L2HGDH (L-2-hydroxyglutarate dehydrogenase)

2008-12-01  

Identity

HGNC
LOCATION
14q21.3
LOCUSID
ALIAS
C14orf160,L2HGA
FUSION GENES

Other Information

Locus ID:

NCBI: 79944
MIM: 609584
HGNC: 20499
Ensembl: ENSG00000087299

Variants:

dbSNP: 79944
ClinVar: 79944
TCGA: ENSG00000087299
COSMIC: L2HGDH

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000087299ENST00000261699C9JVN9
ENSG00000087299ENST00000267436Q9H9P8
ENSG00000087299ENST00000421284Q9H9P8
ENSG00000087299ENST00000554191G3V3E2
ENSG00000087299ENST00000555423G3V272
ENSG00000087299ENST00000555610G3V5S1

Expression (GTEx)

0
1
2
3
4
5
6
7
8
9

Pathways

PathwaySourceExternal ID
Butanoate metabolismKEGGko00650
Butanoate metabolismKEGGhsa00650
MetabolismREACTOMER-HSA-1430728
The citric acid (TCA) cycle and respiratory electron transportREACTOMER-HSA-1428517
Pyruvate metabolism and Citric Acid (TCA) cycleREACTOMER-HSA-71406
Interconversion of 2-oxoglutarate and 2-hydroxyglutarateREACTOMER-HSA-880009

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
373787532023Loss of function variants in L2HGDH gene causing L-2-hydroxyglutaric aciduria.0
373787532023Loss of function variants in L2HGDH gene causing L-2-hydroxyglutaric aciduria.0
321016992020MYC Regulation of D2HGDH and L2HGDH Influences the Epigenome and Epitranscriptome.12
321016992020MYC Regulation of D2HGDH and L2HGDH Influences the Epigenome and Epitranscriptome.12
294583342018Identification of novel L2HGDH mutation in a large consanguineous Pakistani family- a case report.7
299808732018A novel compound heterozygous mutation of the L2HGDH gene in a Chinese boy with L-2-hydroxyglutaric aciduria: case report and literature review.3
302171882018Two novel L2HGDH mutations identified in a rare Chinese family with L-2-hydroxyglutaric aciduria.6
294583342018Identification of novel L2HGDH mutation in a large consanguineous Pakistani family- a case report.7
299808732018A novel compound heterozygous mutation of the L2HGDH gene in a Chinese boy with L-2-hydroxyglutaric aciduria: case report and literature review.3
302171882018Two novel L2HGDH mutations identified in a rare Chinese family with L-2-hydroxyglutaric aciduria.6
268297332016[Analysis of L2HGDH gene mutation in a patient with 2-hydroxyglutaric aciduria].2
268297332016[Analysis of L2HGDH gene mutation in a patient with 2-hydroxyglutaric aciduria].2
245730902014Founder effect confirmation of c.241A>G mutation in the L2HGDH gene and characterization of oxidative stress parameters in six Tunisian families with L-2-hydroxyglutaric aciduria.8
246066022014Elevated plasma 2-hydroxyglutarate in acute myeloid leukaemia: association with the IDH1 SNP rs11554137 and severe renal impairment.1
245730902014Founder effect confirmation of c.241A>G mutation in the L2HGDH gene and characterization of oxidative stress parameters in six Tunisian families with L-2-hydroxyglutaric aciduria.8

Citation

Dessen P

L2HGDH (L-2-hydroxyglutarate dehydrogenase)

Atlas Genet Cytogenet Oncol Haematol. 2008-12-01

Online version: http://atlasgeneticsoncology.org/gene/50477/l2hgdh