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Identification
HMDB Protein ID HMDBP00900
Secondary Accession Numbers
  • 6182
  • HMDBP03646
Name Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
Synonyms
  1. Isocitric dehydrogenase subunit gamma
  2. NAD(+)-specific ICDH subunit gamma
Gene Name IDH3G
Protein Type Unknown
Biological Properties
General Function Involved in magnesium ion binding
Specific Function Not Available
Pathways
  • 2-ketoglutarate dehydrogenase complex deficiency
  • 2-Oxocarboxylic acid metabolism
  • Citrate cycle (TCA cycle)
  • Citric Acid Cycle
  • Congenital lactic acidosis
  • Fumarase deficiency
  • Glutaminolysis and Cancer
  • Mitochondrial complex II deficiency
  • Pyruvate dehydrogenase deficiency (E2)
  • Pyruvate dehydrogenase deficiency (E3)
  • 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
  • Warburg Effect
Reactions
Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADH details
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Ion details
GO Classification
Biological Process
NADH metabolic process
2-oxoglutarate metabolic process
negative regulation of growth
tricarboxylic acid cycle
isocitrate metabolic process
carbohydrate metabolic process
Cellular Component
mitochondrial matrix
nucleolus
Component
mitochondrion
organelle
membrane-bounded organelle
intracellular membrane-bounded organelle
Function
ion binding
cation binding
metal ion binding
binding
nucleotide binding
catalytic activity
isocitrate dehydrogenase activity
isocitrate dehydrogenase (nad+) activity
magnesium ion binding
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
Molecular Function
NAD binding
magnesium ion binding
ATP binding
isocitrate dehydrogenase (NAD+) activity
Process
metabolic process
cellular metabolic process
oxidation reduction
cofactor metabolic process
coenzyme metabolic process
acetyl-coa metabolic process
acetyl-coa catabolic process
tricarboxylic acid cycle
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location X
Locus Xq28
SNPs IDH3G
Gene Sequence
>1182 bp
ATGGCGCTGAAGGTAGCGACCGTCGCCGGCAGCGCCGCGAAGGCGGTGCTCGGGCCAGCC
CTTCTCTGCCGTCCCTGGGAGGTTCTAGGCGCCCACGAGGTCCCCTCGAGGAACATCTTT
TCAGAACAAACAATTCCTCCGTCCGCTAAGTATGGCGGGCGGCACACGGTGACCATGATC
CCAGGGGATGGCATCGGGCCAGAGCTCATGCTGCATGTCAAGTCCGTCTTCAGGCACGCA
TGTGTACCAGTGGACTTTGAAGAGGTGCACGTGAGTTCCAATGCTGATGAAGAGGACATT
CGCAATGCCATCATGGCCATCCGCCGGAACCGCGTGGCCCTGAAGGGCAACATCGAAACC
AACCATAACCTGCCACCGTCGCACAAATCTCGAAACAACATCCTTCGCACCAGCCTGGAC
CTCTATGCCAACGTCATCCACTGTAAGAGCCTTCCAGGCGTGGTGACCCGGCACAAGGAC
ATAGACATCCTCATTGTCCGGGAGAACACAGAGGGCGAGTACAGCAGCCTGGAGCATGAG
AGTGTGGCGGGAGTGGTGGAGAGCCTGAAGATCATCACCAAGGCCAAGTCCCTGCGCATT
GCCGAGTATGCCTTCAAGCTGGCGCAGGAGAGCGGGCGCAAGAAAGTGACGGCCGTGCAC
AAGGCCAACATCATGAAACTGGGCGATGGGCTTTTCCTCCAGTGCTGCAGGGAGGTGGCA
GCCCGCTACCCTCAGATCACCTTCGAGAACATGATTGTGGATAACACCACCATGCAGCTG
GTGTCCCGGCCCCAGCAGTTTGATGTCATGGTGATGCCCAATCTCTATGGCAACATCGTC
AACAATGTCTGCGCGGGACTGGTCGGGGGCCCAGGCCTTGTGGCTGGGGCCAACTATGGC
CATGTGTACGCGGTGTTTGAAACAGCTACGAGGAACACCGGCAAGAGTATCGCCAATAAG
AACATCGCCAACCCCACGGCCACCCTGCTGGCCAGCTGCATGATGCTGGACCACCTCAAG
CTGCACTCCTATGCCACCTCCATCCGTAAGGCTGTCCTGGCATCCATGGACAATGAGAAT
ATGCACACTCCGGACATCGGGGGCCAGGGCACAACATCTGAAGCCATCCAGGACGTCATC
CGCCACATCCGCGTCATCAACGGCCGGGCCGTGGAGGCCTAG
Protein Properties
Number of Residues 393
Molecular Weight 42793.97
Theoretical pI 8.501
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
MALKVATVAGSAAKAVLGPALLCRPWEVLGAHEVPSRNIFSEQTIPPSAKYGGRHTVTMI
PGDGIGPELMLHVKSVFRHACVPVDFEEVHVSSNADEEDIRNAIMAIRRNRVALKGNIET
NHNLPPSHKSRNNILRTSLDLYANVIHCKSLPGVVTRHKDIDILIVRENTEGEYSSLEHE
SVAGVVESLKIITKAKSLRIAEYAFKLAQESGRKKVTAVHKANIMKLGDGLFLQCCREVA
ARYPQITFENMIVDNTTMQLVSRPQQFDVMVMPNLYGNIVNNVCAGLVGGPGLVAGANYG
HVYAVFETATRNTGKSIANKNIANPTATLLASCMMLDHLKLHSYATSIRKAVLASMDNEN
MHTPDIGGQGTTSEAIQDVIRHIRVINGRAVEA
GenBank ID Protein 1167849
UniProtKB/Swiss-Prot ID P51553
UniProtKB/Swiss-Prot Entry Name IDH3G_HUMAN
PDB IDs Not Available
GenBank Gene ID Z68907
GeneCard ID IDH3G
GenAtlas ID IDH3G
HGNC ID HGNC:5386
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Brenner V, Nyakatura G, Rosenthal A, Platzer M: Genomic organization of two novel genes on human Xq28: compact head to head arrangement of IDH gamma and TRAP delta is conserved in rat and mouse. Genomics. 1997 Aug 15;44(1):8-14. [PubMed:9286695 ]
  3. Kim YO, Koh HJ, Kim SH, Jo SH, Huh JW, Jeong KS, Lee IJ, Song BJ, Huh TL: Identification and functional characterization of a novel, tissue-specific NAD(+)-dependent isocitrate dehydrogenase beta subunit isoform. J Biol Chem. 1999 Dec 24;274(52):36866-75. [PubMed:10601238 ]
  4. Simpson JC, Wellenreuther R, Poustka A, Pepperkok R, Wiemann S: Systematic subcellular localization of novel proteins identified by large-scale cDNA sequencing. EMBO Rep. 2000 Sep;1(3):287-92. [PubMed:11256614 ]