Ogdh oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Ogdhprovided by MGI
Official Full Name: oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)provided by MGI
Primary source: MGI:MGI:1098267
See related: Ensembl:ENSMUSG00000020456 AllianceGenome:MGI:1098267
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: E1o; d1401; OGDH-E1; mKIAA4192; 2210403E04Rik; 2210412K19Rik
Summary: Enables oxoglutarate dehydrogenase (NAD+) activity. Involved in brain development. Located in mitochondrion. Part of oxoglutarate dehydrogenase complex. Is expressed in several structures, including alimentary system; genitourinary system; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in oxoglutarate dehydrogenase deficiency. Orthologous to human OGDH (oxoglutarate dehydrogenase). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Broad expression in heart adult (RPKM 260.2), kidney adult (RPKM 89.4) and 22 other tissues See more
Orthologs: human all
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Genomic context

Location:: 11 A1; 11 3.94 cM

Exon count:: 27

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	11	NC_000077.7 (6241597..6309094)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	11	NC_000077.6 (6291597..6359094)

Chromosome 11 - NC_000077.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Selective NADH communication from alpha-ketoglutarate dehydrogenase to mitochondrial transhydrogenase prevents reactive oxygen species formation under reducing conditions in the heart.
Title: Selective NADH communication from α-ketoglutarate dehydrogenase to mitochondrial transhydrogenase prevents reactive oxygen species formation under reducing conditions in the heart.
Triheptanoin alters [U-(13)C6]-glucose incorporation into glycolytic intermediates and increases TCA cycling by normalizing the activities of pyruvate dehydrogenase and oxoglutarate dehydrogenase in a chronic epilepsy mouse model.
Title: Triheptanoin alters [U-(13)C(6)]-glucose incorporation into glycolytic intermediates and increases TCA cycling by normalizing the activities of pyruvate dehydrogenase and oxoglutarate dehydrogenase in a chronic epilepsy mouse model.
chromatin immunoprecipitation-deep sequencing, confirmed the predominant association of OGDH and ACAA2 with H2A.Z-occupied transcription start sites and enhancers, the former of which we confirmed is conserved in both mouse and human tissue
Title: Oxoglutarate dehydrogenase and acetyl-CoA acyltransferase 2 selectively associate with H2A.Z-occupied promoters and are required for histone modifications.
We propose that D2-HG promotes cardiac dysfunction by impairing alpha-ketoglutarate dehydrogenase and induces histone modifications in an ACL-dependent manner
Title: Oncometabolite d-2-hydroxyglutarate impairs α-ketoglutarate dehydrogenase and contractile function in rodent heart.
Taken together, our results indicate that the redox sensing and ROS forming properties of Pdh and Ogdh are linked to S-glutathionylation
Title: Protein S-glutathionylation alters superoxide/hydrogen peroxide emission from pyruvate dehydrogenase complex.
pre-incubation of permeabilized liver mitochondria from mouse depleted of GSH showed an approximately ~3.5-fold increase in Ogdh-mediated O2(-)/H2O2 production that was matched by a significant decrease in NADH formation which could be reversed by Grx2. Taken together, our results demonstrate GSH and GSSG modulate ROS production by Ogdh through S-glutathionylation of different subunits
Title: Induction of mitochondrial reactive oxygen species production by GSH mediated S-glutathionylation of 2-oxoglutarate dehydrogenase.
The nitration degree of alpha-OGDH for diabetic mouse is higher than that for control mouse, indicating that alpha-OGDH of the diabetic mouse suffered from more intense oxidative damage.
Title: A novel targeted proteomics method for identification and relative quantitation of difference in nitration degree of OGDH between healthy and diabetic mouse.
Early detection in thiamine deficiency induce changes in the transcription of this enzyme in brain mitochondria.
Title: Responses of the mitochondrial alpha-ketoglutarate dehydrogenase complex to thiamine deficiency may contribute to regional selective vulnerability.
In the postnatal mouse brain,high mRNA levels of d1401/Ogdh were observed in the olfactory bulb, hippocampus, cerebellum, and pons.
Title: Identification and mRNA expression of Ogdh, QP-C, and two predicted genes in the postnatal mouse brain.
Alpha-ketoglutarate dehydrogenase is a primary site of ROS production in normally functioning mitochondria.
Title: Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (3)
Targeted (2) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

AFMB031WC9 (e-PCR)
- UniSTS:28199

D9Mit34.1 (e-PCR), detects polymorphism
- UniSTS:131903

D10Mit157.2 (e-PCR), detects polymorphism
- UniSTS:126061

RH124874 (e-PCR)
- UniSTS:161696

Ogdh (e-PCR), detects polymorphism
- UniSTS:507109

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables heat shock protein binding	ISO Inferred from Sequence Orthology more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables oxoglutarate dehydrogenase (NAD+) activity	IDA Inferred from Direct Assay more info	PubMed
enables oxoglutarate dehydrogenase (succinyl-transferring) activity	IBA Inferred from Biological aspect of Ancestor more info
enables oxoglutarate dehydrogenase (succinyl-transferring) activity	ISO Inferred from Sequence Orthology more info
enables oxoglutarate dehydrogenase (succinyl-transferring) activity	ISS Inferred from Sequence or Structural Similarity more info
enables protein-folding chaperone binding	ISO Inferred from Sequence Orthology more info
enables thiamine pyrophosphate binding	ISO Inferred from Sequence Orthology more info
enables thiamine pyrophosphate binding	ISS Inferred from Sequence or Structural Similarity more info

Process	Evidence Code	Pubs
involved_in 2-oxoglutarate metabolic process	ISO Inferred from Sequence Orthology more info
involved_in 2-oxoglutarate metabolic process	ISS Inferred from Sequence or Structural Similarity more info
involved_in NADH metabolic process	ISO Inferred from Sequence Orthology more info
involved_in cerebellar cortex development	IEP Inferred from Expression Pattern more info	PubMed
involved_in generation of precursor metabolites and energy	ISS Inferred from Sequence or Structural Similarity more info
involved_in glycolytic process	IEA Inferred from Electronic Annotation more info
involved_in hippocampus development	IEP Inferred from Expression Pattern more info	PubMed
involved_in olfactory bulb mitral cell layer development	IEP Inferred from Expression Pattern more info	PubMed
involved_in pyramidal neuron development	IEP Inferred from Expression Pattern more info	PubMed
involved_in striatum development	IEP Inferred from Expression Pattern more info	PubMed
involved_in succinyl-CoA metabolic process	ISO Inferred from Sequence Orthology more info
involved_in succinyl-CoA metabolic process	ISS Inferred from Sequence or Structural Similarity more info
involved_in tangential migration from the subventricular zone to the olfactory bulb	IEP Inferred from Expression Pattern more info	PubMed
involved_in thalamus development	IEP Inferred from Expression Pattern more info	PubMed
involved_in tricarboxylic acid cycle	IBA Inferred from Biological aspect of Ancestor more info
involved_in tricarboxylic acid cycle	ISO Inferred from Sequence Orthology more info

Component	Evidence Code	Pubs
located_in mitochondrial membrane	ISS Inferred from Sequence or Structural Similarity more info
located_in mitochondrion	HDA more info	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
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in nucleus	ISO Inferred from Sequence Orthology more info
located_in nucleus	ISS Inferred from Sequence or Structural Similarity more info
part_of oxoglutarate dehydrogenase complex	IBA Inferred from Biological aspect of Ancestor more info
part_of oxoglutarate dehydrogenase complex	IMP Inferred from Mutant Phenotype more info	PubMed
part_of oxoglutarate dehydrogenase complex	ISO Inferred from Sequence Orthology more info
part_of oxoglutarate dehydrogenase complex	ISS Inferred from Sequence or Structural Similarity more info

General protein information

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Preferred Names: 2-oxoglutarate dehydrogenase complex component E1

Names: 2-oxoglutarate dehydrogenase, mitochondrial; OGDC-E1; alpha-KGDH-E1; alpha-ketoglutarate dehydrogenase; oxoglutarate dehydrogenase (lipoamide); thiamine diphosphate (ThDP)-dependent 2-oxoglutarate dehydrogenase

NP_001239211.1

EC 1.2.4.2

NP_001239212.1

EC 1.2.4.2

NP_001239216.1

EC 1.2.4.2

NP_001239217.1

EC 1.2.4.2

NP_001348831.1

EC 1.2.4.2

NP_001348832.1

EC 1.2.4.2

NP_001348833.1

EC 1.2.4.2

NP_001348834.1

EC 1.2.4.2

NP_035086.2

EC 1.2.4.2

XP_006514645.1

EC 1.2.4.2

XP_006514647.1

EC 1.2.4.2

XP_030101508.1

EC 1.2.4.2

XP_030101509.1

EC 1.2.4.2

XP_030101510.1

EC 1.2.4.2

XP_030101511.1

EC 1.2.4.2

XP_030101512.1

EC 1.2.4.2

XP_036012306.1

EC 1.2.4.2

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.

mRNA and Protein(s)

NM_001252282.1 → NP_001239211.1 2-oxoglutarate dehydrogenase complex component E1 isoform 1

Status: VALIDATED

Description

Transcript Variant: This variant (1) encodes the longest isoform (1).

Source sequence(s)

AK147289, AK169286, AK220536, AL607152

UniProtKB/Swiss-Prot

Q60597

Conserved Domains (5) summary

PRK09404
Location:49 → 1030

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

cd02016
Location:279 → 542

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:664 → 879

Transket_pyr; Transketolase, pyrimidine binding domain

pfam16078
Location:47 → 82

2-oxogl_dehyd_N; 2-oxoglutarate dehydrogenase N-terminus

pfam16870
Location:886 → 1029

OxoGdeHyase_C; 2-oxoglutarate dehydrogenase C-terminal
NM_001252283.1 → NP_001239212.1 2-oxoglutarate dehydrogenase complex component E1 isoform 2

See identical proteins and their annotated locations for NP_001239212.1

Status: VALIDATED

Description

Transcript Variant: This variant (2) has an alternate coding exon, compared to variant 1. The resulting isoform (2) has a shorter and different internal segment, compared to isoform 1. Variants 2, 6 and 7 encode the same isoform (2).

Source sequence(s)

AK147289, AL607152, BC057354

Consensus CDS

CCDS56758.1

UniProtKB/Swiss-Prot

Q60597

Related

ENSMUSP00000091041.4, ENSMUST00000093350.10

Conserved Domains (1) summary

PRK09404
Location:49 → 1026

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
NM_001252287.2 → NP_001239216.1 2-oxoglutarate dehydrogenase complex component E1 isoform 3 precursor

See identical proteins and their annotated locations for NP_001239216.1

Status: VALIDATED

Description

Transcript Variant: This variant (3) differs in the 5' UTR and a region of the coding sequence, compared to variant 1. The resulting isoform (3) has a shorter and different internal segment, compared to isoform 1. Variants 3, 4 and 8 encode the same isoform (3).

Source sequence(s)

AK147289, AL607152, BY768178, CJ145669

Consensus CDS

CCDS36106.1

UniProtKB/Swiss-Prot

Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2

Related

ENSMUSP00000099090.3, ENSMUST00000101554.9

Conserved Domains (1) summary

PRK09404
Location:49 → 1015

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
NM_001252288.1 → NP_001239217.1 2-oxoglutarate dehydrogenase complex component E1 isoform 4

See identical proteins and their annotated locations for NP_001239217.1

Status: VALIDATED

Description

Transcript Variant: This variant (5) differs in the 5' UTR exon and lacks an in-frame coding exon, compared to variant 1. The resulting isoform (4) lacks an internal segment, compared to isoform 1. Variants 5 and 9 encode the same isoform (4).

Source sequence(s)

AK147289, AL607152, BC049104, BY782774, BY785860

Consensus CDS

CCDS56759.1

UniProtKB/TrEMBL

Z4YJV4

Related

ENSMUSP00000080569.5, ENSMUST00000081894.5

Conserved Domains (1) summary

PRK09404
Location:49 → 1011

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
NM_001361902.1 → NP_001348831.1 2-oxoglutarate dehydrogenase complex component E1 isoform 2

Status: VALIDATED

Source sequence(s)

AK147289, AL607152, BC049104, BC057354, BY125742

Consensus CDS

CCDS56758.1

Conserved Domains (1) summary

PRK09404
Location:49 → 1026

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
NM_001361903.1 → NP_001348832.1 2-oxoglutarate dehydrogenase complex component E1 isoform 2

Status: VALIDATED

Source sequence(s)

AK147289, AL607152, BC057354, CJ145669

Consensus CDS

CCDS56758.1

Conserved Domains (1) summary

PRK09404
Location:49 → 1026

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
NM_001361904.1 → NP_001348833.1 2-oxoglutarate dehydrogenase complex component E1 isoform 3 precursor

Status: VALIDATED

Source sequence(s)

AK147289, AL607152, BY768178, CK620645

Consensus CDS

CCDS36106.1

UniProtKB/Swiss-Prot

Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2

Conserved Domains (1) summary

PRK09404
Location:49 → 1015

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
NM_001361905.1 → NP_001348834.1 2-oxoglutarate dehydrogenase complex component E1 isoform 4

Status: VALIDATED

Source sequence(s)

AK147289, AL607152, BB842096, BC049104

Consensus CDS

CCDS56759.1

UniProtKB/TrEMBL

Z4YJV4

Conserved Domains (1) summary

PRK09404
Location:49 → 1011

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
NM_010956.4 → NP_035086.2 2-oxoglutarate dehydrogenase complex component E1 isoform 3 precursor

See identical proteins and their annotated locations for NP_035086.2

Status: VALIDATED

Description

Transcript Variant: This variant (4) differs in a region of the coding sequence, compared to variant 1. The resulting isoform (3) has a shorter and different internal segment, compared to isoform 1. Variants 3 and 4 encode the same isoform 3.

Source sequence(s)

AK147289, AL607152

Consensus CDS

CCDS36106.1

UniProtKB/Swiss-Prot

Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2

Related

ENSMUSP00000003461.9, ENSMUST00000003461.15

Conserved Domains (1) summary

PRK09404
Location:49 → 1015

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

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

Reference GRCm39 C57BL/6J

Genomic

NC_000077.7 Reference GRCm39 C57BL/6J

Range

6241597..6309094

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_030245651.1 → XP_030101511.1 2-oxoglutarate dehydrogenase complex component E1 isoform X3

UniProtKB/TrEMBL

Z4YJV4

Conserved Domains (1) summary

PRK09404
Location:49 → 1011

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
XM_036156413.1 → XP_036012306.1 2-oxoglutarate dehydrogenase complex component E1 isoform X4

Conserved Domains (1) summary

PRK09404
Location:3 → 804

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
XM_006514582.3 → XP_006514645.1 2-oxoglutarate dehydrogenase complex component E1 isoform X1

See identical proteins and their annotated locations for XP_006514645.1

UniProtKB/Swiss-Prot

Q60597

Conserved Domains (1) summary

PRK09404
Location:49 → 1026

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
XM_030245650.1 → XP_030101510.1 2-oxoglutarate dehydrogenase complex component E1 isoform X3

UniProtKB/TrEMBL

Z4YJV4

Conserved Domains (1) summary

PRK09404
Location:49 → 1011

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
XM_030245649.2 → XP_030101509.1 2-oxoglutarate dehydrogenase complex component E1 isoform X2

UniProtKB/Swiss-Prot

Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2

Conserved Domains (1) summary

PRK09404
Location:49 → 1015

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
XM_006514584.4 → XP_006514647.1 2-oxoglutarate dehydrogenase complex component E1 isoform X1

See identical proteins and their annotated locations for XP_006514647.1

UniProtKB/Swiss-Prot

Q60597

Conserved Domains (1) summary

PRK09404
Location:49 → 1026

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
XM_030245652.1 → XP_030101512.1 2-oxoglutarate dehydrogenase complex component E1 isoform X3

UniProtKB/TrEMBL

Z4YJV4

Conserved Domains (1) summary

PRK09404
Location:49 → 1011

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
XM_030245648.1 → XP_030101508.1 2-oxoglutarate dehydrogenase complex component E1 isoform X2

UniProtKB/Swiss-Prot

Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2

Conserved Domains (1) summary

PRK09404
Location:49 → 1015

sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed