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Protein

Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial

Gene

IDH3A

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

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei115 – 1151SubstrateBy similarity
Binding sitei125 – 1251SubstrateBy similarity
Binding sitei146 – 1461SubstrateBy similarity
Sitei153 – 1531Critical for catalysisBy similarity
Sitei200 – 2001Critical for catalysisBy similarity
Metal bindingi233 – 2331Magnesium or manganeseBy similarity
Binding sitei233 – 2331SubstrateBy similarity
Metal bindingi257 – 2571Magnesium or manganeseBy similarity
Metal bindingi261 – 2611Magnesium or manganeseBy similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Tricarboxylic acid cycle

Keywords - Ligandi

Magnesium, Manganese, Metal-binding, NAD

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000166411-MONOMER.
ReactomeiREACT_1785. Citric acid cycle (TCA cycle).
SABIO-RKP50213.

Names & Taxonomyi

Protein namesi
Recommended name:
Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial (EC:1.1.1.41)
Alternative name(s):
Isocitric dehydrogenase subunit alpha
NAD(+)-specific ICDH subunit alpha
Gene namesi
Name:IDH3A
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 15

Organism-specific databases

HGNCiHGNC:5384. IDH3A.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial matrix Source: Reactome
  • mitochondrion Source: HPA
  • nucleus Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA29632.

Polymorphism and mutation databases

BioMutaiIDH3A.
DMDMi1708399.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 2727MitochondrionBy similarityAdd
BLAST
Chaini28 – 366339Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrialPRO_0000014436Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei77 – 771N6-succinyllysineBy similarity
Modified residuei223 – 2231N6-acetyllysineBy similarity
Modified residuei343 – 3431N6-acetyllysine; alternate1 Publication
Modified residuei343 – 3431N6-succinyllysine; alternateBy similarity
Modified residuei350 – 3501N6-succinyllysineBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiP50213.
PaxDbiP50213.
PRIDEiP50213.

2D gel databases

OGPiP50213.
REPRODUCTION-2DPAGEIPI00030702.

PTM databases

PhosphoSiteiP50213.

Expressioni

Gene expression databases

BgeeiP50213.
CleanExiHS_IDH3A.
ExpressionAtlasiP50213. baseline and differential.
GenevestigatoriP50213.

Organism-specific databases

HPAiHPA041465.
HPA062971.

Interactioni

Subunit structurei

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

Protein-protein interaction databases

BioGridi109645. 24 interactions.
IntActiP50213. 9 interactions.
MINTiMINT-1148360.
STRINGi9606.ENSP00000299518.

Structurei

3D structure databases

ProteinModelPortaliP50213.
SMRiP50213. Positions 4-362.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0473.
GeneTreeiENSGT00550000074918.
HOGENOMiHOG000021113.
HOVERGENiHBG052080.
InParanoidiP50213.
KOiK00030.
OMAiQITTFAY.
OrthoDBiEOG75B85R.
PhylomeDBiP50213.
TreeFamiTF105692.

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: P50213-1) [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
MAGPAWISKV SRLLGAFHNP KQVTRGFTGG VQTVTLIPGD GIGPEISAAV
60 70 80 90 100
MKIFDAAKAP IQWEERNVTA IQGPGGKWMI PSEAKESMDK NKMGLKGPLK
110 120 130 140 150
TPIAAGHPSM NLLLRKTFDL YANVRPCVSI EGYKTPYTDV NIVTIRENTE
160 170 180 190 200
GEYSGIEHVI VDGVVQSIKL ITEGASKRIA EFAFEYARNN HRSNVTAVHK
210 220 230 240 250
ANIMRMSDGL FLQKCREVAE SCKDIKFNEM YLDTVCLNMV QDPSQFDVLV
260 270 280 290 300
MPNLYGDILS DLCAGLIGGL GVTPSGNIGA NGVAIFESVH GTAPDIAGKD
310 320 330 340 350
MANPTALLLS AVMMLRHMGL FDHAARIEAA CFATIKDGKS LTKDLGGNAK
360
CSDFTEEICR RVKDLD
Length:366
Mass (Da):39,592
Last modified:October 1, 1996 - v1
Checksum:i695F6A34F97430CF
GO
Isoform 2 (identifier: P50213-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-78: Missing.

Note: No experimental confirmation available.

Show »
Length:288
Mass (Da):31,381
Checksum:iBF8327BD4AA7D3EC
GO

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 7878Missing in isoform 2. 1 PublicationVSP_014516Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U07681 mRNA. Translation: AAA85639.1.
AL442090 mRNA. Translation: CAC09449.1.
CH471136 Genomic DNA. Translation: EAW99181.1.
CH471136 Genomic DNA. Translation: EAW99182.1.
BC021967 mRNA. Translation: AAH21967.1.
CCDSiCCDS10297.1. [P50213-1]
PIRiS55282.
RefSeqiNP_005521.1. NM_005530.2. [P50213-1]
UniGeneiHs.591110.

Genome annotation databases

EnsembliENST00000299518; ENSP00000299518; ENSG00000166411. [P50213-1]
GeneIDi3419.
KEGGihsa:3419.
UCSCiuc002bdd.3. human. [P50213-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U07681 mRNA. Translation: AAA85639.1.
AL442090 mRNA. Translation: CAC09449.1.
CH471136 Genomic DNA. Translation: EAW99181.1.
CH471136 Genomic DNA. Translation: EAW99182.1.
BC021967 mRNA. Translation: AAH21967.1.
CCDSiCCDS10297.1. [P50213-1]
PIRiS55282.
RefSeqiNP_005521.1. NM_005530.2. [P50213-1]
UniGeneiHs.591110.

3D structure databases

ProteinModelPortaliP50213.
SMRiP50213. Positions 4-362.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109645. 24 interactions.
IntActiP50213. 9 interactions.
MINTiMINT-1148360.
STRINGi9606.ENSP00000299518.

PTM databases

PhosphoSiteiP50213.

Polymorphism and mutation databases

BioMutaiIDH3A.
DMDMi1708399.

2D gel databases

OGPiP50213.
REPRODUCTION-2DPAGEIPI00030702.

Proteomic databases

MaxQBiP50213.
PaxDbiP50213.
PRIDEiP50213.

Protocols and materials databases

DNASUi3419.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000299518; ENSP00000299518; ENSG00000166411. [P50213-1]
GeneIDi3419.
KEGGihsa:3419.
UCSCiuc002bdd.3. human. [P50213-1]

Organism-specific databases

CTDi3419.
GeneCardsiGC15P078423.
HGNCiHGNC:5384. IDH3A.
HPAiHPA041465.
HPA062971.
MIMi601149. gene.
neXtProtiNX_P50213.
PharmGKBiPA29632.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG0473.
GeneTreeiENSGT00550000074918.
HOGENOMiHOG000021113.
HOVERGENiHBG052080.
InParanoidiP50213.
KOiK00030.
OMAiQITTFAY.
OrthoDBiEOG75B85R.
PhylomeDBiP50213.
TreeFamiTF105692.

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000166411-MONOMER.
ReactomeiREACT_1785. Citric acid cycle (TCA cycle).
SABIO-RKP50213.

Miscellaneous databases

ChiTaRSiIDH3A. human.
GeneWikiiIDH3A.
GenomeRNAii3419.
NextBioi13478.
PROiP50213.
SOURCEiSearch...

Gene expression databases

BgeeiP50213.
CleanExiHS_IDH3A.
ExpressionAtlasiP50213. baseline and differential.
GenevestigatoriP50213.

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. "Characterization of a cDNA clone for human NAD(+)-specific isocitrate dehydrogenase alpha-subunit and structural comparison with its isoenzymes from different species."
    Kim Y.O., Oh I.U., Park H.S., Jeng J., Song B.J., Huh T.L.
    Biochem. J. 308:63-68(1995) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Heart.
  2. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
    Tissue: Brain cortex.
  3. 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: Lymph.
  5. Lubec G., Vishwanath V., Chen W.-Q., Sun Y.
    Submitted (DEC-2008) to UniProtKB
    Cited for: PROTEIN SEQUENCE OF 135-146; 179-188 AND 300-316, IDENTIFICATION BY MASS SPECTROMETRY.
    Tissue: Brain, Cajal-Retzius cell and Fetal brain cortex.
  6. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
    Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
    Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-343, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiIDH3A_HUMAN
AccessioniPrimary (citable) accession number: P50213
Secondary accession number(s): D3DW83, Q9H3X0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: April 29, 2015
This is version 152 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, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 15
    Human chromosome 15: 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.