ME2 (gene)

NAD-dependent malic enzyme, mitochondrial is a protein that in humans is encoded by the ME2 gene. 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.

ME2
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 1DO8, 1EFK, 1EFL, 1GZ3, 1GZ4, 1PJ2, 1PJ3, 1PJ4, 1PJL, 1QR6
Identifiers
Aliases	ME2, ODS1, malic enzyme 2
External IDs	OMIM: 154270 MGI: 2147351 HomoloGene: 37615 GeneCards: ME2
Gene location (Human) Chr. Chromosome 18 (human) Band 18q21.2 Start 50,879,080 bp End 50,954,257 bp
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in jejunal mucosa monocyte duodenum right ventricle rectum trabecular bone vena cava seminal vesicula appendix thoracic diaphragm n/a More reference expression data BioGPS More reference expression data
Gene ontology Molecular function malate dehydrogenase (decarboxylating) (NAD+) activity electron transfer activity oxidoreductase activity NAD binding oxaloacetate decarboxylase activity catalytic activity metal ion binding malic enzyme activity malate dehydrogenase (decarboxylating) (NADP+) activity Cellular component mitochondrion mitochondrial matrix Biological process regulation of NADP metabolic process metabolism pyruvate metabolic process malate metabolic process electron transport chain tricarboxylic acid cycle Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 4200 107029 Ensembl ENSG00000082212 n/a UniProt P23368 Q99KE1 RefSeq (mRNA) NM_001168335 NM_002396 NM_145494 RefSeq (protein) NP_001161807 NP_002387 NP_663469 Location (UCSC) Chr 18: 50.88 – 50.95 Mb n/a PubMed search
Wikidata
View/Edit Human View/Edit Mouse

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. S2CID 28855152.
• 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. S2CID 23469024.
• 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. hdl:10261/73615. 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. Bibcode:2002PNAS...9916899M. 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. S2CID 23783563.
• 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. S2CID 8868600.
• 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.