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OGDHL oxoglutarate dehydrogenase L [ Homo sapiens (human) ]

Gene ID: 55753, updated on 5-Mar-2024

Summary

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Official Symbol
OGDHLprovided by HGNC
Official Full Name
oxoglutarate dehydrogenase Lprovided by HGNC
Primary source
HGNC:HGNC:25590
See related
Ensembl:ENSG00000197444 MIM:617513; AllianceGenome:HGNC:25590
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
YOBELN
Summary
The protein encoded by this gene is similar to oxoglutarate dehydrogenase (OGDH) of the OGDH complex, which degrades glucose and glutamate. This gene encodes several isoforms, including some that appear to localize to mitochondria. The encoded protein down-regulates the AKT signaling cascade and can suppress the growth of cervical cancer cells. [provided by RefSeq, Dec 2016]
Expression
Biased expression in kidney (RPKM 82.8), liver (RPKM 20.1) and 3 other tissues See more
Orthologs
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Genomic context

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See OGDHL in Genome Data Viewer
Location:
10q11.23
Exon count:
25
Annotation release Status Assembly Chr Location
RS_2023_10 current GRCh38.p14 (GCF_000001405.40) 10 NC_000010.11 (49734641..49762379, complement)
RS_2023_10 current T2T-CHM13v2.0 (GCF_009914755.1) 10 NC_060934.1 (50583410..50612062, complement)
105.20220307 previous assembly GRCh37.p13 (GCF_000001405.25) 10 NC_000010.10 (50942687..50970425, complement)

Chromosome 10 - NC_000010.11Genomic Context describing neighboring genes Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr10:50819328-50820224 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr10:50820225-50821122 Neighboring gene solute carrier family 18 member A3 Neighboring gene choline O-acetyltransferase Neighboring gene H3K4me1 hESC enhancer GRCh37_chr10:50856078-50856929 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr10:50856930-50857780 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr10:50858633-50859483 Neighboring gene OCT4-NANOG hESC enhancer GRCh37_chr10:50897886-50898488 Neighboring gene OCT4-NANOG-H3K4me1 hESC enhancer GRCh37_chr10:50898489-50899090 Neighboring gene chromosome 10 open reading frame 53 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr10:50965991-50966502 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr10:50966503-50967012 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 2364 Neighboring gene mitogen-activated protein kinase 6 pseudogene 6 Neighboring gene ribosomal protein L21 pseudogene 89

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
  • 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. Silencing of OGDHL promotes liver cancer metastasis by enhancing hypoxia inducible factor 1 α protein stability. Dai W, et al. Cancer Sci, 2023 Apr. PMID 36000493, Free PMC Article
  2. OGDHL closely associates with tumor microenvironment and can serve as a prognostic biomarker for papillary thyroid cancer. Mao M, et al. Cancer Med, 2021 Jan. PMID 33405394, Free PMC Article
  3. OGDHL silencing promotes hepatocellular carcinoma by reprogramming glutamine metabolism. Dai W, et al. J Hepatol, 2020 May. PMID 31899205
  4. OGDHL Expression as a Prognostic Biomarker for Liver Cancer Patients. Jiao Y, et al. Dis Markers, 2019. PMID 31781311, Free PMC Article
  5. Aberrant hypermethylation of OGDHL gene promoter in sporadic colorectal cancer. Khalaj-Kondori M, et al. Curr Probl Cancer, 2020 Feb. PMID 30904169

See all (37) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Evaluating the association of biallelic OGDHL variants with significant phenotypic heterogeneity.
    Title: Evaluating the association of biallelic OGDHL variants with significant phenotypic heterogeneity.
  2. Silencing of OGDHL promotes liver cancer metastasis by enhancing hypoxia inducible factor 1 alpha protein stability.
    Title: Silencing of OGDHL promotes liver cancer metastasis by enhancing hypoxia inducible factor 1 α protein stability.
  3. Bi-allelic variants in OGDHL cause a neurodevelopmental spectrum disease featuring epilepsy, hearing loss, visual impairment, and ataxia.
    Title: Bi-allelic variants in OGDHL cause a neurodevelopmental spectrum disease featuring epilepsy, hearing loss, visual impairment, and ataxia.
  4. OGDHL silencing promotes hepatocellular carcinoma by reprogramming glutamine metabolism.
    Title: OGDHL silencing promotes hepatocellular carcinoma by reprogramming glutamine metabolism.
  5. OGDHL closely associates with tumor microenvironment and can serve as a prognostic biomarker for papillary thyroid cancer.
    Title: OGDHL closely associates with tumor microenvironment and can serve as a prognostic biomarker for papillary thyroid cancer.
  6. Aberrant hypermethylation of OGDHL gene promoter in sporadic colorectal cancer.
    Title: Aberrant hypermethylation of OGDHL gene promoter in sporadic colorectal cancer.
  7. A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis.
    Title: A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis.
  8. Authors identified 29 genes showing promising associations with breast cancer risk. Authors replicated the association for 2 genes, OGDHL and BRCA2, at a Bonferroni-corrected p < 0.05, by genotyping an independent set of samples from 1,628 breast cancer cases and 1,943 controls. The association for OGDHL was primarily driven by three deleterious variants (p.Val827Met, p.Pro839Leu, p.Phe836Ser; p < 0.01 for all).
    Title: Discovery of rare coding variants in OGDHL and BRCA2 in relation to breast cancer risk in Chinese women.
  9. Low OGDHL expression is associated with Hepatocellular Carcinoma.
    Title: OGDHL Expression as a Prognostic Biomarker for Liver Cancer Patients.
  10. inactivation of OGDHL can contribute to cervical tumorigenesis via activation of the AKT signaling pathway and thus support it as an important anti-proliferative gene in cervical cancer.
    Title: OGDHL is a modifier of AKT-dependent signaling and NF-κB function.
Submit: New GeneRIF Correction See all GeneRIFs (11)

Phenotypes

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Find tests for this gene in the NIH Genetic Testing Registry (GTR)

Review eQTL and phenotype association data in this region using PheGenI

Associated conditions

Description Tests
Yoon-Bellen neurodevelopmental syndrome
MedGen: C5562066 OMIM: 619701 GeneReviews: Not available
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Variation

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See variants in ClinVar

See studies and variants in dbVar

See Variation Viewer (GRCh37.p13)

See Variation Viewer (GRCh38)

Genotypes

Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables oxoglutarate dehydrogenase (succinyl-transferring) activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
enables thiamine pyrophosphate binding IEA
Inferred from Electronic Annotation
more info
  
Process Evidence Code Pubs
involved_in 2-oxoglutarate metabolic process ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in glycolytic process IEA
Inferred from Electronic Annotation
more info
  
involved_in tricarboxylic acid cycle IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in tricarboxylic acid cycle ISS
Inferred from Sequence or Structural Similarity
more info
  
Component Evidence Code Pubs
located_in mitochondrial matrix IDA
Inferred from Direct Assay
more info
 PubMed 
is_active_in mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
  
part_of oxoglutarate dehydrogenase complex IBA
Inferred from Biological aspect of Ancestor
more info
  

General protein information

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Preferred Names
2-oxoglutarate dehydrogenase-like, mitochondrial
Names
2-oxoglutarate dehydrogenase complex component E1-like
OGDC-E1-like
alpha-ketoglutarate dehydrogenase-like
oxoglutarate dehydrogenase like
NP_001137468.1
NP_001137469.1
NP_001334748.1
NP_001334749.1
NP_001334750.1
NP_001334751.1
NP_001334752.1
NP_001334753.1
NP_001334754.1
NP_001334755.1
NP_060715.2
XP_011538248.1
XP_047281422.1
XP_054222251.1
XP_054222252.1

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_052636.1 RefSeqGene

    Range
    5057..32739
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001143996.2 → NP_001137468.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform b

    See identical proteins and their annotated locations for NP_001137468.1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (2) lacks an alternate in-frame internal exon, compared to variant 1, resulting in a shorter isoform (b), compared to isoform a. Variants 2 and 5 both encode the same isoform (b).
    Source sequence(s)
    AK296551, BM504223, BQ549806, DA238469, DA411851
    Consensus CDS
    CCDS44390.1
    UniProtKB/Swiss-Prot
    Q9ULD0
    Related
    ENSP00000401356.1, ENST00000419399.4
    Conserved Domains (5) summary
    PRK09404
    Location:51 → 944
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:194 → 457
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:579 → 793
    Transket_pyr; Transketolase, pyrimidine binding domain
    pfam16078
    Location:51 → 68
    2-oxogl_dehyd_N; 2-oxoglutarate dehydrogenase N-terminus
    pfam16870
    Location:800 → 943
    OxoGdeHyase_C; 2-oxoglutarate dehydrogenase C-terminal

  2. NM_001143997.2 → NP_001137469.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform c

    See identical proteins and their annotated locations for NP_001137469.1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (3) lacks two alternate in-frame exons in the 5' coding region and uses a downstream start codon, compared to variant 1. Isoform c has a shorter N-terminus, compared to isoform a. Variants 3, 6, and 7 all encode the same isoform (c).
    Source sequence(s)
    AK303729, BP209348, BQ549806, DA416844
    Consensus CDS
    CCDS44391.1
    UniProtKB/Swiss-Prot
    Q9ULD0
    Related
    ENSP00000390240.1, ENST00000432695.2
    Conserved Domains (4) summary
    PRK09404
    Location:1 → 792
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:42 → 305
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:427 → 641
    Transket_pyr; Transketolase, pyrimidine binding domain
    pfam16870
    Location:648 → 791
    OxoGdeHyase_C; 2-oxoglutarate dehydrogenase C-terminal

  3. NM_001347819.1 → NP_001334748.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform a

    Status: REVIEWED

    Description
    Transcript Variant: This variant (4), as well as variant 1, encodes the longest isoform (a).
    Source sequence(s)
    AC069546
    Consensus CDS
    CCDS7234.1
    UniProtKB/Swiss-Prot
    A8K2G1, B4DKG2, B4E193, Q8TAN9, Q9NVA0, Q9ULD0

  4. NM_001347820.1 → NP_001334749.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform b

    Status: REVIEWED

    Description
    Transcript Variant: This variant (5), as well as variant 2, encodes isoform b.
    Source sequence(s)
    AC069546
    Consensus CDS
    CCDS44390.1
    UniProtKB/Swiss-Prot
    Q9ULD0

  5. NM_001347821.2 → NP_001334750.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform c

    Status: REVIEWED

    Description
    Transcript Variant: This variant (6), as well as variants 3 and 7, encodes isoform c.
    Source sequence(s)
    AC069546
    Consensus CDS
    CCDS44391.1
    UniProtKB/Swiss-Prot
    Q9ULD0

  6. NM_001347822.1 → NP_001334751.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform c

    Status: REVIEWED

    Description
    Transcript Variant: This variant (7), as well as variants 3 and 6, encodes isoform c.
    Source sequence(s)
    AC069546
    Consensus CDS
    CCDS44391.1
    UniProtKB/Swiss-Prot
    Q9ULD0

  7. NM_001347823.1 → NP_001334752.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform d

    Status: REVIEWED

    Description
    Transcript Variant: This variant (8), as well as variant 9, encodes isoform d.
    Source sequence(s)
    AC069546

  8. NM_001347824.2 → NP_001334753.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform d

    Status: REVIEWED

    Description
    Transcript Variant: This variant (9), as well as variant 8, encodes isoform d.
    Source sequence(s)
    AC069546

  9. NM_001347825.2 → NP_001334754.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform e

    Status: REVIEWED

    Source sequence(s)
    AC069546

  10. NM_001347826.1 → NP_001334755.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform f

    Status: REVIEWED

    Source sequence(s)
    AC069546

  11. NM_018245.3 → NP_060715.2  2-oxoglutarate dehydrogenase-like, mitochondrial isoform a

    See identical proteins and their annotated locations for NP_060715.2

    Status: REVIEWED

    Description
    Transcript Variant: This variant (1) encodes the longest isoform (a). Variants 1 and 4 both encode the same isoform (a).
    Source sequence(s)
    AB033116, BC026320, BQ549806, DA238469, DA411851
    Consensus CDS
    CCDS7234.1
    UniProtKB/Swiss-Prot
    A8K2G1, B4DKG2, B4E193, Q8TAN9, Q9NVA0, Q9ULD0
    Related
    ENSP00000363216.4, ENST00000374103.9
    Conserved Domains (5) summary
    PRK09404
    Location:51 → 1001
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:251 → 514
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:636 → 850
    Transket_pyr; Transketolase, pyrimidine binding domain
    pfam16078
    Location:51 → 76
    2-oxogl_dehyd_N; 2-oxoglutarate dehydrogenase N-terminus
    pfam16870
    Location:857 → 1000
    OxoGdeHyase_C; 2-oxoglutarate dehydrogenase C-terminal

RNA

  1. NR_144682.2 RNA Sequence

    Status: REVIEWED

    Source sequence(s)
    AC069546

  2. NR_144683.2 RNA Sequence

    Status: REVIEWED

    Source sequence(s)
    AC069546

  3. NR_144684.2 RNA Sequence

    Status: REVIEWED

    Source sequence(s)
    AC069546

  4. NR_144685.2 RNA Sequence

    Status: REVIEWED

    Source sequence(s)
    AC069546

  5. NR_144686.2 RNA Sequence

    Status: REVIEWED

    Source sequence(s)
    AC069546

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2023_10

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

Reference GRCh38.p14 Primary Assembly

Genomic

  1. NC_000010.11 Reference GRCh38.p14 Primary Assembly

    Range
    49734641..49762379 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_011539946.3 → XP_011538248.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform X1

    See identical proteins and their annotated locations for XP_011538248.1

    UniProtKB/Swiss-Prot
    A8K2G1, B4DKG2, B4E193, Q8TAN9, Q9NVA0, Q9ULD0
    Conserved Domains (5) summary
    PRK09404
    Location:51 → 1001
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:251 → 514
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:636 → 850
    Transket_pyr; Transketolase, pyrimidine binding domain
    pfam16078
    Location:51 → 76
    2-oxogl_dehyd_N; 2-oxoglutarate dehydrogenase N-terminus
    pfam16870
    Location:857 → 1000
    OxoGdeHyase_C; 2-oxoglutarate dehydrogenase C-terminal

  2. XM_047425466.1 → XP_047281422.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform X2

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060934.1 Alternate T2T-CHM13v2.0

    Range
    50583410..50612062 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_054366276.1 → XP_054222251.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform X1

    UniProtKB/Swiss-Prot
    A8K2G1, B4DKG2, B4E193, Q8TAN9, Q9NVA0, Q9ULD0

  2. XM_054366277.1 → XP_054222252.1  2-oxoglutarate dehydrogenase-like, mitochondrial isoform X2

Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q9ULD0.3 GenPept UniProtKB/Swiss-Prot:Q9ULD0

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