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Protein Page:
IDH1 (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
IDH1 an oxidoreductase that catalyzes the third step of the TCA cycle: the oxidative decarboxylation of isocitrate, consuming NADP(+), and producing alpha-ketoglutarate (alpha-KG) and CO2. 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, IDH1 has apparent tumor suppressor activity. Homo-dimerization is required for activity. Each subunit binds 1 magnesium or manganese ion. IDH1 is located in the cytosol and peroxisomes. Somatic mutations affecting arginine 132 (R132) are found in 80% of grade II?III gliomas and secondary glioblastomas in humans, and cause disease in a tissue-specific fashion. Only a single copy of the gene has been found to be mutated in tumours. Mutations of R132 to H, C, S, G, V, or L have been reported to be neomorphic, abolishing the conversion of isocitrate to alpha-KG. Instead, alpha-KG is converted to R(-)-2-hydroxyglutarate (2HG). Elevated levels of 2HG correlate with an elevated risk of malignant brain tumors. Neomorphic mutations in IDH1 and IDH2 convert alpha-KG to 2-hydroxyglutarate (2-HG) occur in a high percentage of patients with cytogenetically normal acute myeloid leukemia (AML). Note: This description may include information from UniProtKB.
Protein type: Other Amino Acids Metabolism - glutathione; Carbohydrate Metabolism - citrate (TCA) cycle; EC 1.1.1.42; Oxidoreductase
Cellular Component: peroxisomal matrix; mitochondrion; cytoplasm; peroxisome; cytosol
Molecular Function: protein homodimerization activity; magnesium ion binding; isocitrate dehydrogenase (NADP+) activity; NADP binding; NAD binding; receptor binding
Biological Process: glyoxylate cycle; NADPH regeneration; isocitrate metabolic process; glutathione metabolic process; tricarboxylic acid cycle; response to steroid hormone stimulus; response to oxidative stress; cellular lipid metabolic process; female gonad development; 2-oxoglutarate metabolic process
Reference #:  O75874 (UniProtKB)
Alt. Names/Synonyms: Cytosolic NADP-isocitrate dehydrogenase; IDCD; IDH; IDH1; IDHC; IDP; IDPC; isocitrate dehydrogenase 1 (NADP+), soluble; Isocitrate dehydrogenase [NADP] cytoplasmic; NADP(+)-specific ICDH; NADP+-specific ICDH; NADP-dependent isocitrate dehydrogenase, cytosolic; NADP-dependent isocitrate dehydrogenase, peroxisomal; Oxalosuccinate decarboxylase; PICD
Gene Symbols: IDH1
Molecular weight: 46,659 Da
Basal Isoelectric point: 6.53  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
Select Structure to View Below

IDH1

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 1 K4 ____MSKKISGGSVV
0 1 T19-p EMQGDEMtRIIWELI
0 1 K27 RIIWELIKEKLIFPy
0 1 K27 RIIWELIKEKLIFPy
0 2 Y34-p KEKLIFPyVELDLHs
0 11 S41-p yVELDLHsyDLGIEN
0 3 Y42-p VELDLHsyDLGIENR
0 2 K58-ac ATNDQVTkDAAEAIK
0 2 K58-ub ATNDQVTkDAAEAIK
0 1 K58 ATNDQVTKDAAEAIK
0 14 T75-p NVGVKCAtItPDEkR
0 14 T77-p GVKCAtItPDEkRVE
0 54 K81-ac AtItPDEkRVEEFKL
0 1 K81 AtItPDEKRVEEFKL
0 8 K93-ac FKLKQMWkSPNGTIR
0 1 K93 FKLKQMWKSPNGTIR
0 2 K126-ac RLVSGWVkPIIIGRH
0 2 K126-ub RLVSGWVkPIIIGRH
0 12 Y135-p IIIGRHAyGDQyRAT
0 9 Y139-p RHAyGDQyRATDFVV
0 2 K151-ac FVVPGPGkVEITYTP
0 2 K151-ub FVVPGPGkVEITYTP
0 1 K151 FVVPGPGKVEITYTP
0 1 S159 VEITYTPSDGTQKVT
0 1 S159 VEITYTPSDGTQKVT
0 1 S159 VEITYTPSDGTQKVT
0 1 Y208-p LSKGWPLyLSTKNtI
0 1 K212 WPLyLSTKNtILKKy
0 1 T214-p LyLSTKNtILKKyDG
0 1 Y219-p KNtILKKyDGRFkDI
0 10 K224-ac KKyDGRFkDIFQEIy
0 2 K224-ub KKyDGRFkDIFQEIy
0 14 Y231-p kDIFQEIyDkQYKSQ
0 6 K233-ac IFQEIyDkQYKSQFE
0 2 K233-ub IFQEIyDkQYKSQFE
0 1 K233 IFQEIyDKQYKSQFE
0 3 Q234 FQEIyDkQYKSQFEA
0 1 K236 EIyDkQYKSQFEAQk
0 1 K243-ac KSQFEAQkIWYEHRL
0 1 K243 KSQFEAQKIWYEHRL
0 1 S261-p MVAQAMKsEGGFIWA
0 1 Y319-p VTRHYRMyQkGQEtS
0 2 K321-ac RHYRMyQkGQEtSTN
0 2 K321-ub RHYRMyQkGQEtSTN
0 1 T325-p MyQkGQEtSTNPIAS
0 1 K345 RGLAHRAKLDNNkEL
0 1 K345 RGLAHRAKLDNNkEL
0 1 K350-ub RAKLDNNkELAFFAN
0 1 K381 KDLAACIKGLPNVQR
0 2 S389-p GLPNVQRsDyLNTFE
0 32 Y391-p PNVQRsDyLNTFEFM
0 2 K400 NTFEFMDKLGENLKI
0 1 K400 NTFEFMDKLGENLKI
  mouse

 
K4-ub ____MSRkIQGGSVV
T19 EMQGDEMTRIIWELI
K27-ub RIIWELIkEKLILPY
K27-sc RIIWELIkEKLILPY
Y34 kEKLILPYVELDLHS
S41 YVELDLHSYDLGIEN
Y42 VELDLHSYDLGIENR
K58-ac ATNDQVTkDAAEAIK
K58-ub ATNDQVTkDAAEAIK
K58-sc ATNDQVTkDAAEAIK
T75 NVGVKCATITPDEkR
T77 GVKCATITPDEkRVE
K81-ac ATITPDEkRVEEFKL
K81-ub ATITPDEkRVEEFKL
K93-ac FKLKQMWkSPNGTIR
K93-ub FKLKQMWkSPNGTIR
K126-ac RLVTGWVkPIIIGRH
K126-ub RLVTGWVkPIIIGRH
Y135-p IIIGRHAyGDQYRAT
Y139 RHAyGDQYRATDFVV
K151-ac FVVPGPGkVEITYTP
K151-ub FVVPGPGkVEITYTP
K151-sc FVVPGPGkVEITYTP
K159-ac VEITYTPkDGTQKVT
K159-ub VEITYTPkDGTQKVT
K159-sc VEITYTPkDGTQKVT
Y208 LSKGWPLYLSTkNTI
K212-ub WPLYLSTkNTILKKY
T214 LYLSTkNTILKKYDG
Y219 kNTILKKYDGRFkDI
K224-ac KKYDGRFkDIFQEIY
K224-ub KKYDGRFkDIFQEIY
Y231 kDIFQEIYDkkYkSQ
K233-ac IFQEIYDkkYkSQFE
K233-ub IFQEIYDkkYkSQFE
K233-sc IFQEIYDkkYkSQFE
K234-ac FQEIYDkkYkSQFEA
K236-ub EIYDkkYkSQFEAQk
K243 kSQFEAQKICYEHRL
K243-ub kSQFEAQkICYEHRL
S261 MVAQAMKSEGGFIWA
Y319 VTRHYRMYQkGQETS
K321 RHYRMYQKGQETSTN
K321-ub RHYRMYQkGQETSTN
T325 MYQkGQETSTNPIAS
K345-ac RGLAHRAkLDNNTEL
K345-ub RGLAHRAkLDNNTEL
T350 RAkLDNNTELSFFAK
K381-ub KDLAACIkGLPNVQR
S389-p GLPNVQRsDyLNTFE
Y391-p PNVQRsDyLNTFEFM
K400-ac NTFEFMDkLGENLKA
K400-ub NTFEFMDkLGENLKA
  rat

 
K4 ____MSRKIHGGSVV
T19 EMQGDEMTRIIWELI
K27 RIIWELIKEKLILPY
K27 RIIWELIKEKLILPY
Y34 KEKLILPYVELDLHS
S41 YVELDLHSYDLGIEN
Y42 VELDLHSYDLGIENR
K58 ATNDQVTKDAAEAIK
K58 ATNDQVTKDAAEAIK
K58 ATNDQVTKDAAEAIK
T75 NVGVKCATITPDEKR
T77 GVKCATITPDEKRVE
K81 ATITPDEKRVEEFKL
K81 ATITPDEKRVEEFKL
K93 FKLKQMWKSPNGTIR
K93 FKLKQMWKSPNGTIR
K126 RLVTGWVKPIIIGRH
K126 RLVTGWVKPIIIGRH
Y135 IIIGRHAYGDQYRAT
Y139 RHAYGDQYRATDFVV
K151 FVVPGPGKVEITYTP
K151 FVVPGPGKVEITYTP
K151 FVVPGPGKVEITYTP
K159 VEITYTPKDGSQKVT
K159 VEITYTPKDGSQKVT
K159 VEITYTPKDGSQKVT
Y208 LSKGWPLYLSTKNTI
K212 WPLYLSTKNTILKKY
T214 LYLSTKNTILKKYDG
Y219 KNTILKKYDGRFKDI
K224 KKYDGRFKDIFQEIy
K224 KKYDGRFKDIFQEIy
Y231-p KDIFQEIyDKQYKSK
K233 IFQEIyDKQYKSKFE
K233 IFQEIyDKQYKSKFE
K233 IFQEIyDKQYKSKFE
Q234 FQEIyDKQYKSKFEA
K236 EIyDKQYKSKFEAQK
K243 KSKFEAQKIWYEHRL
K243 KSKFEAQKIWYEHRL
S261 MVAQAMKSEGGFIWA
Y319 VTRHYRMYQKGQETS
K321 RHYRMYQKGQETSTN
K321 RHYRMYQKGQETSTN
T325 MYQKGQETSTNPIAS
K345 RGLAHRAKLDNNTEL
K345 RGLAHRAKLDNNTEL
T350 RAKLDNNTELSFFAN
K381 KDLAACIKGLPNVQR
S389 GLPNVQRSDYLNTFE
Y391 PNVQRSDYLNTFEFM
K400 NTFEFMDKLGENLKA
K400 NTFEFMDKLGENLKA
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