HMBS Antikörper (Alexa Fluor 750)

Produktnummer ABIN5004749

Produktdetails anti-HMBS Antikörper (Alexa Fluor 750)

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HMBS Antikörper ist konjugiert mit Alexa Fluor 750

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

Kreuzreaktivität: Human, Maus, Ratte

Aufreinigung: Purified by Protein A.

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % 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: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu HMBS

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Andere Bezeichnung: HMBS (HMBS Produkte)

Synonyme: PBG-D antikoerper, PBGD antikoerper, PORC antikoerper, UPS antikoerper, URO-S antikoerper, hemC antikoerper, pbg-d antikoerper, pbgd antikoerper, ups antikoerper, SPAC806.01 antikoerper, DDBDRAFT_0186148 antikoerper, DDBDRAFT_0231417 antikoerper, DDB_0186148 antikoerper, DDB_0231417 antikoerper, Hmbs antikoerper, NV50236 antikoerper, T25658 antikoerper, Ups antikoerper, Uros1 antikoerper, hmbs antikoerper, zgc:64128 antikoerper, hmbsl antikoerper, id:ibd5004 antikoerper, im:7140060 antikoerper, zgc:110690 antikoerper, hydroxymethylbilane synthase antikoerper, hydroxymethylbilane synthase S homeolog antikoerper, hydroxymethylbilane synthase L homeolog antikoerper, hydroxymethylbilane synthase (predicted) antikoerper, Hydroxymethylbilane synthase antikoerper, hydroxymethylbilane synthase a antikoerper, hydroxymethylbilane synthase, b antikoerper, HMBS antikoerper, Hmbs antikoerper, hmbs antikoerper, hmbs.2.S antikoerper, hmbs.L antikoerper, CNC02250 antikoerper, hem3 antikoerper, hemC antikoerper, Trad_0335 antikoerper, Plabr_2100 antikoerper, Sgly_3113 antikoerper, SMLT_RS19660 antikoerper, PMI_RS16580 antikoerper, hmbsa antikoerper, hmbsb antikoerper

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