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Protein

Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial

Gene

IDH3G

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalytic activityi

Isocitrate + NAD+ = 2-oxoglutarate + CO2 + NADH.

Cofactori

Mg2+By similarity, Mn2+By similarityNote: Binds 1 Mg(2+) or Mn2+ ion per subunit.By similarity

Enzyme regulationi

Activated by increasing ADP/ATP ratios and by Ca2+.By similarity

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei136 – 1361SubstrateBy similarity
Binding sitei167 – 1671SubstrateBy similarity
Sitei174 – 1741Critical for catalysisBy similarity
Sitei221 – 2211Critical for catalysisBy similarity
Metal bindingi254 – 2541Magnesium or manganeseBy similarity
Binding sitei254 – 2541SubstrateBy similarity

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi56 – 8429NADSequence AnalysisAdd
BLAST
Nucleotide bindingi309 – 3168ATPSequence Analysis

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Tricarboxylic acid cycle

Keywords - Ligandi

ATP-binding, Magnesium, Manganese, Metal-binding, NAD, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000067829-MONOMER.
ReactomeiREACT_118595. Mitochondrial protein import.
REACT_1785. Citric acid cycle (TCA cycle).
SABIO-RKP51553.

Names & Taxonomyi

Protein namesi
Recommended name:
Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial (EC:1.1.1.41)
Alternative name(s):
Isocitric dehydrogenase subunit gamma
NAD(+)-specific ICDH subunit gamma
Gene namesi
Name:IDH3G
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome X

Organism-specific databases

HGNCiHGNC:5386. IDH3G.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA29634.

Polymorphism and mutation databases

BioMutaiIDH3G.
DMDMi1708404.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3939MitochondrionBy similarityAdd
BLAST
Chaini40 – 393354Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrialPRO_0000014449Add
BLAST

Proteomic databases

MaxQBiP51553.
PaxDbiP51553.
PRIDEiP51553.

PTM databases

PhosphoSiteiP51553.

Expressioni

Gene expression databases

BgeeiP51553.
CleanExiHS_IDH3G.
ExpressionAtlasiP51553. baseline and differential.
GenevestigatoriP51553.

Organism-specific databases

HPAiHPA000425.
HPA002017.

Interactioni

Subunit structurei

Heterooligomer of subunits alpha, beta, and gamma in the apparent ratio of 2:1:1.By similarity

Protein-protein interaction databases

BioGridi109647. 18 interactions.
IntActiP51553. 2 interactions.
MINTiMINT-3018829.
STRINGi9606.ENSP00000217901.

Structurei

3D structure databases

ProteinModelPortaliP51553.
SMRiP51553. Positions 50-386.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0473.
GeneTreeiENSGT00590000083091.
HOGENOMiHOG000021113.
HOVERGENiHBG052080.
InParanoidiP51553.
KOiK00030.
OMAiLRPCKTY.
OrthoDBiEOG75B85R.
PhylomeDBiP51553.
TreeFamiTF315033.

Family and domain databases

Gene3Di3.40.718.10. 1 hit.
InterProiIPR019818. IsoCit/isopropylmalate_DH_CS.
IPR001804. Isocitrate/isopropylmalate_DH.
IPR004434. Isocitrate_DH_NAD.
IPR024084. IsoPropMal-DH-like_dom.
[Graphical view]
PANTHERiPTHR11835. PTHR11835. 1 hit.
PfamiPF00180. Iso_dh. 1 hit.
[Graphical view]
TIGRFAMsiTIGR00175. mito_nad_idh. 1 hit.
PROSITEiPS00470. IDH_IMDH. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P51553-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MALKVATVAG SAAKAVLGPA LLCRPWEVLG AHEVPSRNIF SEQTIPPSAK
60 70 80 90 100
YGGRHTVTMI PGDGIGPELM LHVKSVFRHA CVPVDFEEVH VSSNADEEDI
110 120 130 140 150
RNAIMAIRRN RVALKGNIET NHNLPPSHKS RNNILRTSLD LYANVIHCKS
160 170 180 190 200
LPGVVTRHKD IDILIVRENT EGEYSSLEHE SVAGVVESLK IITKAKSLRI
210 220 230 240 250
AEYAFKLAQE SGRKKVTAVH KANIMKLGDG LFLQCCREVA ARYPQITFEN
260 270 280 290 300
MIVDNTTMQL VSRPQQFDVM VMPNLYGNIV NNVCAGLVGG PGLVAGANYG
310 320 330 340 350
HVYAVFETAT RNTGKSIANK NIANPTATLL ASCMMLDHLK LHSYATSIRK
360 370 380 390
AVLASMDNEN MHTPDIGGQG TTSEAIQDVI RHIRVINGRA VEA
Length:393
Mass (Da):42,794
Last modified:October 1, 1996 - v1
Checksum:iA0870F2B7D228A37
GO
Isoform 2 (identifier: P51553-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     361-393: MHTPDIGGQGTTSEAIQDVIRHIRVINGRAVEA → VRFPSHPTLLPRPVSPCSLL

Note: Gene prediction based on EST data.

Show »
Length:380
Mass (Da):41,468
Checksum:i2D5987CC70725245
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti267 – 2671F → L in AAH01902 (PubMed:15489334).Curated

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei361 – 39333MHTPD…RAVEA → VRFPSHPTLLPRPVSPCSLL in isoform 2. CuratedVSP_046771Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z68907 mRNA. Translation: CAA93143.1.
Z68129 Genomic DNA. Translation: CAA92214.1.
U52111 Genomic DNA. No translation available.
U40272 mRNA. Translation: AAD09357.1.
BC000933 mRNA. Translation: AAH00933.1.
BC001902 mRNA. Translation: AAH01902.1.
CCDSiCCDS14730.1. [P51553-1]
CCDS44019.1. [P51553-2]
RefSeqiNP_004126.1. NM_004135.3. [P51553-1]
NP_777358.1. NM_174869.2. [P51553-2]
UniGeneiHs.410197.

Genome annotation databases

EnsembliENST00000217901; ENSP00000217901; ENSG00000067829. [P51553-1]
ENST00000370092; ENSP00000359110; ENSG00000067829. [P51553-2]
GeneIDi3421.
KEGGihsa:3421.
UCSCiuc004fip.4. human. [P51553-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z68907 mRNA. Translation: CAA93143.1.
Z68129 Genomic DNA. Translation: CAA92214.1.
U52111 Genomic DNA. No translation available.
U40272 mRNA. Translation: AAD09357.1.
BC000933 mRNA. Translation: AAH00933.1.
BC001902 mRNA. Translation: AAH01902.1.
CCDSiCCDS14730.1. [P51553-1]
CCDS44019.1. [P51553-2]
RefSeqiNP_004126.1. NM_004135.3. [P51553-1]
NP_777358.1. NM_174869.2. [P51553-2]
UniGeneiHs.410197.

3D structure databases

ProteinModelPortaliP51553.
SMRiP51553. Positions 50-386.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109647. 18 interactions.
IntActiP51553. 2 interactions.
MINTiMINT-3018829.
STRINGi9606.ENSP00000217901.

PTM databases

PhosphoSiteiP51553.

Polymorphism and mutation databases

BioMutaiIDH3G.
DMDMi1708404.

Proteomic databases

MaxQBiP51553.
PaxDbiP51553.
PRIDEiP51553.

Protocols and materials databases

DNASUi3421.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000217901; ENSP00000217901; ENSG00000067829. [P51553-1]
ENST00000370092; ENSP00000359110; ENSG00000067829. [P51553-2]
GeneIDi3421.
KEGGihsa:3421.
UCSCiuc004fip.4. human. [P51553-1]

Organism-specific databases

CTDi3421.
GeneCardsiGC0XM153051.
HGNCiHGNC:5386. IDH3G.
HPAiHPA000425.
HPA002017.
MIMi300089. gene.
neXtProtiNX_P51553.
PharmGKBiPA29634.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG0473.
GeneTreeiENSGT00590000083091.
HOGENOMiHOG000021113.
HOVERGENiHBG052080.
InParanoidiP51553.
KOiK00030.
OMAiLRPCKTY.
OrthoDBiEOG75B85R.
PhylomeDBiP51553.
TreeFamiTF315033.

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000067829-MONOMER.
ReactomeiREACT_118595. Mitochondrial protein import.
REACT_1785. Citric acid cycle (TCA cycle).
SABIO-RKP51553.

Miscellaneous databases

ChiTaRSiIDH3G. human.
GeneWikiiIDH3G.
GenomeRNAii3421.
NextBioi13490.
PROiP51553.
SOURCEiSearch...

Gene expression databases

BgeeiP51553.
CleanExiHS_IDH3G.
ExpressionAtlasiP51553. baseline and differential.
GenevestigatoriP51553.

Family and domain databases

Gene3Di3.40.718.10. 1 hit.
InterProiIPR019818. IsoCit/isopropylmalate_DH_CS.
IPR001804. Isocitrate/isopropylmalate_DH.
IPR004434. Isocitrate_DH_NAD.
IPR024084. IsoPropMal-DH-like_dom.
[Graphical view]
PANTHERiPTHR11835. PTHR11835. 1 hit.
PfamiPF00180. Iso_dh. 1 hit.
[Graphical view]
TIGRFAMsiTIGR00175. mito_nad_idh. 1 hit.
PROSITEiPS00470. IDH_IMDH. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "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."
    Brenner V., Nyakatura G., Rosenthal A., Platzer M.
    Genomics 44:8-14(1997) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA] (ISOFORM 1).
    Tissue: Brain.
  2. "Identification and functional characterization of a novel, tissue-specific NAD+-dependent isocitrate dehydrogenase beta subunit isoform."
    Kim Y.-O., Koh H.-J., Kim S.-H., Jo S.-H., Huh J.-W., Jeong K.-S., Lee I.J., Song B.J., Huh T.-L.
    J. Biol. Chem. 274:36866-36875(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Heart.
  3. "The DNA sequence of the human X chromosome."
    Ross M.T., Grafham D.V., Coffey A.J., Scherer S., McLay K., Muzny D., Platzer M., Howell G.R., Burrows C., Bird C.P., Frankish A., Lovell F.L., Howe K.L., Ashurst J.L., Fulton R.S., Sudbrak R., Wen G., Jones M.C.
    , Hurles M.E., Andrews T.D., Scott C.E., Searle S., Ramser J., Whittaker A., Deadman R., Carter N.P., Hunt S.E., Chen R., Cree A., Gunaratne P., Havlak P., Hodgson A., Metzker M.L., Richards S., Scott G., Steffen D., Sodergren E., Wheeler D.A., Worley K.C., Ainscough R., Ambrose K.D., Ansari-Lari M.A., Aradhya S., Ashwell R.I., Babbage A.K., Bagguley C.L., Ballabio A., Banerjee R., Barker G.E., Barlow K.F., Barrett I.P., Bates K.N., Beare D.M., Beasley H., Beasley O., Beck A., Bethel G., Blechschmidt K., Brady N., Bray-Allen S., Bridgeman A.M., Brown A.J., Brown M.J., Bonnin D., Bruford E.A., Buhay C., Burch P., Burford D., Burgess J., Burrill W., Burton J., Bye J.M., Carder C., Carrel L., Chako J., Chapman J.C., Chavez D., Chen E., Chen G., Chen Y., Chen Z., Chinault C., Ciccodicola A., Clark S.Y., Clarke G., Clee C.M., Clegg S., Clerc-Blankenburg K., Clifford K., Cobley V., Cole C.G., Conquer J.S., Corby N., Connor R.E., David R., Davies J., Davis C., Davis J., Delgado O., Deshazo D., Dhami P., Ding Y., Dinh H., Dodsworth S., Draper H., Dugan-Rocha S., Dunham A., Dunn M., Durbin K.J., Dutta I., Eades T., Ellwood M., Emery-Cohen A., Errington H., Evans K.L., Faulkner L., Francis F., Frankland J., Fraser A.E., Galgoczy P., Gilbert J., Gill R., Gloeckner G., Gregory S.G., Gribble S., Griffiths C., Grocock R., Gu Y., Gwilliam R., Hamilton C., Hart E.A., Hawes A., Heath P.D., Heitmann K., Hennig S., Hernandez J., Hinzmann B., Ho S., Hoffs M., Howden P.J., Huckle E.J., Hume J., Hunt P.J., Hunt A.R., Isherwood J., Jacob L., Johnson D., Jones S., de Jong P.J., Joseph S.S., Keenan S., Kelly S., Kershaw J.K., Khan Z., Kioschis P., Klages S., Knights A.J., Kosiura A., Kovar-Smith C., Laird G.K., Langford C., Lawlor S., Leversha M., Lewis L., Liu W., Lloyd C., Lloyd D.M., Loulseged H., Loveland J.E., Lovell J.D., Lozado R., Lu J., Lyne R., Ma J., Maheshwari M., Matthews L.H., McDowall J., McLaren S., McMurray A., Meidl P., Meitinger T., Milne S., Miner G., Mistry S.L., Morgan M., Morris S., Mueller I., Mullikin J.C., Nguyen N., Nordsiek G., Nyakatura G., O'dell C.N., Okwuonu G., Palmer S., Pandian R., Parker D., Parrish J., Pasternak S., Patel D., Pearce A.V., Pearson D.M., Pelan S.E., Perez L., Porter K.M., Ramsey Y., Reichwald K., Rhodes S., Ridler K.A., Schlessinger D., Schueler M.G., Sehra H.K., Shaw-Smith C., Shen H., Sheridan E.M., Shownkeen R., Skuce C.D., Smith M.L., Sotheran E.C., Steingruber H.E., Steward C.A., Storey R., Swann R.M., Swarbreck D., Tabor P.E., Taudien S., Taylor T., Teague B., Thomas K., Thorpe A., Timms K., Tracey A., Trevanion S., Tromans A.C., d'Urso M., Verduzco D., Villasana D., Waldron L., Wall M., Wang Q., Warren J., Warry G.L., Wei X., West A., Whitehead S.L., Whiteley M.N., Wilkinson J.E., Willey D.L., Williams G., Williams L., Williamson A., Williamson H., Wilming L., Woodmansey R.L., Wray P.W., Yen J., Zhang J., Zhou J., Zoghbi H., Zorilla S., Buck D., Reinhardt R., Poustka A., Rosenthal A., Lehrach H., Meindl A., Minx P.J., Hillier L.W., Willard H.F., Wilson R.K., Waterston R.H., Rice C.M., Vaudin M., Coulson A., Nelson D.L., Weinstock G., Sulston J.E., Durbin R.M., Hubbard T., Gibbs R.A., Beck S., Rogers J., Bentley D.R.
    Nature 434:325-337(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    Tissue: Placenta and Skin.
  5. "Systematic subcellular localization of novel proteins identified by large-scale cDNA sequencing."
    Simpson J.C., Wellenreuther R., Poustka A., Pepperkok R., Wiemann S.
    EMBO Rep. 1:287-292(2000) [PubMed] [Europe PMC] [Abstract]
    Cited for: SUBCELLULAR LOCATION.
  6. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  7. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiIDH3G_HUMAN
AccessioniPrimary (citable) accession number: P51553
Secondary accession number(s): E9PDD5, Q9BUU5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: May 27, 2015
This is version 163 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome X
    Human chromosome X: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.