 retention, leading to retention of introns 12 and 11, resolved by skipping the entire intron11-exon12-intron12-exon13-mutated intron13 block. RT-PCR of heteronuclear RNA intermediates (hnRNA), comparison of spliced exon cluster intermediates between control and patient fibroblasts Human mutation Medium 23281106
2002 Human testis-specific OXCT2 (h-Scot-t), a paralog of OXCT1, is encoded by an intronless gene located within an intron of the BMP8 gene at 1p34.1-35.3. The protein localizes to the mitochondria-containing midpiece of ejaculated spermatozoa, suggesting a role in ketone body metabolism for sperm energy. (Note: this describes the OXCT2/SCOT-t paralog, not OXCT1 itself.) cDNA cloning, PCR-based genomic structure analysis, immunolocalization in sperm Molecular human reproduction Low 11756565
2003 In mouse testis, OXCT1 (SCOT-s) is expressed exclusively in somatic cells (Leydig and Sertoli cells), while the germ-cell-specific paralog SCOT-t is expressed in germ cells. SCOT enzymatic activity was measured in both Leydig cell fractions (SCOT-s) and sperm fractions (SCOT-t), with sperm activity 2.5-fold higher than Leydig cells. In situ hybridization/RT-PCR for cell-type expression, enzyme activity assay in isolated cell fractions International journal of andrology Medium 12534938
2021 OXCT1 promotes gemcitabine resistance in pancreatic ductal adenocarcinoma through the NF-κB signaling pathway. OXCT1 overexpression inhibited apoptosis after gemcitabine treatment, and this resistance was reversed by an NF-κB inhibitor. Knockdown of OXCT1 sensitized cells to gemcitabine both in vitro and in vivo. OXCT1 overexpression/knockdown in PDAC cell lines, flow cytometry (apoptosis), colony formation, RTCA cytotoxicity, GSEA pathway analysis, NF-κB inhibitor rescue, mouse tumor models Frontiers in oncology Medium 34804914
2019 OXCT1 knockdown in ovine preadipocytes promoted lipid accumulation, while overexpression had the converse effect, establishing that OXCT1 negatively regulates adipocyte lipid deposition. OXCT1 expression increased during adipocyte differentiation but decreased dramatically at day 8. OXCT1 knockdown and overexpression in ovine preadipocytes, lipid accumulation assay (Oil Red O staining), gene expression profiling during differentiation Biochemical and biophysical research communications Low 30928098

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 101 26824445
2022 SCOT: Single-Cell Multi-Omics Alignment with Optimal Transport. Journal of computational biology : a journal of computational molecular cell biology 82 35050714
2016 Diagnostic Strategies for the Evaluation of Chest Pain: Clinical Implications From SCOT-HEART and PROMISE. Journal of the American College of Cardiology 75 26892420
2024 OXCT1 functions as a succinyltransferase, contributing to hepatocellular carcinoma via succinylating LACTB. Molecular cell 68 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 61 23036114
2017 The role of OXCT1 in the pathogenesis of cancer as a rate-limiting enzyme of ketone body metabolism. Life sciences 58 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 56 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 43 38759889
2013 Start Codon Targeted (SCoT) marker reveals genetic diversity of Dendrobium nobile Lindl., an endangered medicinal orchid species. Gene 38 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
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 28 32146386
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
2015 Potential of Start Codon Targeted (SCoT) markers to estimate genetic diversity and relationships among Chinese Elymus sibiricus accessions. Molecules (Basel, Switzerland) 27 25853316
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 A structural mapping of mutations causing succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency. Journal of inherited metabolic disease 26 23420214
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
2022 Frataxin controls ketone body metabolism through regulation of OXCT1. PNAS nexus 25 36016708
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
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
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 24 20460097
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 24 19074611
2025 OXCT1 succinylation and activation by SUCLA2 promotes ketolysis and liver tumor growth. Molecular cell 22 39862868
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
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
2018 Development of Species-Specific SCAR Markers, Based on a SCoT Analysis, to Authenticate Physalis (Solanaceae) Species. Frontiers in genetics 19 29910824
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
2021 Succinyl-CoA:3-oxoacid coenzyme A transferase (SCOT) deficiency: A rare and potentially fatal metabolic disease. Biochimie 17 33596448
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 17 33707873
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
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
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
2023 Neuroprotective mechanisms of OXCT1 via the SIRT3-SOD2 pathway after traumatic brain injury. Brain research 10 36921750
2021 OXCT1 Enhances Gemcitabine Resistance Through NF-κB Pathway in Pancreatic Ductal Adenocarcinoma. Frontiers in oncology 10 34804914
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
2013 RpoS and oxidative stress conditions regulate succinyl-CoA: 3-ketoacid-coenzyme A transferase (SCOT) expression in Burkholderia pseudomallei. Microbiology and immunology 10 23808410
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 Efficiency of RAPD and SCoT Markers in the Genetic Diversity Assessment of the Common Bean. Plants (Basel, Switzerland) 9 37570917
2021 DNA Fingerprinting and Genetic Relationships Similarities Among the Accessions/Species of Ocimum Using SCoT and ISSR Markers System. Molecular biotechnology 9 33754283
2021 Genetic relationships and diversity analysis in Turkish laurel (Laurus nobilis L.) germplasm using ISSR and SCoT markers. Molecular biology reports 9 34148209
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
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
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 7 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 Single-Cell Multiomics Integration by SCOT. Journal of computational biology : a journal of computational molecular cell biology 6 34985990
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
2023 Impacts of ZnO as a nanofertilizer on fenugreek: some biochemical parameters and SCoT analysis. Journal, genetic engineering & biotechnology 5 37126122
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 A Rare Cause of Life-Threatening Ketoacidosis: Novel Compound Heterozygous OXCT1 Mutations Causing Succinyl-CoA:3-Ketoacid CoA Transferase Deficiency. Yonsei medical journal 5 30799594
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 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 4 36360279
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
2024 Development of highly discriminatory SCoT- and CBDP-based SCAR fingerprint for authentication of Indian senna (Senna alexandrina Mill.) formerly Cassia angustifolia Vahl.). Frontiers in plant science 3 39027666
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
2022 Using of DNA-Barcoding, SCoT and SDS-PAGE Protein to Assess Soma-Clonal Variation in Micro-Propagated Fig (Ficus carica L.) Plant. Pakistan journal of biological sciences : PJBS 2 35638512

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