Phenylalanine Hydroxylase antibody

Catalog No. ABIN498690

The Rabbit Polyclonal anti-Phenylalanine Hydroxylase antibody (ABIN498690) specifically detects Phenylalanine Hydroxylase in WB and IHC (p).

Quick Overview for Phenylalanine Hydroxylase antibody (ABIN498690)

Target: Phenylalanine Hydroxylase

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Phenylalanine Hydroxylase antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Product Details anti-Phenylalanine Hydroxylase Antibody

Specificity: This antibody detects endogenous levels of PAH protein. (region surrounding Arg400)

Cross-Reactivity (Details): Species reactivity (expected):Mouse and Rat.
Species reactivity (tested):Human.

Purification: Affinity chromatography

Purity: > 95 % by SDS-PAGE

Application Details

Application Notes: Western Blot: 1/500 - 1/1000. Immunohistochemistry: 1/50 - 1/200.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user.

Restrictions: For Research Use only

Handling

Concentration: 1,0 mg/mL

Buffer: Phosphate buffered saline (PBS), pH 7.2., 0.05 % sodium azide

Preservative: Sodium azide

Precaution of Use: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handling Advice: Avoid repeated freezing and thawing.

Storage: 4 °C/-20 °C

Storage Comment: Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.

Target Details for Phenylalanine Hydroxylase

Target: Phenylalanine Hydroxylase

Alternative Name: PAH

Target Type: Chemical

Background: 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. Numerous mutations can impair the PAH gene, which result in decreased enzyme activity and give rise to varying degrees of PKU. Multiple isozymes of PAH have been reported to exist, but these are most likely allelic variants of PAH that produce protein subunits with slightly different charge and electrophoretic migration.Synonyms: PH, PKU, PKU1, Phe-4-monooxygenase, Phenylalanine 4-monooxygenase, Phenylalanine Hydroxylase, Phenylalanine-4-hydroxylase

Molecular Weight: approx. 55 kDa

Gene ID: 5053

NCBI Accession: NP_000268

UniProt: P00439