Rabbit PAH Polyclonal Antibody | anti-PAH antibody

PAH (R400) Polyclonal Antibody

Cat. Num. AAA3004170

• Gene Names: PAH; PH; PKU; PKU1
• Reactivity: Human, Mouse, Rat
• Applications: Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: PAH; Polyclonal Antibody; PAH (R400) Polyclonal Antibody; Phenylalanine-4-hydroxylase; Phe-4-monooxygenase; anti-PAH antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: PAH (R400) polyclonal antibody detects endogenous levels of PAH protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 452

• Applicable Applications for anti-PAH antibody: Immunohistochemistry (IHC)
• Application Notes: IHC: 1:50-1:200
• Immunogen: Synthetic peptide, corresponding to amino acids 371-420 of Human PAH.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Immunohistochemistry (IHC)

(Immunohistochemistry (IHC) analyzes of PAH (R400) pAb in paraffin-embedded human brain tissue.)

Related Product Information for anti-PAH antibody

The PAH gene encodes the enzyme phenylalanine hydroxylase (PAH), which converts phenylalanine to tyrosine and is the rate-limiting enzyme in phenylalanine catabolism. Mammalian PAH is a soluble, homotetrameric protein which is abundantly expressed in human liver. Deficiency of PAH activity results in the autosomal recessive disorder phenylketonuria (PKU), which is characterized by mental retardation unless a low phenylalanine diet is introduced early in life. The PAH gene, which maps to human chromosome 12q23.2, contains all the genetic information necessary to code for functional PAH, demonstrating that a single gene is involved in the classic disease phenotype.

NCBI and Uniprot Product Information

• NCBI GI #: 129973
• NCBI GeneID: 5053
• UniProt Accession #: P00439
• Molecular Weight: ~ 52kDa
• NCBI Official Full Name: Phenylalanine-4-hydroxylase
• NCBI Official Synonym Full Names: phenylalanine hydroxylase
• NCBI Official Symbol: PAH
• NCBI Official Synonym Symbols: PH; PKU; PKU1
• NCBI Protein Information: phenylalanine-4-hydroxylase
• UniProt Protein Name: Phenylalanine-4-hydroxylase
• Protein Family: Phenylalanine-4-hydroxylase
• UniProt Gene Name: PAH
• UniProt Synonym Gene Names: PAH
• UniProt Entry Name: PH4H_HUMAN

NCBI Description

This gene encodes a member of the biopterin-dependent aromatic amino acid hydroxylase protein family. The encoded phenylalanine hydroxylase enzyme hydroxylates phenylalanine to tyrosine and is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. [provided by RefSeq, Aug 2017]

Uniprot Description

PAH: phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.

Protein type: EC 1.14.16.1; Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; Oxidoreductase

Chromosomal Location of Human Ortholog: 12q22-q24.2

Cellular Component: cytosol

Molecular Function: amino acid binding; iron ion binding; phenylalanine 4-monooxygenase activity

Biological Process: L-phenylalanine catabolic process; catecholamine biosynthetic process; amino acid biosynthetic process; neurotransmitter biosynthetic process

Disease: Phenylketonuria