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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
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.
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
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
9643089 3'-5'-exoribonuclease activity of XRN2 [nucleoplasm] Gradual processing of the ITS1 from the nucleolus to the cytoplasm during synthesis of the human 18S rRNA
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
9643840 4-hydroxy-2-oxoglutarate aldolase activity of HOGA1 tetramer [mitochondrial matrix] Purification and properties of rat liver 2-keto-4-hydroxyglutarate aldolase
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
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
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
9730741 ABC-type vitamin B12 transporter activity of ABCD4 mutant:LBRD1 complexes [lysosomal membrane] Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism
9730748 ABC-type vitamin B12 transporter activity of ABCD4:LMBRD1 [lysosomal membrane] The lysosomal protein ABCD4 can transport vitamin B12 across liposomal membranes in vitro
9643529 ADP-ribose diphosphatase activity of NUDT5 dimer [cytosol] Crystal structures of human NUDT5 reveal insights into the structural basis of the substrate specificity
9643121 ADP-ribose diphosphatase activity of NUDT9 [mitochondrial matrix] The crystal structure and mutational analysis of human NUDT9
9643890 ADP-specific glucokinase activity of ADPGK:Mg2+ [extracellular region] T cell activation is driven by an ADP-dependent glucokinase linking enhanced glycolysis with mitochondrial reactive oxygen species generation
9644036 AMP deaminase activity of AMPD tetramers [cytosol] Platelet AMP deaminase. Purification and kinetic studies
9643356 AMP-activated protein kinase activity of Ampk:AMP [nucleoplasm] AMP-activated protein kinase (AMPK) action in skeletal muscle via direct phosphorylation of PGC-1alpha
9643243 ATPase activity of Spliceosomal Intermediate C (Bact) Complex [nucleoplasm] Contribution of DEAH-box protein DHX16 in human pre-mRNA splicing
9643605 ATPase activity of polyadenylated, capped mRNA:CBC:EJC:TREX:SRSF proteins [nucleoplasm] ATP is required for interactions between UAP56 and two conserved mRNA export proteins, Aly and CIP29, to assemble the TREX complex
9643627 ATPase activity, coupled to movement of substances of PEX2:PEX10:PEX12:Ub:PEX5L:PEX7:PEX13:PEX14:PEX1:PEX6:PEX26:ZFAND6 [peroxisomal membrane] The peroxisomal AAA ATPase complex prevents pexophagy and development of peroxisome biogenesis disorders
9643097 ATPase activity, coupled to movement of substances of PEX2:PEX10:PEX12:Ub:PEX5S,L:PEX1:PEX6:PEX26:ZFAND6 [peroxisomal membrane] AWP1/ZFAND6 functions in Pex5 export by interacting with cys-monoubiquitinated Pex5 and Pex6 AAA ATPase
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