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Product Name PCBD1 Chinese Name 蝶呤甲醇胺脱水酶1抗体 Alias 4 alpha hydroxy tetrahydropterin dehydratase; 4-alpha-hydroxy-tetrahydropterin dehydratase; 6 pyruvoyl tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1); 6 pyruvoyl tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha; DCoH; Dimerization cofactor of hepatic nuclear factor 1 alpha; Dimerization cofactor of hepatocyte nuclear factor 1 alpha; Dimerization cofactor of hepatocyte nuclear factor 1-alpha; Dimerization cofactor of HNF1; Dimerizing cofactor for HNF1; PCBD 1; PCBD; PCBD1; PCD; Phenylalanine hydroxylase stimulating protein; Phenylalanine hydroxylase-stimulating protein; PHS; PHS_HUMAN; Pterin 4 alpha carbinolamine dehydratase; Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1); Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha; Pterin carbinolamine dehydratase; Pterin-4-alpha-carbinolamine dehydratase. Research Area Tumour Cell biology Signal transduction Epigenetics Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Human, Rat, Cow, Rabbit, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 12kDa Cellular localization The nucleus cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human PCBD1: 2-50/104 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail This gene encodes a member of the pterin-4-alpha-carbinolamine dehydratase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. The encoded protein functions as both a dehydratase involved in tetrahydrobiopterin biosynthesis, and as a cofactor for HNF1A-dependent transcription. A deficiency of this enzyme leads to hyperphenylalaninemia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]
Function:
Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2. Coactivator for HNF1A-dependent transcription. Regulates the dimerization of homeodomain protein HNF1A and enhances its transcriptional activity.
Subcellular Location:
Cytoplasm. Nucleus. Cytoplasmic and/or nuclear.
DISEASE:
Defects in PCBD1 are the cause of BH4-deficient hyperphenylalaninemia type D (HPABH4D) [MIM:264070]; also known as hyperphenylalaninemia with primapterinuria. HPABH4D is characterized by the excretion of 7-substituted pterins in the urine of affected patients.
Similarity:
Belongs to the pterin-4-alpha-carbinolamine dehydratase family.
SWISS:
P61457
Gene ID:
5092
Database links:Entrez Gene: 395729 Chicken
Entrez Gene: 5092 Human
Entrez Gene: 13180 Mouse
Omim: 126090 Human
SwissProt: P61457 Human
SwissProt: P61458 Mouse
Unigene: 3192 Human
Unigene: 39473 Mouse
Unigene: 81180 Rat
Product Picture Sample:
Testis (Mouse) Lysate at 40 ug
Primary: Anti-PCBD1 (SL19905R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 12 kD
Observed band size: 12 kD
Paraformaldehyde-fixed, paraffin embedded (Mouse colon); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PCBD1) Polyclonal Antibody, Unconjugated (SL19905R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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