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Identification
HMDB Protein ID HMDBP00858
Secondary Accession Numbers
  • 6139
  • HMDBP03797
Name Malate dehydrogenase, mitochondrial
Synonyms Not Available
Gene Name MDH2
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific Function Not Available
Pathways
  • 2-ketoglutarate dehydrogenase complex deficiency
  • Citrate cycle (TCA cycle)
  • Citric Acid Cycle
  • Congenital lactic acidosis
  • Fructose-1,6-diphosphatase deficiency
  • Fumarase deficiency
  • Gluconeogenesis
  • Glutaminolysis and Cancer
  • Glycogen Storage Disease Type 1A (GSD1A) or Von Gierke Disease
  • Glycogenosis, Type IA. Von gierke disease
  • Glycogenosis, Type IB
  • Glycogenosis, Type IC
  • Glyoxylate and dicarboxylate metabolism
  • Malate-Aspartate Shuttle
  • Mitochondrial complex II deficiency
  • Phosphoenolpyruvate carboxykinase deficiency 1 (PEPCK1)
  • Pyruvate dehydrogenase deficiency (E2)
  • Pyruvate dehydrogenase deficiency (E3)
  • Pyruvate metabolism
  • The oncogenic action of 2-hydroxyglutarate
  • The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria
  • The Oncogenic Action of Fumarate
  • The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria
  • The Oncogenic Action of Succinate
  • Triosephosphate isomerase
Reactions
L-Malic acid + NAD → Oxalacetic acid + NADH details
L-Malic acid + NAD → Oxalacetic acid + NADH + Hydrogen Ion details
GO Classification
Biological Process
NADH metabolic process
malate metabolic process
cellular carbohydrate metabolic process
tricarboxylic acid cycle
gluconeogenesis
oxaloacetate metabolic process
internal protein amino acid acetylation
Cellular Component
mitochondrial matrix
plasma membrane
nucleus
mitochondrial inner membrane
Function
l-malate dehydrogenase activity
binding
catalytic activity
oxidoreductase activity, acting on the ch-oh group of donors, nad or nadp as acceptor
oxidoreductase activity, acting on ch-oh group of donors
oxidoreductase activity
malate dehydrogenase activity
Molecular Function
L-malate dehydrogenase activity
malate dehydrogenase (NADP+) activity
nucleotide binding
Process
metabolic process
primary metabolic process
carbohydrate metabolic process
cellular carbohydrate metabolic process
cellular metabolic process
organic acid metabolic process
oxoacid metabolic process
carboxylic acid metabolic process
oxidation reduction
dicarboxylic acid metabolic process
malate metabolic process
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location 7
Locus 7cen-q22
SNPs MDH2
Gene Sequence
>1017 bp
ATGCTCTCCGCCCTCGCCCGGCCTGTCAGCGCTGCTCTCCGCCGCAGCTTCAGCACCTCA
GCCCAGAACAATGCTAAAGTAGCTGTGCTAGGGGCCTCTGGAGGCATCGGGCAGCCACTT
TCACTTCTCCTGAAGAACAGCCCCTTGGTGAGCCGCCTGACCCTCTATGATATCGCGCAC
ACACCCGGAGTGGCCGCAGATCTGAGCCACATCGAGACCAAAGCCGCTGTGAAAGGCTAC
CTCGGACCTGAACAGCTGCCTGACTGCCTGAAAGGTTGTGATGTGGTAGTTATTCCGGCT
GGAGTCCCCAGAAAGCCAGGCATGACCCGGGACGACCTGTTCAACACCAATGCCACGATT
GTGGCCACCCTGACCGCTGCCTGTGCCCAGCACTGCCCGGAAGCCATGATCTGCGTCATT
GCCAATCCGGTTAATTCCACCATCCCCATCACAGCAGAAGTTTTCAAGAAGCATGGAGTG
TACAACCCCAACAAAATCTTCGGCGTGACGACCCTGGACATCGTCAGAGCCAACACCTTT
GTTGCAGAGCTGAAGGGTTTGGATCCAGCTCGAGTCAACGTCCCTGTCATTGGTGGCCAT
GCTGGGAAGACCATCATCCCCCTGATCTCTCAGTGCACCCCCAAGGTGGACTTTCCCCAG
GACCAGCTGACAGCACTCACTGGGCGGATCCAGGAGGCCGGCACGGAGGTGGTCAAGGCT
AAAGCCGGAGCAGGCTCTGCCACCCTCTCCATGGCGTATGCCGGCGCCCGCTTTGTCTTC
TCCCTTGTGGATGCAATGAATGGAAAGGAAGGTGTTGTGGAATGTTCCTTCGTTAAGTCA
CAGGAAACGGAATGTACCTACTTCTCCACACCGCTGCTGCTTGGGAAAAAGGGCATCGAG
AAGAACCTGGGCATCGGCAAAGTCTCCTCTTTTGAGGAGAAGATGATCTCGGATGCCATC
CCCGAGCTGAAGGCCTCCATCAAGAAGGGGGAAGATTTCGTGAAGACCCTGAAGTGA
Protein Properties
Number of Residues 338
Molecular Weight 35502.935
Theoretical pI 8.676
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Malate dehydrogenase, mitochondrial
MLSALARPASAALRRSFSTSAQNNAKVAVLGASGGIGQPLSLLLKNSPLVSRLTLYDIAH
TPGVAADLSHIETKAAVKGYLGPEQLPDCLKGCDVVVIPAGVPRKPGMTRDDLFNTNATI
VATLTAACAQHCPEAMICVIANPVNSTIPITAEVFKKHGVYNPNKIFGVTTLDIVRANTF
VAELKGLDPARVNVPVIGGHAGKTIIPLISQCTPKVDFPQDQLTALTGRIQEAGTEVVKA
KAGAGSATLSMAYAGARFVFSLVDAMNGKEGVVECSFVKSQETECTYFSTPLLLGKKGIE
KNLGIGKVSSFEEKMISDAIPELKASIKKGEDFVKTLK
GenBank ID Protein 2906146
UniProtKB/Swiss-Prot ID P40926
UniProtKB/Swiss-Prot Entry Name MDHM_HUMAN
PDB IDs
GenBank Gene ID AF047470
GeneCard ID MDH2
GenAtlas ID MDH2
HGNC ID HGNC:6971
References
General References
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