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SUGCT succinyl-CoA:glutarate-CoA transferase [ Homo sapiens (human) ]

Gene ID: 79783, updated on 2-Nov-2024

Summary

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Official Symbol
SUGCTprovided by HGNC
Official Full Name
succinyl-CoA:glutarate-CoA transferaseprovided by HGNC
Primary source
HGNC:HGNC:16001
See related
Ensembl:ENSG00000175600 MIM:609187; AllianceGenome:HGNC:16001
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
GA3; ORF19; DERP13; C7orf10
Summary
This gene encodes a protein that is similar to members of the CaiB/baiF CoA-transferase protein family. Mutations in this gene are associated with glutaric aciduria type III. Alternate splicing results in multiple transcript variants. [provided by RefSeq, Jul 2010]
Expression
Biased expression in kidney (RPKM 3.4), liver (RPKM 1.2) and 12 other tissues See more
Orthologs
mouse all
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Genomic context

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See SUGCT in Genome Data Viewer
Location:
7p14.1
Exon count:
24
Annotation release Status Assembly Chr Location
RS_2024_08 current GRCh38.p14 (GCF_000001405.40) 7 NC_000007.14 (40135005..41038816)
RS_2024_08 current T2T-CHM13v2.0 (GCF_009914755.1) 7 NC_060931.1 (40292462..41196673)
RS_2024_09 previous assembly GRCh37.p13 (GCF_000001405.25) 7 NC_000007.13 (40174604..40900362)

Chromosome 7 - NC_000007.14Genomic Context describing neighboring genes Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 18117 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 25890 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 18118 Neighboring gene CDK7 strongly-dependent group 2 enhancer GRCh37_chr7:40186973-40188172 Neighboring gene OCT4-NANOG-H3K27ac hESC enhancer GRCh37_chr7:40188525-40189421 Neighboring gene HIG1 hypoxia inducible domain family member 1A pseudogene 7 Neighboring gene THUMP domain containing 3 pseudogene 1 Neighboring gene M-phase specific PLK1 interacting protein Neighboring gene uncharacterized LOC105375245 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 25891 Neighboring gene MPRA-validated peak6500 silencer Neighboring gene ATAC-STARR-seq lymphoblastoid active region 25892 Neighboring gene Sharpr-MPRA regulatory region 14136 Neighboring gene uncharacterized LOC105375244 Neighboring gene OCT4-NANOG hESC enhancer GRCh37_chr7:40608892-40609475 Neighboring gene SUGCT antisense RNA 1 Neighboring gene uncharacterized LOC105375243 Neighboring gene MED14-independent group 3 enhancer GRCh37_chr7:40750799-40751998 Neighboring gene MED14-independent group 3 enhancer GRCh37_chr7:40756566-40757765 Neighboring gene H3K27ac hESC enhancer GRCh37_chr7:40854869-40855369 Neighboring gene uncharacterized LOC112267984 Neighboring gene uncharacterized LOC105375242 Neighboring gene BRD4-independent group 4 enhancer GRCh37_chr7:40924401-40925600 Neighboring gene MED14-independent group 3 enhancer GRCh37_chr7:41045810-41047009 Neighboring gene long intergenic non-protein coding RNA 1450 Neighboring gene P300/CBP strongly-dependent group 1 enhancer GRCh37_chr7:41068008-41069207 Neighboring gene Sharpr-MPRA regulatory region 15680 Neighboring gene OCT4-NANOG hESC enhancer GRCh37_chr7:41098757-41099608 Neighboring gene MED14-independent group 3 enhancer GRCh37_chr7:41104940-41106139 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 25894 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 25895 Neighboring gene P300/CBP strongly-dependent group 1 enhancer GRCh37_chr7:41146908-41148107 Neighboring gene uncharacterized LOC105375241 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 18119 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 25896 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 25897 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 25898 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr7:41174879-41175380 Neighboring gene long intergenic non-protein coding RNA 1449 Neighboring gene uncharacterized LOC105375246

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: HPA RNA-seq normal tissues HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Apr 4 07:08:55 2018

Bibliography

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Related articles in PubMed

  1. Genetic mapping of glutaric aciduria, type 3, to chromosome 7 and identification of mutations in c7orf10. Sherman EA, et al. Am J Hum Genet, 2008 Nov. PMID 18926513, Free PMC Article
  2. Novel contiguous gene deletion in peruvian girl with Trichothiodystrophy type 4 and glutaric aciduria type 3. La Serna-Infantes J, et al. Eur J Med Genet, 2018 Jul. PMID 29421601
  3. C7orf10 encodes succinate-hydroxymethylglutarate CoA-transferase, the enzyme that converts glutarate to glutaryl-CoA. Marlaire S, et al. J Inherit Metab Dis, 2014 Jan. PMID 23893049
  4. Glutaric aciduria type 3 is a naturally occurring biochemical trait in inbred mice of 129 substrains. Leandro J, et al. Mol Genet Metab, 2021 Feb. PMID 33483254
  5. Molecular genetic studies of human chromosome 7 in Russell-Silver syndrome. Nakabayashi K, et al. Genomics, 2002 Feb. PMID 11829489

See all (27) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Glutaric aciduria type 3 is a naturally occurring biochemical trait in inbred mice of 129 substrains.
    Title: Glutaric aciduria type 3 is a naturally occurring biochemical trait in inbred mice of 129 substrains.
  2. Chromosome microarray analysis showed a 125kb homozygous pathogenic deletion, which includes genes MPLKIP and SUGCT, not described before. This is the first case described in Peru of a novel contiguous gene deletion of Trichothiodystrophy type 4 and Glutaric aciduria type 3 performed by chromosome microarray analysis.
    Title: Novel contiguous gene deletion in peruvian girl with Trichothiodystrophy type 4 and glutaric aciduria type 3.
  3. C7orf10 encodes succinate-hydroxymethylglutarate CoA-transferase, which is the enzyme that converts glutarate to glutaryl-CoA
    Title: C7orf10 encodes succinate-hydroxymethylglutarate CoA-transferase, the enzyme that converts glutarate to glutaryl-CoA.
  4. Identified as a candidate disease gene for OXPHOS disorders by next-generation sequencing
    Title: Molecular diagnosis of infantile mitochondrial disease with targeted next-generation sequencing.
  5. Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
    Title: Variation at the NFATC2 locus increases the risk of thiazolidinedione-induced edema in the Diabetes REduction Assessment with ramipril and rosiglitazone Medication (DREAM) study.
  6. Observational study of gene-disease association. (HuGE Navigator)
    Title: Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.
  7. Clinical trial of gene-disease association and gene-environment interaction. (HuGE Navigator)
    Title: Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score.
  8. Geneic mapping of GA3 to chromosome 7 and identification of mutations in c7orf10 are reported.
    Title: Genetic mapping of glutaric aciduria, type 3, to chromosome 7 and identification of mutations in c7orf10.
Submit: New GeneRIF Correction

Phenotypes

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Review eQTL and phenotype association data in this region using PheGenI

Associated conditions

Description Tests
Glutaryl-CoA oxidase deficiency
MedGen: C0342873 OMIM: 231690 GeneReviews: Not available
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EBI GWAS Catalog

Description
Genome-wide meta-analysis identifies new susceptibility loci for migraine.
EBI GWAS Catalog

Variation

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See variants in ClinVar

See studies and variants in dbVar

See Variation Viewer (GRCh37.p13)

See Variation Viewer (GRCh38)

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Clone Names

  • FLJ11808, FLJ53872

Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables succinate-hydroxymethylglutarate CoA-transferase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables succinate-hydroxymethylglutarate CoA-transferase activity IDA
Inferred from Direct Assay
more info
 PubMed 
Component Evidence Code Pubs
located_in mitochondrion HTP PubMed 
is_active_in mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in mitochondrion IDA
Inferred from Direct Assay
more info
 PubMed 

General protein information

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Preferred Names
succinate--hydroxymethylglutarate CoA-transferase
Names
Russel-Silver syndrome candidate
dermal papilla-derived protein 13
succinylCoA:glutarate-CoA transferase
NP_001180240.2
NP_001180241.2
NP_001180242.2
NP_079004.2
XP_006715838.2
XP_011513827.2
XP_011513828.1
XP_011513829.2
XP_011513831.2
XP_011513832.2
XP_016868110.1
XP_016868111.2
XP_047276796.1
XP_047276797.1
XP_047276798.1
XP_047276799.1
XP_054214984.1
XP_054214985.1
XP_054214986.1
XP_054214987.1
XP_054214988.1
XP_054214989.1
XP_054214990.1
XP_054214991.1
XP_054214992.1
XP_054214993.1
XP_054214994.1

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_023422.3 RefSeqGene

    Range
    5002..730760
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001193311.2 → NP_001180240.2  succinate--hydroxymethylglutarate CoA-transferase isoform 1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (1) represents the longest transcript and encodes the longest isoform (1).
    Source sequence(s)
    AC005160, AK296048, BC098318
    Consensus CDS
    CCDS55104.2
    Related
    ENSP00000486291.2, ENST00000628514.3
    Conserved Domains (1) summary
    COG1804
    Location:39 → 458
    CaiB; Crotonobetainyl-CoA:carnitine CoA-transferase CaiB and related acyl-CoA transferases [Lipid transport and metabolism]

  2. NM_001193312.2 → NP_001180241.2  succinate--hydroxymethylglutarate CoA-transferase isoform 2

    Status: REVIEWED

    Description
    Transcript Variant: This variant (2) lacks two in-frame exons in the coding region, compared to variant 1. This variant encodes isoform 2, which is shorter than isoform 1.
    Source sequence(s)
    AC005160, BC098310
    Consensus CDS
    CCDS55106.2
    Related
    ENSP00000385222.3, ENST00000401647.7
    Conserved Domains (1) summary
    COG1804
    Location:39 → 384
    CaiB; Crotonobetainyl-CoA:carnitine CoA-transferase CaiB and related acyl-CoA transferases [Lipid transport and metabolism]

  3. NM_001193313.2 → NP_001180242.2  succinate--hydroxymethylglutarate CoA-transferase isoform 3

    Status: REVIEWED

    Description
    Transcript Variant: This variant (3) lacks an in-frame exon in the coding region, compared to variant 1. This variant encodes isoform 3, which is shorter than isoform 1.
    Source sequence(s)
    AC005160, AK021870, AK296048
    Consensus CDS
    CCDS55105.2
    UniProtKB/Swiss-Prot
    A4D1W5, B4DJF0, B4DR73, Q4KMW4, Q4KMW8, Q4KMZ0, Q8TE00, Q8TEY1, Q9HAC7
    UniProtKB/TrEMBL
    H0Y4N1
    Related
    ENSP00000338475.5, ENST00000335693.9
    Conserved Domains (1) summary
    COG1804
    Location:39 → 432
    CaiB; Crotonobetainyl-CoA:carnitine CoA-transferase CaiB and related acyl-CoA transferases [Lipid transport and metabolism]

  4. NM_024728.3 → NP_079004.2  succinate--hydroxymethylglutarate CoA-transferase isoform 4

    Status: REVIEWED

    Description
    Transcript Variant: This variant (4) has multiple differences in the coding region but maintains the reading frame, compared to variant 1. This variant encodes isoform 4, which is shorter than isoform 1.
    Source sequence(s)
    AC005160, AK021870, AK299133
    Conserved Domains (1) summary
    COG1804
    Location:39 → 421
    CaiB; Crotonobetainyl-CoA:carnitine CoA-transferase CaiB and related acyl-CoA transferases [Lipid transport and metabolism]

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2024_08

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p14 Primary Assembly

Genomic

  1. NC_000007.14 Reference GRCh38.p14 Primary Assembly

    Range
    40135005..41038816
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_047420840.1 → XP_047276796.1  succinate--hydroxymethylglutarate CoA-transferase isoform X4

  2. XM_047420843.1 → XP_047276799.1  succinate--hydroxymethylglutarate CoA-transferase isoform X11

  3. XM_011515525.4 → XP_011513827.2  succinate--hydroxymethylglutarate CoA-transferase isoform X1

  4. XM_006715775.4 → XP_006715838.2  succinate--hydroxymethylglutarate CoA-transferase isoform X2

  5. XM_011515527.4 → XP_011513829.2  succinate--hydroxymethylglutarate CoA-transferase isoform X6

  6. XM_011515529.4 → XP_011513831.2  succinate--hydroxymethylglutarate CoA-transferase isoform X9

  7. XM_047420841.1 → XP_047276797.1  succinate--hydroxymethylglutarate CoA-transferase isoform X8

  8. XM_017012622.3 → XP_016868111.2  succinate--hydroxymethylglutarate CoA-transferase isoform X7

  9. XM_011515530.4 → XP_011513832.2  succinate--hydroxymethylglutarate CoA-transferase isoform X12

  10. XM_047420842.1 → XP_047276798.1  succinate--hydroxymethylglutarate CoA-transferase isoform X10

  11. XM_011515526.2 → XP_011513828.1  succinate--hydroxymethylglutarate CoA-transferase isoform X3

    Conserved Domains (1) summary
    COG1804
    Location:20 → 465
    CaiB; Crotonobetainyl-CoA:carnitine CoA-transferase CaiB and related acyl-CoA transferases [Lipid transport and metabolism]

  12. XM_017012621.1 → XP_016868110.1  succinate--hydroxymethylglutarate CoA-transferase isoform X5

RNA

  1. XR_007060157.1 RNA Sequence

  2. XR_007060158.1 RNA Sequence

  3. XR_007060156.1 RNA Sequence

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060931.1 Alternate T2T-CHM13v2.0

    Range
    40292462..41196673
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_054359018.1 → XP_054214993.1  succinate--hydroxymethylglutarate CoA-transferase isoform X11

  2. XM_054359009.1 → XP_054214984.1  succinate--hydroxymethylglutarate CoA-transferase isoform X1

  3. XM_054359010.1 → XP_054214985.1  succinate--hydroxymethylglutarate CoA-transferase isoform X2

  4. XM_054359013.1 → XP_054214988.1  succinate--hydroxymethylglutarate CoA-transferase isoform X6

  5. XM_054359016.1 → XP_054214991.1  succinate--hydroxymethylglutarate CoA-transferase isoform X9

  6. XM_054359015.1 → XP_054214990.1  succinate--hydroxymethylglutarate CoA-transferase isoform X8

  7. XM_054359014.1 → XP_054214989.1  succinate--hydroxymethylglutarate CoA-transferase isoform X7

  8. XM_054359019.1 → XP_054214994.1  succinate--hydroxymethylglutarate CoA-transferase isoform X12

  9. XM_054359017.1 → XP_054214992.1  succinate--hydroxymethylglutarate CoA-transferase isoform X10

  10. XM_054359011.1 → XP_054214986.1  succinate--hydroxymethylglutarate CoA-transferase isoform X3

  11. XM_054359012.1 → XP_054214987.1  succinate--hydroxymethylglutarate CoA-transferase isoform X5

RNA

  1. XR_008487756.1 RNA Sequence

  2. XR_008487757.1 RNA Sequence

  3. XR_008487755.1 RNA Sequence

Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q9HAC7.3 GenPept UniProtKB/Swiss-Prot:Q9HAC7

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