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Entries: CatalystActivityReference
Identifier Name
9643378 (R)-2-hydroxyglutarate dehydrogenase activity of D2HGDH [mitochondrial matrix] Identification of a dehydrogenase acting on D-2-hydroxyglutarate
9643509 (R)-2-hydroxyglutarate dehydrogenase activity of IDH1 R132mutant dimers [cytosol] Cancer-associated IDH1 mutations produce 2-hydroxyglutarate
9643909 (R)-3-amino-2-methylpropionate-pyruvate transaminase activity of Agxt2 tetramer [mitochondrial matrix] Purification, characterization and inhibition of D-3-aminoisobutyrate aminotransferase from the rat liver
9643840 (R,S)-4-hydroxy-2-oxoglutarate aldolase activity of HOGA1 tetramer [mitochondrial matrix] Structural and biochemical studies of human 4-hydroxy-2-oxoglutarate aldolase: implications for hydroxyproline metabolism in primary hyperoxaluria
9913890 (S)-2-hydroxy-acid oxidase activity of HAO2 tetramer [peroxisomal matrix] Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases
9643958 1,4-alpha-glucan branching enzyme activity of GBE1 [cytosol] Hepatic and neuromuscular forms of glycogen storage disease type IV caused by mutations in the same glycogen-branching enzyme gene.
9773946 1,5-anhydro-D-fructose reductase activity of AKR1E2 [cytosol] Characterization of htAKR, a novel gene product in the aldo-keto reductase family specifically expressed in human testis
9947644 1-phosphatidylinositol 4-kinase activator activity of ARF1/3:GTP [Golgi membrane] ARF mediates recruitment of PtdIns-4-OH kinase-beta and stimulates synthesis of PtdIns(4,5)P2 on the Golgi complex
9643715 1-phosphatidylinositol-5-phosphate 4-kinase activity of PIP4K2 dimers [cytosol] Evolutionarily conserved structural changes in phosphatidylinositol 5-phosphate 4-kinase (PI5P4K) isoforms are responsible for differences in enzyme activity and localization
9643321 1-pyrroline-5-carboxylate dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix] Cloning, characterization, and expression of cDNAs encoding human delta 1-pyrroline-5-carboxylate dehydrogenase
9929442 1-pyrroline-5-carboxylate dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix] Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline
9916389 13-lipoxin reductase activity of PTGR1 [cytosol] Oxidoreductases in lipoxin A4 metabolic inactivation: a novel role for 15-oxoprostaglandin 13-reductase/leukotriene B4 12-hydroxydehydrogenase in inflammation
9643943 2-(3-amino-3-carboxypropyl)histidine synthase activity of DPH1:DPH2:DPH3 [cytosol] Diphthamide biosynthesis requires an organic radical generated by an iron-sulphur enzyme
9643448 2-aminoadipate transaminase activity of AADAT dimer [mitochondrial matrix] Crystal structure of human kynurenine aminotransferase II
9643728 2-hydroxy-ATP hydrolase activity of NUDT1 [cytosol] The oxidized forms of dATP are substrates for the human MutT homologue, the hMTH1 protein
9643113 2-hydroxy-dATP hydrolase activity of NUDT1 [cytosol] The oxidized forms of dATP are substrates for the human MutT homologue, the hMTH1 protein
9643344 2-hydroxyglutarate dehydrogenase activity of L2HGDH [mitochondrial inner membrane] The gene mutated in l-2-hydroxyglutaric aciduria encodes l-2-hydroxyglutarate dehydrogenase
9643080 2-oxoglutarate-dependent dioxygenase activity of FIH1:AscH-:Fe2+ dimer [cytosol] Catalytic properties of the asparaginyl hydroxylase (FIH) in the oxygen sensing pathway are distinct from those of its prolyl 4-hydroxylases
9643846 2-oxoglutarate-dependent dioxygenase activity of TYW5:Fe2+ dimer [cytoplasm] Expanding role of the jumonji C domain as an RNA hydroxylase
9643383 3',5'-cyclic-nucleotide phosphodiesterase activity of p-S295-PDE3B [cytosol] Type III cGMP-inhibited cyclic nucleotide phosphodiesterases (PDE3 gene family)
9836941 3'-5' RNA helicase activity of SUPV3L1:PNPT1 [mitochondrial matrix] Human mitochondrial SUV3 and polynucleotide phosphorylase form a 330-kDa heteropentamer to cooperatively degrade double-stranded RNA with a 3'-to-5' directionality
9643714 3'-5'-exodeoxyribonuclease activity of MRN:CtIP [nucleoplasm] The 3' to 5' exonuclease activity of Mre 11 facilitates repair of DNA double-strand breaks
9945798 3-beta-hydroxysteroid 3-dehydrogenase (NADP+) activity of HSD17B7 [endoplasmic reticulum membrane] Closing the gap: identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis
9945785 3-beta-hydroxysteroid dehydrogenase [NAD(P)+]/C4-decarboxylase activity of NSDHL [endoplasmic reticulum membrane] The gene mutated in bare patches and striated mice encodes a novel 3beta-hydroxysteroid dehydrogenase
9914130 3-hydroxyacyl-CoA dehydratase activity of HSD17B4 dimer [peroxisomal matrix] Mutational spectrum of D-bifunctional protein deficiency and structure-based genotype-phenotype analysis
9643191 3-hydroxyacyl-CoA dehydrogenase activity of HSD17B10 tetramer [mitochondrial matrix] 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency Is caused by mutations in the HADH2 gene
9644046 3-hydroxyisobutyryl-CoA hydrolase activity of HIBCH [mitochondrial matrix] Primary structure and tissue-specific expression of human beta-hydroxyisobutyryl-coenzyme A hydrolase.
9644030 3R-hydroxyacyl-CoA dehydratase activity of PTPLs [endoplasmic reticulum membrane] Characterization of four mammalian 3-hydroxyacyl-CoA dehydratases involved in very long-chain fatty acid synthesis
9643183 4-alpha-glucanotransferase activity of AGL [cytosol] Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease.
9643835 4-alpha-hydroxytetrahydrobiopterin dehydratase activity of PCBD1 tetramer [cytosol] Phenyalanine hydroxylase-stimulating protein/pterin-4 alpha-carbinolamine dehydratase from rat and human liver. Purification, characterization, and complete amino acid sequence
9930618 4-hydroxyglutamate transaminase activity of GOT2 dimer [mitochondrial matrix] Metabolism of gamma-hydroxyglutamic acid. I. Conversion to alpha-hydroxy-gamma-ketoglutarate by purified glutamic-aspartic transaminase to rat liver
9643848 4-hydroxyphenylpyruvate dioxygenase activity of HPD:AscH-:Fe2+ dimer [cytosol] Mutations in the 4-hydroxyphenylpyruvate dioxygenase gene (HPD) in patients with tyrosinemia type III
9643469 4-trimethylammoniobutyraldehyde dehydrogenase activity of ALDH9A1 tetramer [cytosol] Kinetic and structural analysis of human ALDH9A1
9643089 5'-3' RNA exonuclease activity of XRN2 [nucleoplasm] Gradual processing of the ITS1 from the nucleolus to the cytoplasm during synthesis of the human 18S rRNA
9915451 5'-flap endonuclease activity of MGME1 [mitochondrial matrix] MGME1 processes flaps into ligatable nicks in concert with DNA polymerase γ during mtDNA replication
9643849 5'-nucleotidase activity of NT5C dimer [cytosol] Cytoplasmic 5'(3')-nucleotidase from human placenta
9643499 5'-nucleotidase activity of NT5C1A tetramer [cytosol] Human cytosolic 5'-nucleotidase I. Characterization and role in nucleoside analog resistance
9643271 5'-nucleotidase activity of NT5C2 tetramer [cytosol] Crystal structure of human cytosolic 5'-nucleotidase II: insights into allosteric regulation and substrate recognition
9643361 5'-nucleotidase activity of NT5C3 holoenzyme [cytosol] Pyrimidine nucleotidases from human erythrocyte possess phosphotransferase activities specific for pyrimidine nucleotides
9643223 5'-nucleotidase activity of NT5E:Zn2+ dimer [plasma membrane] Primary structure of human placental 5'-nucleotidase and identification of the glycolipid anchor in the mature form
9643417 5'-nucleotidase activity of NT5M dimer [mitochondrial matrix] Human mitochondrial 5'-deoxynucleotidase. Overproduction in cultured cells and functional aspects
9644031 6,7-dihydropteridine reductase activity of QDPR dimer [cytosol] Structure and expression of human dihydropteridine reductase
9643768 6-phosphofructo-2-kinase activity of PFKFB dimers [cytosol] Molecular cloning, sequence analysis, and expression of a human liver cDNA coding for fructose-6-P,2-kinase:fructose-2,6-bisphosphatase
9644014 6-phosphofructokinase activity of PFK tetramer [cytosol] Functional expression of human mutant phosphofructokinase in yeast: genetic defects in French Canadian and Swiss patients with phosphofructokinase deficiency
9643681 6-phosphogluconolactonase activity of PGLS [cytosol] Identification of the cDNA encoding human 6-phosphogluconolactonase, the enzyme catalyzing the second step of the pentose phosphate pathway(1)
9643633 7-dehydrocholesterol reductase activity of DHCR7 [endoplasmic reticulum membrane] Molecular cloning and expression of the human delta7-sterol reductase
9643814 8-hydroxy-dADP phosphatase activity of NUDT18-1 [cytosol] Human MTH3 (NUDT18) Protein Hydrolyzes Oxidized Forms of Guanosine and Deoxyguanosine Diphosphates: COMPARISON WITH MTH1 AND MTH2
9731238 8-oxo-(d)RTP hydrolase activity of NUDT1 [cytosol] Human MTH1 protein hydrolyzes the oxidized ribonucleotide, 2-hydroxy-ATP
9643199 8-oxo-7,8-dihydrodeoxyguanosine triphosphate pyrophosphatase activity of NUDT1 [cytosol] Cloning and expression of cDNA for a human enzyme that hydrolyzes 8-oxo-dGTP, a mutagenic substrate for DNA synthesis
9643300 8-oxo-7,8-dihydrodeoxyguanosine triphosphate pyrophosphatase activity of NUDT15 [cytosol] Human MTH3 (NUDT18) Protein Hydrolyzes Oxidized Forms of Guanosine and Deoxyguanosine Diphosphates: COMPARISON WITH MTH1 AND MTH2
9643491 8-oxo-dGDP phosphatase activity of NUDT15 [cytosol] Human MTH3 (NUDT18) Protein Hydrolyzes Oxidized Forms of Guanosine and Deoxyguanosine Diphosphates: COMPARISON WITH MTH1 AND MTH2
9643473 8-oxo-dGDP phosphatase activity of NUDT18-1 [cytosol] Human MTH3 (NUDT18) Protein Hydrolyzes Oxidized Forms of Guanosine and Deoxyguanosine Diphosphates: COMPARISON WITH MTH1 AND MTH2
9643858 8-oxo-dGDP phosphatase activity of NUDT18-1 [cytosol] Human MTH3 (NUDT18) Protein Hydrolyzes Oxidized Forms of Guanosine and Deoxyguanosine Diphosphates: COMPARISON WITH MTH1 AND MTH2
9915868 ABC-type transporter activity of ABCC1 [plasma membrane] The MRP gene encodes an ATP-dependent export pump for leukotriene C4 and structurally related conjugates
9759428 ABC-type vitamin B12 transporter activity of ABCC1 [plasma membrane] Vitamin B12 transport from food to the body's cells--a sophisticated, multistep pathway
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