HMBS Antikörper (N-Term)

Produktnummer ABIN2785718

Produktdetails anti-HMBS Antikörper

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Bindungsspezifität: N-Term

Reaktivität: Human, Maus, Ratte, Kaninchen, Hund, Pferd, Meerschweinchen, Rind (Kuh), Zebrafisch (Danio rerio), Schaf

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HMBS Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Sequenz: MRVIRVGTRK SQLARIQTDS VVATLKASYP GLQFEIIAMS TTGDKILDTA

Homologie: Cow: 100%, Dog: 100%, Guinea Pig: 100%, Horse: 100%, Human: 100%, Mouse: 100%, Rabbit: 100%, Rat: 100%, Sheep: 100%, Zebrafish: 100%

Produktmerkmale: This is a rabbit polyclonal antibody against HMBS. It was validated on Western Blot using a cell lysate as a positive control.

Aufreinigung: Affinity Purified

Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human HMBS

Anwendungsinformationen

Applikationshinweise: Optimal working dilutions should be determined experimentally by the investigator.

Kommentare:

Antigen size: 344 AA

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: Lot specific

Buffer: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

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 freeze-thaw cycles.

Lagerung: -20 °C

Informationen zur Lagerung: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.

Referenzen für anti-HMBS Antikörper (ABIN2785718)

Kuo, Lee, Chuang, Huang: "Acute intermittent porphyria with peripheral neuropathy: a follow-up study after hematin treatment." in: Journal of the neurological sciences, Vol. 260, Issue 1-2, pp. 231-5, (2007) (PubMed).

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: HMBS is a member of the hydroxymethylbilane synthase superfamily. It is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria.This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described.
Alias Symbols: PBG-D, PBGD, UPS, PORC
Protein Interaction Partner: RANBP9, APP, UBC,
Protein Size: 344

Molekulargewicht: 38 kDa

Gen-ID: 3145

NCBI Accession: NM_001024382, NP_001019553

UniProt: P08397