L2hgdh L-2-hydroxyglutarate dehydrogenase [Rattus norvegicus (Norway rat)] - Gene

Summary

Official Symbol: L2hgdhprovided by RGD
Official Full Name: L-2-hydroxyglutarate dehydrogenaseprovided by RGD
Primary source: RGD:1306250
See related: Ensembl:ENSRNOG00000004857 AllianceGenome:RGD:1306250
Gene type: protein coding
RefSeq status: PROVISIONAL
Organism: Rattus norvegicus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
Also known as: RGD1306250
Summary: Predicted to enable 2-hydroxyglutarate dehydrogenase activity. Predicted to be involved in small molecule metabolic process. Predicted to be located in membrane and mitochondrion. Human ortholog(s) of this gene implicated in 2-hydroxyglutaric aciduria; L-2-hydroxyglutaric aciduria; cerebellar ataxia; and hereditary spastic paraplegia. Orthologous to human L2HGDH (L-2-hydroxyglutarate dehydrogenase). [provided by Alliance of Genome Resources, Jul 2025]
Expression: Biased expression in Kidney (RPKM 57.7), Heart (RPKM 55.7) and 9 other tissues See more
Orthologs: all
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Genomic context

Location:: 6q24

Exon count:: 11

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCr8 (GCF_036323735.1)	6	NC_086024.1 (93900427..93941534, complement)
RS_2023_06	previous assembly	mRatBN7.2 (GCF_015227675.2)	6	NC_051341.1 (88164429..88205585, complement)
106	previous assembly	Rnor_6.0 (GCF_000001895.5)	6	NC_005105.4 (92016560..92057643, complement)

Chromosome 6 - NC_086024.1 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages
Description: 320 RNA samples isolated from 11 organs (adrenal gland, brain, heart, kidney, liver, lung, muscle, spleen, thymus, and testes or uterus) from both sexes of Fischer 344 rats across four developmental stages (2-, 6-, 21-, and 104-weeks-old)
BioProject: PRJNA238328
Publication: PMID 24510058
Analysis date: Mon Jun 6 17:44:12 2016

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

L-2-hydroxyglutarate accumulates as a result of a deficiency in FAD-linked L-2-hydroxyglutarate dehydrogenase. mitochondrial L-malate dehydrogenase is responsible for the excretion of L-2-hydroxyglutarate in this condition.
Title: L-2-hydroxyglutaric aciduria, a defect of metabolite repair.

Submit: New GeneRIF Correction

General gene information

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Gene Ontology Provided by RGD

Function	Evidence Code	Pubs
enables (S)-2-hydroxyglutarate dehydrogenase activity	IBA Inferred from Biological aspect of Ancestor more info
enables (S)-2-hydroxyglutarate dehydrogenase activity	IEA Inferred from Electronic Annotation more info
enables (S)-2-hydroxyglutarate dehydrogenase activity	ISO Inferred from Sequence Orthology more info
enables oxidoreductase activity, acting on CH-OH group of donors	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in small molecule metabolic process	IBA Inferred from Biological aspect of Ancestor more info

Component	Evidence Code	Pubs
located_in membrane	IEA Inferred from Electronic Annotation more info
located_in membrane	ISO Inferred from Sequence Orthology more info
is_active_in mitochondrial inner membrane	IEA Inferred from Electronic Annotation more info
is_active_in mitochondrial inner membrane	ISO Inferred from Sequence Orthology more info
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IEA Inferred from Electronic Annotation more info
located_in mitochondrion	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: L-2-hydroxyglutarate dehydrogenase, mitochondrial

NP_001101498.1

EC 1.1.99.2

XP_038968180.1

EC 1.1.99.2

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_001108028.1 → NP_001101498.1 L-2-hydroxyglutarate dehydrogenase, mitochondrial

See identical proteins and their annotated locations for NP_001101498.1

Status: PROVISIONAL

Source sequence(s)

CH473947

UniProtKB/TrEMBL

A6HBX1, D3ZVS2

Related

ENSRNOP00000006473.4, ENSRNOT00000006473.6

Conserved Domains (2) summary

PRK11728
Location:61 → 457

PRK11728; hydroxyglutarate oxidase; Provisional

cl21454
Location:49 → 448

NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

RefSeqs of Annotated Genomes: GCF_036323735.1-RS_2024_02

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

Reference GRCr8

Genomic

NC_086024.1 Reference GRCr8

Range

93900427..93941534 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_039112252.2 → XP_038968180.1 L-2-hydroxyglutarate dehydrogenase, mitochondrial isoform X1

Conserved Domains (1) summary

PRK11728
Location:61 → 180

PRK11728; L-2-hydroxyglutarate oxidase