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Protein Page:
IDH2 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
IDH2 a mitochondrial oxidoreductase that catalyzes the third step of the citric acid cycle: the oxidative decarboxylation of isocitrate, consuming NADP(+), and producing alpha-ketoglutarate (alpha-KG) and CO2. Can also use oxalosuccinate as a substrate. Alpha-KG is an activator the dioxygenases that hydroxylate the transcription factor HIF and lead to its degradation by VHL. Since HIF turns on oncogenic pathways, IDH2 has apparent tumor suppressor activity. Homo-dimerization is required for activity. Each subunit binds 1 magnesium or manganese ion. May tightly associate or interact with the pyruvate dehydrogenase complex. A somatic mutation resulting in the R172K conversion results in a neomorphic enzyme activity that converts isocitrate to R(-)-2-hydroxyglutarate (2HG) occurs in a significant percentage of patients with cytogenetically normal acute myeloid leukemia (AML). Elevated levels of 2HG are also correlated with an elevated risk of malignant brain tumors. Note: This description may include information from UniProtKB.
Protein type: Mitochondrial; Oxidoreductase; EC 1.1.1.42; Carbohydrate Metabolism - citrate (TCA) cycle; Other Amino Acids Metabolism - glutathione
Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane
Molecular Function: magnesium ion binding; isocitrate dehydrogenase (NADP+) activity; NAD binding
Biological Process: glyoxylate cycle; isocitrate metabolic process; cellular metabolic process; tricarboxylic acid cycle; carbohydrate metabolic process; 2-oxoglutarate metabolic process
Reference #:  P48735 (UniProtKB)
Alt. Names/Synonyms: ICD-M; IDH; IDH2; IDHM; IDHP; IDP; IDPM; isocitrate dehydrogenase 2 (NADP+), mitochondrial; Isocitrate dehydrogenase [NADP], mitochondrial; isocitrate dehydrogenase, mitochondrial; mNADP-IDH; NADP(+)-specific ICDH; NADP+-specific ICDH; Oxalosuccinate decarboxylase
Gene Symbols: IDH2
Molecular weight: 50,909 Da
Basal Isoelectric point: 8.88  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
Select Structure to View Below

IDH2

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 2 K45 HYADKRIKVAKPVVE
0 2 K48 DKRIKVAKPVVEMDG
0 1 K48 DKRIKVAKPVVEMDG
0 2 K67-ac RIIWQFIkEKLILPH
0 1 K67 RIIWQFIKEKLILPH
0 1 K69 IWQFIkEKLILPHVD
0 72 K80-ac PHVDIQLkYFDLGLP
0 1 K80 PHVDIQLKYFDLGLP
0 2 T97-p DQTDDQVtIDsALAT
0 2 S100-p DDQVtIDsALATQky
0 35 K106-ac DsALATQkysVAVKC
0 1 K106 DsALATQKysVAVKC
0 2 K106 DsALATQKysVAVKC
0 6 Y107-p sALATQkysVAVKCA
0 5 S108-p ALATQkysVAVKCAt
0 1 T115-p sVAVKCAtITPDEAR
0 9 K133-ac FKLKKMWkSPNGTIR
0 6 K155-ac FREPIICkNIPRLVP
0 17 K166-ac RLVPGWTkPITIGRH
0 2 K166-ub RLVPGWTkPITIGRH
0 2 K166 RLVPGWTKPITIGRH
0 11 Y179-p RHAHGDQykAtDFVA
0 45 K180-ac HAHGDQykAtDFVAD
0 1 K180 HAHGDQyKAtDFVAD
0 2 K180-sc HAHGDQykAtDFVAD
0 1 T182-p HGDQykAtDFVADRA
0 1 T191-p FVADRAGtFKMVFTP
0 2 K193 ADRAGtFKMVFTPkD
0 4 K199 FKMVFTPKDGSGVKE
0 1 K199 FKMVFTPKDGSGVKE
0 2 K199-sc FKMVFTPkDGSGVKE
0 1 K242-ac CFQYAIQkKWPLyMS
0 1 K243 FQYAIQkKWPLyMST
0 1 Y247-p IQkKWPLyMSTKNTI
0 3 K256-ac STKNTILkAYDGRFk
0 1 K256 STKNTILKAYDGRFk
0 2 K256-sc STKNTILkAYDGRFk
0 20 K263-ac kAYDGRFkDIFQEIF
0 2 K263-ub kAYDGRFkDIFQEIF
0 10 K272-ac IFQEIFDkHYkTDFD
0 1 K272 IFQEIFDKHYkTDFD
0 2 K275-ac EIFDkHYkTDFDKNk
0 1 K275-sc EIFDkHYkTDFDKNk
0 1 K280 HYkTDFDKNkIWYEH
0 2 K280 HYkTDFDKNkIWYEH
0 3 K282-ac kTDFDKNkIWYEHRL
0 1 K282 kTDFDKNKIWYEHRL
0 1 S301-p VAQVLKSsGGFVWAC
0 1 K360 RHYREHQKGRPTSTN
0 1 K360 RHYREHQKGRPTSTN
0 6 K384-ac RGLEHRGkLDGNQDL
0 1 K384 RGLEHRGKLDGNQDL
0 1 K384 RGLEHRGKLDGNQDL
0 3 K400 RFAQMLEKVCVETVE
0 1 K400 RFAQMLEKVCVETVE
0 5 K413-ac VESGAMTkDLAGCIH
0 5 S423-p AGCIHGLsNVKLNEH
0 7 K442-ac TDFLDTIkSNLDRAL
0 1 R451 NLDRALGRQ______
  mouse

 
K45-ac HYAEKRIkVEkPVVE
K48-ac EKRIkVEkPVVEMDG
K48-ub EKRIkVEkPVVEMDG
K67-ac RIIWQFIkEkLILPH
K67-ub RIIWQFIkEkLILPH
K69-ac IWQFIkEkLILPHVD
K80-ac PHVDVQLkYFDLGLP
K80-sc PHVDVQLkYFDLGLP
T97 DQTNDQVTIDSALAT
S100 NDQVTIDSALATQkY
K106-ac DSALATQkYSVAVKC
K106-ub DSALATQkYSVAVKC
K106-sc DSALATQkYSVAVKC
Y107 SALATQkYSVAVKCA
S108 ALATQkYSVAVKCAT
T115 SVAVKCATITPDEAR
K133-ac FKLKKMWkSPNGTIR
K155-ac FREPIICkNIPRLVP
K166-ac RLVPGWTkPITIGRH
K166-ub RLVPGWTkPITIGRH
K166-sc RLVPGWTkPITIGRH
Y179 RHAHGDQYkATDFVV
K180-ac HAHGDQYkATDFVVD
K180-ub HAHGDQYkATDFVVD
K180-sc HAHGDQYkATDFVVD
T182 HGDQYkATDFVVDRA
T191 FVVDRAGTFkLVFTP
K193-ac VDRAGTFkLVFTPkD
K199-ac FkLVFTPkDGSSAKE
K199-ub FkLVFTPkDGSSAKE
K199-sc FkLVFTPkDGSSAKE
K242 CFQYSIQKkWPLYLS
K243-sc FQYSIQKkWPLYLST
Y247 IQKkWPLYLSTKNTI
K256-ac STKNTILkAYDGRFk
K256-ub STKNTILkAYDGRFk
K256-sc STKNTILkAYDGRFk
K263-ac kAYDGRFkDIFQEIF
K263-ub kAYDGRFkDIFQEIF
K272-ac IFQEIFDkHYkTDFD
K272-ub IFQEIFDkHYkTDFD
K275-ac EIFDkHYkTDFDkNk
K275-sc EIFDkHYkTDFDkNk
K280-ac HYkTDFDkNkIWYEH
K280-sc HYkTDFDkNkIWYEH
K282-ac kTDFDkNkIWYEHRL
K282-sc kTDFDkNkIWYEHRL
S301-p VAQVLKSsGGFVWAC
K360-ac RHYREHQkGRPTSTN
K360-sc RHYREHQkGRPTSTN
K384-ac RGLEHRGkLDGNQDL
K384-ub RGLEHRGkLDGNQDL
K384-sc RGLEHRGkLDGNQDL
K400-ac RFAQTLEkVCVQTVE
K400-sc RFAQTLEkVCVQTVE
K413-ac VESGAMTkDLAGCIH
S423-p AGCIHGLsNVKLNEH
K442-ac TDFLDTIkSNLDRAL
K451-ac NLDRALGkQ______
  rat

 
K45-ac HYAEKRIkVEkPVVE
K48-ac EKRIkVEkPVVEMDG
K48 EKRIkVEKPVVEMDG
K67-ac RIIWQFIkEkLILPH
K67 RIIWQFIKEkLILPH
K69-ac IWQFIkEkLILPHVD
K80-ac PHVDVQLkYFDLGLP
K80 PHVDVQLKYFDLGLP
T97 DQTNDQVTIDSALAT
S100 NDQVTIDSALATQkY
K106-ac DSALATQkYSVAVKC
K106 DSALATQKYSVAVKC
K106 DSALATQKYSVAVKC
Y107 SALATQkYSVAVKCA
S108 ALATQkYSVAVKCAT
T115 SVAVKCATITPDEAR
K133-ac FKLKKMWkSPNGTIR
K155-ac FREPIICkNIPRLVP
K166-ac RLVPGWTkPITIGRH
K166 RLVPGWTKPITIGRH
K166 RLVPGWTKPITIGRH
Y179-p RHAHGDQykATDFVV
K180-ac HAHGDQykATDFVVD
K180 HAHGDQyKATDFVVD
K180 HAHGDQyKATDFVVD
T182 HGDQykATDFVVDRA
M191 FVVDRAGMFkLVFTP
K193-ac VDRAGMFkLVFTPkD
K199-ac FkLVFTPkDGSGAKE
K199 FkLVFTPKDGSGAKE
K199 FkLVFTPKDGSGAKE
K242 CFQYSIQKKWPLYLS
K243 FQYSIQKKWPLYLST
Y247 IQKKWPLYLSTKNTI
K256-ac STKNTIMkAYDGRFk
K256 STKNTIMKAYDGRFk
K256 STKNTIMKAYDGRFk
K263-ac kAYDGRFkDIFQEIF
K263 kAYDGRFKDIFQEIF
K272-ac IFQEIFDkHYkTDFD
K272 IFQEIFDKHYkTDFD
K275-ac EIFDkHYkTDFDkNk
K275 EIFDkHYKTDFDkNk
K280-ac HYkTDFDkNkIWYEH
K280 HYkTDFDKNkIWYEH
K282-ac kTDFDkNkIWYEHRL
K282 kTDFDkNKIWYEHRL
S301 VAQVLKSSGGFVWAC
K360 RHYREHQKGRPTSTN
K360 RHYREHQKGRPTSTN
K384-ac RGLEHRGkLDGNQDL
K384 RGLEHRGKLDGNQDL
K384 RGLEHRGKLDGNQDL
K400-ac RFAQTLEkVCVQTVE
K400 RFAQTLEKVCVQTVE
K413-ac VESGAMTkDLAGCIH
S423 AGCIHGLSNVKLNEH
K442-ac TDFLDTIkSNLDRAL
K451 NLDRALGKQ______
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