HMBS Antikörper

Produktnummer ABIN1386078

Dieses Anti-HMBS-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von HMBS in WB, IF (p) und IHC (p). Geeignet für Human, Maus und Ratte.

Kurzübersicht für HMBS Antikörper (ABIN1386078)

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HMBS Antikörper ist unkonjugiert

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

Produktdetails anti-HMBS Antikörper

Kreuzreaktivität: Human, Maus, Ratte

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human HMBS/PBGD

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
IHC-P 1:200-400
IF(IHC-P) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Konservierungsmittel: ProClin

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Haltbarkeit: 12 months

Details zu HMBS

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Andere Bezeichnung: HMBS

Hintergrund:

Synonyms: HEM3_HUMAN, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, Porphobilinogen deaminase, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase.

Background: PBGD, also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.

Gen-ID: 3145