HMBS antibody [N2C3]

Eine Lieferung in diese Region ist nicht möglich.

SKU:: GTX113460
Zusätzliche Namen:: hydroxymethylbilane synthase , PBG-D , PBGD , PORC , UPS
Anwendung:: WB
Konzentration:: 1 mg/ml
Physischer Zustand:: Liquid
Spezies-Reaktivität:: Human
Aufreinigung:: Affinity Purified
Lagerbedingungen:: -20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
Hersteller:: Genetex
Host:: Rabbit
Reaktivitäten:: Human, Mouse
Buffer:: 0.1M Tris, 0.1M Glycine, 10% Glycerol, 0.01% Thimerosal.
Immunogen:: Recombinant protein encompassing a sequence within the center region of human HMBS. The exact sequence is proprietary.
Klon:: N2C3
Uniprot:: P08397
Synonyme:: Hydroxymethylbilane synthase;PBG-D;PBGD;PORC;porphobilinogen deaminase;porphyria, acute; Chester type;pre-uroporphyrinogen synthase;UPS;uroporphyrinogen I synthase;uroporphyrinogen I synthetase
Weitere Details:: 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. [provided by RefSeq]