Phenylalanine Hydroxylase Antikörper

Produktnummer ABIN498690

Der Kaninchen Polyklonal anti-Phenylalanine Hydroxylase Antikörper (ABIN498690) detektiert spezifisch Phenylalanine Hydroxylase in WB und IHC (p).

Kurzübersicht für Phenylalanine Hydroxylase Antikörper (ABIN498690)

Target: Phenylalanine Hydroxylase

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Phenylalanine Hydroxylase Antikörper ist unkonjugiert

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

Produktdetails anti-Phenylalanine Hydroxylase Antikörper

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

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

Aufreinigung: Affinity chromatography

Reinheit: > 95 % by SDS-PAGE

Anwendungsinformationen

Applikationshinweise: 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.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Konzentration: 1,0 mg/mL

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

Konservierungsmittel: Sodium azide

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

Handhabung: Avoid repeated freezing and thawing.

Lagerung: 4 °C/-20 °C

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

Details zu Phenylalanine Hydroxylase

Target: Phenylalanine Hydroxylase

Andere Bezeichnung: PAH

Substanzklasse: Chemical

Hintergrund: 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

Molekulargewicht: approx. 55 kDa

Gen-ID: 5053

NCBI Accession: NP_000268

UniProt: P00439