Antibody Name:	Anti-PCBD1 Antibody
Antibody SKU:	CAB6392
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-104 of human PCBD1 (NP_000272.1).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:10 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	SW620, Mouse liver, Mouse kidney, Mouse pancreas, Rat liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-104 of human PCBD1 (NP_000272.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	MAGK AHRL SAEE RDQL LPNL RAVG WNEL EGRD AIFK QFHF KDFN RAFG FMTR VALQ AEKL DHHP EWFN VYNK VHIT LSTH ECAG LSER DINL ASFI EQVA VSMT
Gene ID:	5092
Uniprot:	P61457
Cellular Location:	Cytoplasm, Nucleus
Calculated MW:	12kDa
Observed MW:	12kDa

Synonyms:

PCBD1, DCOH, PCBD, PCD, PHS

Background:

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.

UniProt Protein Function:	PCBD1: 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. Defects in PCBD1 are the cause of BH4-deficient hyperphenylalaninemia type D (HPABH4D); also known as hyperphenylalaninemia with primapterinuria. HPABH4D is characterized by the excretion of 7-substituted pterins in the urine of affected patients. Belongs to the pterin-4-alpha-carbinolamine dehydratase family.
UniProt Protein Details:	Protein type:Transcription, coactivator/corepressor; Oxidoreductase; Lyase; EC 4.2.1.96 Chromosomal Location of Human Ortholog: 10q22 Cellular Component: nucleoplasm; cytoplasm; cytosol Molecular Function:identical protein binding; protein binding; 4-alpha-hydroxytetrahydrobiopterin dehydratase activity; transcription coactivator activity; phenylalanine 4-monooxygenase activity Biological Process: L-phenylalanine catabolic process; tetrahydrobiopterin biosynthetic process; transcription, DNA-dependent; protein heterooligomerization; regulation of protein homodimerization activity; positive regulation of transcription, DNA-dependent; protein homotetramerization Disease: Hyperphenylalaninemia, Bh4-deficient, D
NCBI Summary:	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]
UniProt Code:	P61457
NCBI GenInfo Identifier:	47606444
NCBI Gene ID:	5092
NCBI Accession:	P61457.2
UniProt Secondary Accession:	P61457,P70519, P80095, Q9D930,
UniProt Related Accession:	P61457
Molecular Weight:	104
NCBI Full Name:	Pterin-4-alpha-carbinolamine dehydratase
NCBI Synonym Full Names:	pterin-4 alpha-carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha
NCBI Official Symbol:	PCBD1
NCBI Official Synonym Symbols:	PCD; PHS; DCOH; PCBD
NCBI Protein Information:	pterin-4-alpha-carbinolamine dehydratase; dimerizing cofactor for HNF1; 4-alpha-hydroxy-tetrahydropterin dehydratase; phenylalanine hydroxylase-stimulating protein; 6-pyruvoyl-tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1)
UniProt Protein Name:	Pterin-4-alpha-carbinolamine dehydratase
UniProt Synonym Protein Names:	4-alpha-hydroxy-tetrahydropterin dehydratase; Dimerization cofactor of hepatocyte nuclear factor 1-alpha; DCoH; Dimerization cofactor of HNF1; Phenylalanine hydroxylase-stimulating protein; Pterin carbinolamine dehydratase; PCD
Protein Family:	Pterin-4-alpha-carbinolamine dehydratase
UniProt Gene Name:	PCBD1
UniProt Entry Name:	PHS_HUMAN

	Western blot analysis of extracts of various cell lines, using PCBD1 antibody (CAB6392) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
	Immunofluorescence analysis of MCF7 cells using PCBD1 antibody (CAB6392). Blue: DAPI for nuclear staining.