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Identification
HMDB Protein ID HMDBP00862
Secondary Accession Numbers
  • 6143
  • HMDBP06006
Name NAD-dependent malic enzyme, mitochondrial
Synonyms
  1. Malic enzyme 2
  2. NAD-ME
Gene Name ME2
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity
Specific Function Not Available
Pathways
  • Leigh Syndrome
  • Primary hyperoxaluria II, PH2
  • Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency)
  • Pyruvate Dehydrogenase Complex Deficiency
  • Pyruvate kinase deficiency
  • Pyruvate Metabolism
  • Pyruvate metabolism
Reactions
L-Malic acid + NAD → Pyruvic acid + CO(2) + NADH details
Oxalacetic acid → Pyruvic acid + CO(2) details
L-Malic acid + NAD → Pyruvic acid + Carbon dioxide + NADH + Hydrogen Ion details
GO Classification
Biological Process
malate metabolic process
Cellular Component
mitochondrial matrix
mitochondrion
Function
ion binding
cation binding
metal ion binding
binding
nucleotide binding
catalytic activity
nad or nadh binding
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
malic enzyme activity
Molecular Function
electron carrier activity
NAD binding
metal ion binding
malate dehydrogenase (decarboxylating) activity
malate dehydrogenase (oxaloacetate-decarboxylating) activity
Process
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 18
Locus 18q21
SNPs ME2
Gene Sequence
>1755 bp
ATGTTGTCCCGGTTAAGAGTAGTTTCCACCACTTGTACTTTGGCATGTCGACATTTGCAC
ATAAAAGAAAAAGGCAAGCCACTTATGCTGAACCCAAGAACAAACAAGGGAATGGCATTT
ACTTTACAAGAACGACAAATGCTTGGTCTTCAAGGACTTCTACCTCCCAAAATAGAGACA
CAAGATATTCAAGCCTTACGATTTCATAGAAACTTGAAGAAAATGACTAGCCCTTTGGAA
AAATATATCTACATAATGGGAATACAAGAAAGAAATGAGAAATTGTTTTATAGAATACTG
CAAGATGACATTGAGAGTTTAATGCCAATTGTATATACACCGACGGTTGGTCTTGCCTGC
TCCCAGTATGGACACATCTTTAGAAGACCTAAGGGATTATTTATTTCGATCTCAGACAGA
GGTCATGTTAGATCAATTGTGGATAACTGGCCAGAAAATCATGTTAAGGCTGTTGTAGTG
ACTGATGGAGAGAGAATTCTGGGTCTTGGAGATCTGGGTGTCTATGGAATGGGAATTCCA
GTAGGAAAACTTTGTTTGTATACAGCTTGTGCAGGAATACGGCCTGATAGATGCCTGCCA
GTGTGTATTGATGTGGGAACTGATAATATCGCACTCTTAAAAGACCCATTTTACATGGGC
TTGTACCAGAAACGAGATCGCACACAACAGTATGATGACCTGATTGATGAGTTTATGAAA
GCTATTACTGACAGATATGGCCGGAACACACTCATTCAGTTCGAAGACTTTGGAAATCAT
AATGCATTCAGGTTCTTGAGAAAGTACCGAGAAAAATATTGTACTTTCAATGATGATATT
CAAGGGACAGCTGCAGTAGCTCTAGCAGGTCTTCTTGCAGCACAAAAAGTTATTAGTAAA
CCAATCTCCGAACACAAAATCTTATTCCTTGGAGCAGGAGAGGCTGCTCTTGGAATTGCA
AATCTTATAGTTATGTCTATGGTAGAAAATGGCCTGTCAGAACAAGAGGCACAAAAGAAA
ATCTGGATGTTTGACAAGTATGGTTTATTAGTTAAGGGACGGAAAGCAAAAATAGATAGT
TATCAGGAACCATTTACTCACTCAGCCCCAGAGAGCATACCTGATACTTTTGAAGATGCA
GTGAATATACTGAAGCCTTCAACTATTATTGGAGTTGCAGGTGCTGGCCGTCTTTTCACT
CCTGATGTAATCAGAGCCATGGCCTCTATCAATGAAAGGCCTGTAATATTTGCATTAAGT
AATCCTACAGCACAGGCAGAGTGCACGGCTGAAGAAGCATATACACTTACAGAGGGCAGG
TGTTTGTTTGCCAGTGGCAGTCCATTTGGGCCAGTGAAACTTACAGATGGGCGAGTCTTT
ACACCAGGTCAAGGAAACAATGTTTATATTTTTCCAGGTGTGGCTTTAGCTGTTATTCTC
TGTAACACCCGGCATATTAGTGACAGTGTTTTCCTAGAAGCTGCAAAGGCCCTGACAAGC
CAATTGACAGATGAAGAGCTAGCCCAAGGGAGACTTTACCCACCGCTTGCTAATATTCAG
GAAGTTTCTATTAACATTGCTATTAAAGTTACAGAATACCTATATGCTAATAAAATGGCT
TTCCGATACCCAGAACCTGAAGACAAGGCCAAATATGTTAAAGAAAGAACATGGCGGAGT
GAATATGATTCCCTGCTGCCAGATGTGTATGAATGGCCAGAATCTGCATCAAGCCCTCCT
GTGATAACAGAATAG
Protein Properties
Number of Residues 584
Molecular Weight 53585.73
Theoretical pI 8.54
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>NAD-dependent malic enzyme, mitochondrial
MLSRLRVVSTTCTLACRHLHIKEKGKPLMLNPRTNKGMAFTLQERQMLGLQGLLPPKIET
QDIQALRFHRNLKKMTSPLEKYIYIMGIQERNEKLFYRILQDDIESLMPIVYTPTVGLAC
SQYGHIFRRPKGLFISISDRGHVRSIVDNWPENHVKAVVVTDGERILGLGDLGVYGMGIP
VGKLCLYTACAGIRPDRCLPVCIDVGTDNIALLKDPFYMGLYQKRDRTQQYDDLIDEFMK
AITDRYGRNTLIQFEDFGNHNAFRFLRKYREKYCTFNDDIQGTAAVALAGLLAAQKVISK
PISEHKILFLGAGEAALGIANLIVMSMVENGLSEQEAQKKIWMFDKYGLLVKGRKAKIDS
YQEPFTHSAPESIPDTFEDAVNILKPSTIIGVAGAGRLFTPDVIRAMASINERPVIFALS
NPTAQAECTAEEAYTLTEGRCLFASGSPFGPVKLTDGRVFTPGQGNNVYIFPGVALAVIL
CNTRHISDSVFLEAAKALTSQLTDEELAQGRLYPPLANIQEVSINIAIKVTEYLYANKMA
FRYPEPEDKAKYVKERTWRSEYDSLLPDVYEWPESASSPPVITE
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID P23368
UniProtKB/Swiss-Prot Entry Name MAOM_HUMAN
PDB IDs
GenBank Gene ID M55905
GeneCard ID ME2
GenAtlas ID ME2
HGNC ID HGNC:6984
References
General References
  1. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
  2. Gevaert K, Goethals M, Martens L, Van Damme J, Staes A, Thomas GR, Vandekerckhove J: Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides. Nat Biotechnol. 2003 May;21(5):566-9. Epub 2003 Mar 31. [PubMed:12665801 ]
  3. Loeber G, Infante AA, Maurer-Fogy I, Krystek E, Dworkin MB: Human NAD(+)-dependent mitochondrial malic enzyme. cDNA cloning, primary structure, and expression in Escherichia coli. J Biol Chem. 1991 Feb 15;266(5):3016-21. [PubMed:1993674 ]
  4. Yanaihara N, Kohno T, Takakura S, Takei K, Otsuka A, Sunaga N, Takahashi M, Yamazaki M, Tashiro H, Fukuzumi Y, Fujimori Y, Hagiwara K, Tanaka T, Yokota J: Physical and transcriptional map of a 311-kb segment of chromosome 18q21, a candidate lung tumor suppressor locus. Genomics. 2001 Mar 1;72(2):169-79. [PubMed:11401430 ]
  5. Xu Y, Bhargava G, Wu H, Loeber G, Tong L: Crystal structure of human mitochondrial NAD(P)(+)-dependent malic enzyme: a new class of oxidative decarboxylases. Structure. 1999;7(8):877-889. [PubMed:10467136 ]
  6. Yang Z, Floyd DL, Loeber G, Tong L: Structure of a closed form of human malic enzyme and implications for catalytic mechanism. Nat Struct Biol. 2000 Mar;7(3):251-7. [PubMed:10700286 ]
  7. Yang Z, Lanks CW, Tong L: Molecular mechanism for the regulation of human mitochondrial NAD(P)+-dependent malic enzyme by ATP and fumarate. Structure. 2002 Jul;10(7):951-60. [PubMed:12121650 ]
  8. Tao X, Yang Z, Tong L: Crystal structures of substrate complexes of malic enzyme and insights into the catalytic mechanism. Structure. 2003 Sep;11(9):1141-50. [PubMed:12962632 ]