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Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1)

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PCD; PHS; DCOH; PCBD; 6-Pyruvoyl-Tetrahydropterin Synthase; Dimerization Cofactor Of Hepatocyte Nuclear Factor 1 Alpha; Phenylalanine hydroxylase-stimulating protein

Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1)
CCC J748
HGNC 8646
MGI 94873
PubMed 24204001
RGD 3263
UniProt P61457
Wiki PCBD1
PCBD1 encodes pterin-4 alpha-carbinolamine dehydratase, an enzyme involved in phenylalanine hydroxylation. A deficiency of this enzyme leads to hyperphenylalaninemia.
human PCBD/DCOH was present predominantly in liver and kidney, with significant amounts in testis and ovary, trace amounts in lung, and undetectable levels in whole brain, heart, and spleen. It was expressed in all of the cells that were examined. It was also present in the nucleus of HeLa cells, which lack HNF1, and in the cytoplasm of fibroblasts, that have little or no BH4. PCBD is a widely distributed protein, with differential expression in different tissues and cells that is regulated at both the transcriptional and translational levels.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Pterin 4 Alpha Carbinolamine Dehydratase 1 (PCBD1) ELISA Kit Customized Service Offer
  1. "Characterization of a cofactor that regulates dimerization of a mammalian homeodomain protein."Science 254:1762-1767(1991) [PubMed] [Europe PMC] [Abstract]
  2. "Characterization of the human PCBD gene encoding the bifunctional protein pterin-4 alpha-carbinolamine dehydratase/dimerization cofactor for the transcription factor HNF-1 alpha."Biochem. Biophys. Res. Commun. 210:966-973(1995) [PubMed] [Europe PMC] [Abstract]
  3. "Characterization of expression of the gene for human pterin carbinolamine dehydratase/dimerization cofactor of HNF1."DNA Cell Biol. 18:243-252(1999) [PubMed] [Europe PMC] [Abstract]
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  5. "Phenylalanine hydroxylase-stimulating protein/pterin-4 alpha-carbinolamine dehydratase from rat and human liver. Purification, characterization, and complete amino acid sequence."J. Biol. Chem. 268:4828-4831(1993) [PubMed] [Europe PMC] [Abstract]
  6. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  7. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  8. "Mutation in the 4a-carbinolamine dehydratase gene leads to mild hyperphenylalaninemia with defective cofactor metabolism."Am. J. Hum. Genet. 53:768-774(1993) [PubMed] [Europe PMC] [Abstract]
  9. "Mutations in the pterin-4alpha-carbinolamine dehydratase (PCBD) gene cause a benign form of hyperphenylalaninemia."Hum. Genet. 103:162-167(1998) [PubMed] [Europe PMC] [Abstract]