Preferred Label : pterin-4a-carbinolamine dehydratase;

MeSH note : bifunctional protein which converts pterin-4a-carbinolamine to quinoid dihydrobiopterin and functions as dimerization cofactor for HNF-1alpha; deficiency results in an atypical form of phenylketonuria; RefSeq NM_025273 (mouse); NM_000281 (human); NM_001007601 (rat);

CISMeF synonym : Pcbd1 protein, rat; PhhB protein, pseudomonas aeruginosa; 6-pyruvoyl-tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1) protein, human;

MeSH synonym : pt-c dehydratase; 4a-hydroxytetrahydrobiopterin dehydratase; dimerization cofactor of HNF-1alpha; 4a-carbinolamine dehydratase; pterin-4alpha-carbinolamine dehydratase; 4alpha-carbinolamine dehydratase; phenylalanine hydroxylase-stimulating protein;

MeSH hyponym : dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1) 1, rat; pterin 4 alpha carbinolamine dehydratase, rat; DCoH protein, human; phenylalanine hydroxylase-stimulating protein, human; pterin-4 alpha-carbinolamine dehydratase, human; 6-pyruvoyl-tetrahydropterin synthase-dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1) protein, human; dimerization cofactor of hepatic nuclear factor 1-alpha, human; pterin-4 alpha-carbinolamine dehydratase, mouse; dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1) 1, mouse; Dcoh protein, mouse;

MeSH Related Number : PCBD1 protein, human; Pcbd1 protein, mouse; 146588-46-7 (human DCoH); 146588-47-8 (mouse DCoH);

Registry Number MeSH : EC 4.2.1.96;

Related CAS MeSH : 146588-46-7 (PCBD1 protein, human); 146588-47-8 (Pcbd1 protein, mouse);

related registry number list (CISMeF) : 146588-46-7 (human DCoH); 146588-47-8 (mouse DCoH);

Is substance : O;

UNII : EC 4.2.1.96;

Details


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http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=1578
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12/06/2024


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