MDH2 (malate dehydrogenase 2)

2015-05-01  

Identity

HGNC
LOCATION
7q11.23
LOCUSID
ALIAS
DEE51,EIEE51,M-MDH,MDH,MGC:3559,MOR1
FUSION GENES

Other Information

Locus ID:

NCBI: 4191
MIM: 154100
HGNC: 6971
Ensembl: ENSG00000146701

Variants:

dbSNP: 4191
ClinVar: 4191
TCGA: ENSG00000146701
COSMIC: MDH2

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000146701ENST00000315758P40926
ENSG00000146701ENST00000315758A0A024R4K3
ENSG00000146701ENST00000432020P40926
ENSG00000146701ENST00000443006G3XAL0
ENSG00000146701ENST00000461263U3KQ63

Expression (GTEx)

0
50
100
150
200
250
300
350
400

Pathways

PathwaySourceExternal ID
Citrate cycle (TCA cycle)KEGGko00020
Pyruvate metabolismKEGGko00620
Glyoxylate and dicarboxylate metabolismKEGGko00630
Citrate cycle (TCA cycle)KEGGhsa00020
Pyruvate metabolismKEGGhsa00620
Glyoxylate and dicarboxylate metabolismKEGGhsa00630
Cysteine and methionine metabolismKEGGko00270
Cysteine and methionine metabolismKEGGhsa00270
Metabolic pathwaysKEGGhsa01100
Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetateKEGGhsa_M00011
Citrate cycle (TCA cycle, Krebs cycle)KEGGM00009
Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetateKEGGM00011
Carbon metabolismKEGGhsa01200
Carbon metabolismKEGGko01200
Citrate cycle (TCA cycle, Krebs cycle)KEGGhsa_M00009
MetabolismREACTOMER-HSA-1430728
Metabolism of carbohydratesREACTOMER-HSA-71387
Glucose metabolismREACTOMER-HSA-70326
GluconeogenesisREACTOMER-HSA-70263
The citric acid (TCA) cycle and respiratory electron transportREACTOMER-HSA-1428517
Pyruvate metabolism and Citric Acid (TCA) cycleREACTOMER-HSA-71406
Citric acid cycle (TCA cycle)REACTOMER-HSA-71403

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
347186102022Gain of Function of Malate Dehydrogenase 2 and Familial Hyperglycemia.4
347666282022Bi-allelic variants in MDH2: Expanding the clinical phenotype.3
361390142022Structural Comparison of hMDH2 Complexed with Natural Substrates and Cofactors: The Importance of Phosphate Binding for Active Conformation and Catalysis.0
361598202022Integrated genomic, transcriptomic and metabolomic analysis reveals MDH2 mutation-induced metabolic disorder in recurrent focal segmental glomerulosclerosis.0
347186102022Gain of Function of Malate Dehydrogenase 2 and Familial Hyperglycemia.4
347666282022Bi-allelic variants in MDH2: Expanding the clinical phenotype.3
361390142022Structural Comparison of hMDH2 Complexed with Natural Substrates and Cofactors: The Importance of Phosphate Binding for Active Conformation and Catalysis.0
361598202022Integrated genomic, transcriptomic and metabolomic analysis reveals MDH2 mutation-induced metabolic disorder in recurrent focal segmental glomerulosclerosis.0
335656872021Urinary malate dehydrogenase 2 is a new biomarker for early detection of non-small-cell lung cancer.13
335656872021Urinary malate dehydrogenase 2 is a new biomarker for early detection of non-small-cell lung cancer.13
300084762018Role of MDH2 pathogenic variant in pheochromocytoma and paraganglioma patients.25
300084762018Role of MDH2 pathogenic variant in pheochromocytoma and paraganglioma patients.25
279893242017Mutations in MDH2, Encoding a Krebs Cycle Enzyme, Cause Early-Onset Severe Encephalopathy.35
284337112017MDH2 is an RNA binding protein involved in downregulation of sodium channel Scn1a expression under seizure condition.8
279893242017Mutations in MDH2, Encoding a Krebs Cycle Enzyme, Cause Early-Onset Severe Encephalopathy.35

Citation

Dessen P

MDH2 (malate dehydrogenase 2)

Atlas Genet Cytogenet Oncol Haematol. 2015-05-01

Online version: http://atlasgeneticsoncology.org/gene/55198/mdh2-(malate-dehydrogenase-2)