ME2 (gene)

Malic enzyme 2, NAD(+)-dependent, mitochondrial
PDB rendering based on 1do8.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1DO8​, 1EFK​, 1EFL​, 1GZ3​, 1GZ4​, 1PJ2​, 1PJ3​, 1PJ4​, 1PJL​, 1QR6​
Identifiers
Symbols	ME2 ; ODS1
External IDs	OMIM: 154270 MGI: 2147351 HomoloGene: 37615 GeneCards: ME2 Gene
EC number	1.1.1.38
Gene ontology Molecular function • malic enzyme activity • malate dehydrogenase (decarboxylating) (NAD+) activity • oxaloacetate decarboxylase activity • electron carrier activity • metal ion binding • NAD binding Cellular component • mitochondrion • mitochondrial matrix • intracellular membrane-bounded organelle Biological process • malate metabolic process • oxidation-reduction process • regulation of NADP metabolic process Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	4200	107029
Ensembl	ENSG00000082212	ENSMUSG00000024556
UniProt	P23368	Q99KE1
RefSeq (mRNA)	NM_001168335	NM_145494
RefSeq (protein)	NP_001161807	NP_663469
Location (UCSC)	Chr 18: 50.88 – 50.95 Mb	Chr 18: 73.77 – 73.82 Mb
PubMed search

NAD-dependent malic enzyme, mitochondrial is a protein that in humans is encoded by the ME2 gene.^[1][2]

This gene encodes a mitochondrial NAD-dependent malic enzyme, a homotetrameric protein, that catalyzes the oxidative decarboxylation of malate to pyruvate. It had previously been weakly linked to a syndrome known as Friedreich ataxia that has since been shown to be the result of mutation in a completely different gene.^[2]

References

1. Loeber G, Infante AA, Maurer-Fogy I, Krystek E, Dworkin MB (Mar 1991). "Human NAD(+)-dependent mitochondrial malic enzyme. cDNA cloning, primary structure, and expression in Escherichia coli". J Biol Chem 266 (5): 3016–21. PMID 1993674.
2. "Entrez Gene: ME2 malic enzyme 2, NAD(+)-dependent, mitochondrial".

Further reading

• Siebert G, Ritter H, Kömpf J (1980). "Mitochondrial malic enzyme (E.C.1.1.1.40) in human leukocytes: formal genetics and population genetics.". Hum. Genet. 51 (3): 319–22. doi:10.1007/bf00283401. PMID 511162.
• Saha N, Jeremiah SJ, Povey S (1978). "Further data on mitochondrial malic enzyme in man.". Hum. Hered. 28 (6): 421–5. doi:10.1159/000152993. PMID 680703.
• Taroni F, Gellera C, Di Donato S (1988). "Evidence for two distinct mitochondrial malic enzymes in human skeletal muscle: purification and properties of the NAD(P)+-dependent enzyme.". Biochim. Biophys. Acta 916 (3): 446–54. doi:10.1016/0167-4838(87)90191-9. PMID 3689803.
• Bukato G, Kochan Z, Swierczyński J (1996). "Different regulatory properties of the cytosolic and mitochondrial forms of malic enzyme isolated from human brain.". Int. J. Biochem. Cell Biol. 27 (10): 1003–8. doi:10.1016/1357-2725(95)00080-9. PMID 7496989.
• Bukato G, Kochan Z, Swierczyński J (1995). "Purification and properties of cytosolic and mitochondrial malic enzyme isolated from human brain.". Int. J. Biochem. Cell Biol. 27 (1): 47–54. doi:10.1016/1357-2725(94)00057-3. PMID 7757881.
• Xu Y, Bhargava G, Wu H, et al. (1999). "Crystal structure of human mitochondrial NAD(P)+-dependent malic enzyme: a new class of oxidative decarboxylases.". Structure 7 (8): R877–89. doi:10.1016/S0969-2126(99)80115-4. PMID 10477256.
• Yang Z, Floyd DL, Loeber G, Tong L (2000). "Structure of a closed form of human malic enzyme and implications for catalytic mechanism.". Nat. Struct. Biol. 7 (3): 251–7. doi:10.1038/73378. PMID 10700286.
• Yang Z, Batra R, Floyd DL, et al. (2000). "Potent and competitive inhibition of malic enzymes by lanthanide ions.". Biochem. Biophys. Res. Commun. 274 (2): 440–4. doi:10.1006/bbrc.2000.3163. PMID 10913357.
• Butta N, González-Manchón C, Arias-Salgado EG, et al. (2001). "Cloning and functional characterization of the 5' flanking region of the human mitochondrial malic enzyme gene. Regulatory role of Sp1 and AP-2.". Eur. J. Biochem. 268 (10): 3017–27. doi:10.1046/j.1432-1327.2001.02194.x. PMID 11358520.
• Yanaihara N, Kohno T, Takakura S, et al. (2001). "Physical and transcriptional map of a 311-kb segment of chromosome 18q21, a candidate lung tumor suppressor locus.". Genomics 72 (2): 169–79. doi:10.1006/geno.2000.6454. PMID 11401430.
• Yang Z, Lanks CW, Tong L (2002). "Molecular mechanism for the regulation of human mitochondrial NAD(P)+-dependent malic enzyme by ATP and fumarate.". Structure 10 (7): 951–60. doi:10.1016/S0969-2126(02)00788-8. PMID 12121650.
• Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
• Gevaert K, Goethals M, Martens L, et al. (2004). "Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides.". Nat. Biotechnol. 21 (5): 566–9. doi:10.1038/nbt810. PMID 12665801.
• Tao X, Yang Z, Tong L (2004). "Crystal structures of substrate complexes of malic enzyme and insights into the catalytic mechanism.". Structure 11 (9): 1141–50. doi:10.1016/S0969-2126(03)00168-0. PMID 12962632.
• Navarro-Lérida I, Martínez Moreno M, Roncal F, et al. (2004). "Proteomic identification of brain proteins that interact with dynein light chain LC8.". Proteomics 4 (2): 339–46. doi:10.1002/pmic.200300528. PMID 14760703.
• Greenberg DA, Cayanis E, Strug L, et al. (2005). "Malic enzyme 2 may underlie susceptibility to adolescent-onset idiopathic generalized epilepsy.". Am. J. Hum. Genet. 76 (1): 139–46. doi:10.1086/426735. PMC 1196416. PMID 15532013.
• Hung HC, Chien YC, Hsieh JY, et al. (2005). "Functional roles of ATP-binding residues in the catalytic site of human mitochondrial NAD(P)+-dependent malic enzyme.". Biochemistry 44 (38): 12737–45. doi:10.1021/bi050510b. PMID 16171388.
• Kimura K, Wakamatsu A, Suzuki Y, et al. (2006). "Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes.". Genome Res. 16 (1): 55–65. doi:10.1101/gr.4039406. PMC 1356129. PMID 16344560.