HMBS Antikörper (Middle Region)

Produktnummer ABIN2785719

Produktdetails anti-HMBS Antikörper

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Bindungsspezifität: Middle Region

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

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HMBS Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Sequenz: SSLRRAAQLQ RKFPHLEFRS IRGNLNTRLR KLDEQQEFSA IILATAGLQR

Homologie: Cow: 93%, Dog: 93%, Goat: 93%, Guinea Pig: 100%, Horse: 93%, Human: 100%, Mouse: 93%, Rabbit: 100%, Rat: 100%, Sheep: 93%, Zebrafish: 91%

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 middle region of human HMBS

Anwendungsinformationen

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

Kommentare:

Antigen size: 361 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 (ABIN2785719)

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)

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: 361

Molekulargewicht: 40 kDa

Gen-ID: 3145

NCBI Accession: NM_000190, NP_000181

UniProt: P08397