Phenylalanine Hydroxylase anticorps

N° du produit ABIN498690

Cet anticorps anti-Phenylalanine Hydroxylase Polyclonal Lapin (ABIN498690) détecte spécifiquement Phenylalanine Hydroxylase dans WB et IHC (p).

Aperçu rapide pour Phenylalanine Hydroxylase anticorps (ABIN498690)

Antigène: Phenylalanine Hydroxylase

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Phenylalanine Hydroxylase est non-conjugé

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

Détail du produit anti-Phenylalanine Hydroxylase anticorps

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

Réactivité croisée (Details): Species reactivity (expected):Mouse and Rat.
Species reactivity (tested):Human.

Purification: Affinity chromatography

Pureté: > 95 % by SDS-PAGE

Information d'application

Indications d'application: 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

Stockage

Concentration: 1,0 mg/mL

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

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Conseil sur la manipulation: Avoid repeated freezing and thawing.

Stock: 4 °C/-20 °C

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

Détails sur Phenylalanine Hydroxylase

Antigène: Phenylalanine Hydroxylase

Autre désignation: PAH

Classe de substances: Chemical

Sujet: 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

Poids moléculaire: approx. 55 kDa

ID gène: 5053

NCBI Accession: NP_000268

UniProt: P00439