Rabbit Anti-PCBD1 antibody | Sunlong Medical

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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 cofact

Cat:

SL19905R

Species Reactivity：

Mouse,(predicted: Human,Rat,Cow,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human PCBD1:2-50/104

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

WB=1:500-2000ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Product Overview：

Sample: Testis (Mouse) Lysate at 40 ugPrimary: Anti-PCBD1 (SL19905R) at 1/300 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 12 kDObserved 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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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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-SH2. 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 SH4-deficient hyperphenylalaninemia type D (HPASH4D) [MIM:212870]; also known as hyperphenylalaninemia with primapterinuria. HPASH4D 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: 1336 Mouse

Entrez Gene: 29700 Rat

Omim: 126090 Human

SwissProt: P61457 Human

SwissProt: P61458 Mouse

SwissProt: P61459 Rat

Unigene: 3192 Human

Unigene: 39473 Mouse

Unigene: 8236 Rat

Picture

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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