UROS Antibody (monoclonal) (M01)
Mouse monoclonal antibody raised against a full length recombinant UROS.
|Application ||WB, E|
|Calculated MW||28628 Da|
|Other Names||Uroporphyrinogen-III synthase, UROIIIS, UROS, Hydroxymethylbilane hydrolyase [cyclizing], Uroporphyrinogen-III cosynthase, UROS|
|Target/Specificity||UROS (AAH02573, 1 a.a. ~ 265 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.|
|Format||Clear, colorless solution in phosphate buffered saline, pH 7.2 .|
|Storage||Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.|
|Precautions||UROS Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures.|
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Provided below are standard protocols that you may find useful for product applications.
The protein encoded by this gene catalyzes the fourth step of porphyrin biosynthesis in the heme biosynthetic pathway. Defects in this gene cause congenital erythropoietic porphyria (Gunther's disease).
Congenital erythropoietic porphyria: a novel uroporphyrinogen III synthase branchpoint mutation reveals underlying wild-type alternatively spliced transcripts. Bishop DF, et al. Blood, 2010 Feb 4. PMID 19965637.Uroporphyrinogen III synthase mutations related to congenital erythropoietic porphyria identify a key helix for protein stability. Fortian A, et al. Biochemistry, 2009 Jan 20. PMID 19099412.Hypoxia decreases the expression of the two enzymes responsible for producing linear and cyclic tetrapyrroles in the heme biosynthetic pathway. Vargas PD, et al. FEBS J, 2008 Dec. PMID 19021769.Human uroporphyrinogen III synthase: NMR-based mapping of the active site. Cunha L, et al. Proteins, 2008 May 1. PMID 18004775.Identification of mutations in the uroporphyrinogen III cosynthase gene in German patients with congenital erythropoietic porphyria. Wiederholt T, et al. Physiol Res, 2006. PMID 17298225.
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