HMBS Antikörper

Produktnummer ABIN1386078

Der Kaninchen Polyklonal anti-HMBS Antikörper (ABIN1386078) detektiert spezifisch HMBS in IF, IHC (p) und IHC (fro). Dieser Antikörper reagiert spezifisch mit Proben aus Human.

Kurzübersicht für HMBS Antikörper (ABIN1386078)

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HMBS Antikörper ist unkonjugiert

Applikation: Immunofluorescence (IF), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Produktdetails anti-HMBS Antikörper

Verwendungszweck: HMBS Polyclonal Antibody

Kreuzreaktivität: Human, Maus, Ratte

Aufreinigung: Purified by Protein A.

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

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 4C. Store at -20C 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