HMBS antibody [N2C3]

Cannot supply to this region.

SKU:: GTX113460
Additional Names:: hydroxymethylbilane synthase , PBG-D , PBGD , PORC , UPS
Application:: WB
Concentration:: 1 mg/ml
Physical State:: Liquid
Species Reactivity:: Human
Purification:: Affinity Purified
Storage Conditions:: -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.
Supplier:: Genetex
Host:: Rabbit
Reactivities:: 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.
Clone:: N2C3
Uniprot:: P08397
Synonyms:: Hydroxymethylbilane synthase;PBG-D;PBGD;PORC;porphobilinogen deaminase;porphyria, acute; Chester type;pre-uroporphyrinogen synthase;UPS;uroporphyrinogen I synthase;uroporphyrinogen I synthetase
Extra 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]