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HMBS Antibody (Center) Blocking peptide

Synthetic peptide

     
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Product Information
Primary Accession P08397
Clone Names 90121163
Additional Information
Gene ID 3145
Other Names Porphobilinogen deaminase, PBG-D, Hydroxymethylbilane synthase, HMBS, Pre-uroporphyrinogen synthase, HMBS, PBGD, UPS
Format Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name HMBS
Synonyms PBGD, UPS
Function As part of the heme biosynthetic pathway, catalyzes the sequential polymerization of four molecules of porphobilinogen to form hydroxymethylbilane, also known as preuroporphyrinogen (PubMed:18936296, PubMed:19138865, PubMed:23815679, PubMed:18004775). Catalysis begins with the assembly of the dipyrromethane cofactor by the apoenzyme from two molecules of porphobilinogen or from preuroporphyrinogen. The covalently linked cofactor acts as a primer, around which the tetrapyrrole product is assembled. In the last step of catalysis, the product, preuroporphyrinogen, is released, leaving the cofactor bound to the holodeaminase intact (PubMed:18936296).
Cellular Location Cytoplasm.
Tissue Location [Isoform 1]: Is ubiquitously expressed.
Research Areas
Citations (0)
citation

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Background

This gene encodes a member of the hydroxymethylbilanesynthase superfamily. The encoded protein is the third enzyme ofthe heme biosynthetic pathway and catalyzes the head to tailcondensation of four porphobilinogen molecules into the linearhydroxymethylbilane. Mutations in this gene are associated with theautosomal dominant disease acute intermittent porphyria.Alternatively spliced transcript variants encoding differentisoforms have been described.

References

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)Surin, V.L., et al. Genetika 46(4):540-552(2010)Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)Gill, R., et al. Biochem. J. 420(1):17-25(2009)Ulbrichova, D., et al. FEBS J. 276(7):2106-2115(2009)

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$ 277.78
Cat# BP12917c
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