Details of Protein

General Information of Protein (ID: PRT00536)
Name Oxalosuccinate decarboxylase (IDH1)
Synonyms   Click to Show/Hide Synonyms of This Protein
IDH; Cytosolic NADP-isocitrate dehydrogenase; IDP; NADP(+)-specific ICDH; Oxalosuccinate decarboxylase; IDH1; PICD
Gene Name IDH1 Gene ID
3417
UniProt ID
O75874
Family Oxidoreductases (EC 1)
EC Number   EC: 1.1.1.42  (Click to Show/Hide the Complete EC Tree)
Oxidoreductase
CH-OH donor oxidoreductase
NAD/NADP oxidoreductase
EC: 1.1.1.42
  Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein
Sequence
MSKKISGGSVVEMQGDEMTRIIWELIKEKLIFPYVELDLHSYDLGIENRDATNDQVTKDA
AEAIKKHNVGVKCATITPDEKRVEEFKLKQMWKSPNGTIRNILGGTVFREAIICKNIPRL
VSGWVKPIIIGRHAYGDQYRATDFVVPGPGKVEITYTPSDGTQKVTYLVHNFEEGGGVAM
GMYNQDKSIEDFAHSSFQMALSKGWPLYLSTKNTILKKYDGRFKDIFQEIYDKQYKSQFE
AQKIWYEHRLIDDMVAQAMKSEGGFIWACKNYDGDVQSDSVAQGYGSLGMMTSVLVCPDG
KTVEAEAAHGTVTRHYRMYQKGQETSTNPIASIFAWTRGLAHRAKLDNNKELAFFANALE
EVSIETIEAGFMTKDLAACIKGLPNVQRSDYLNTFEFMDKLGENLKIKLAQAKL
Structure
1T09 ; 1T0L ; 3INM ; 3MAP ; 3MAR ; 3MAS ; 4I3K ; 4I3L ; 4KZO ; 4L03 ; 4L04 ; 4L06 ; 4UMX ; 4UMY ; 4XRX ; 4XS3 ; 5DE1 ; 5GIR ; 5K10 ; 5K11 ; 5L57 ; 5L58 ; 5LGE ; 5SUN ; 5SVF ; 5TQH ; 5YFM ; 5YFN ; 6ADG ; 6B0Z ; 6BKX ; 6BKY ; 6BKZ ; 6BL0 ; 6BL1 ; 6BL2 ; 6IO0 ; 6O2Y ; 6O2Z ; 6PAY ; 6Q6F ; 6U4J ; 6VEI ; 6VG0
Regulatory Network
Full List of Metabolite(s) Regulated by This Protein
      Organic acids and derivatives
            2-Hydroxyglutarate Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1], [2], [3]
                      Introduced Variation Mutation (R132H) of IDH1
                      Induced Change 2-Hydroxyglutarate concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Brain cancer [ICD-11: 2A00]
                      Details It is reported that R132H mutation of IDH1 leads to the increase of 2-hydroxyglutarate levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [4]
                      Introduced Variation Mutation (protein: R132-R132H) of IDH1
                      Induced Change 2-Hydroxyglutarate concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Acute myeloid leukaemia [ICD-11: 2A60]
                      Details It is reported that mutation (protein with R132-R132H) of IDH1 leads to the increase of 2-hydroxyglutarate levels compared with control group.
            Oxoglutaric acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [5]
                      Introduced Variation Mutation (R132H) of IDH1
                      Induced Change Oxoglutaric acid concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Brain cancer [ICD-11: 2A00]
                      Details It is reported that R132H mutation of IDH1 leads to the decrease of oxoglutaric acid levels compared with control group.
References
1 Cancer-associated IDH1 mutations produce 2-hydroxyglutarate. Nature. 2009 Dec 10;462(7274):739-44.
2 Mutant IDH1 Promotes Glioma Formation In Vivo. Cell Rep. 2018 May 1;23(5):1553-1564.
3 Autophagy and oxidative stress in gliomas with IDH1 mutations. Acta Neuropathol. 2014 Feb;127(2):221-33.
4 The common feature of leukemia-associated IDH1 and IDH2 mutations is a neomorphic enzyme activity converting alpha-ketoglutarate to 2-hydroxyglutarate. Cancer Cell. 2010 Mar 16;17(3):225-34.
5 Glioma-derived mutations in IDH1 dominantly inhibit IDH1 catalytic activity and induce HIF-1alpha. Science. 2009 Apr 10;324(5924):261-5.

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