LEPRE1 Antibody (monoclonal) (M01)
Mouse monoclonal antibody raised against a partial recombinant LEPRE1.
|Application ||WB, IHC, IF, E|
|Calculated MW||83394 Da|
|Other Names||Prolyl 3-hydroxylase 1, Growth suppressor 1, Leucine- and proline-enriched proteoglycan 1, Leprecan-1, LEPRE1, GROS1, P3H1|
|Target/Specificity||LEPRE1 (AAH15309, 1 a.a. ~ 100 a.a) partial 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||LEPRE1 Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures.|
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This gene encodes an enzyme that is a member of the collagen prolyl hydroxylase family. These enzymes are localized to the endoplasmic reticulum and their activity is required for proper collagen synthesis and assembly. Mutations in this gene are associated with osteogenesis imperfecta type VIII. Two alternatively spliced transcript variants encoding different isoforms have been described. Other variants may exist, but their biological validity has not been determined.
1.Proteomic dissection of the VHL interactome.Lai Y, Song M, Hakala K, Weintraub ST, Shiio Y.J Proteome Res. 2011 Oct 11.2.Severe osteogenesis imperfecta in cyclophilin B-deficient mice.Choi JW, Sutor SL, Lindquist L, Evans GL, Madden BJ, Bergen HR 3rd, Hefferan TE, Yaszemski MJ, Bram RJ.PLoS Genet. 2009 Dec;5(12):e1000750. Epub 2009 Dec 4.3.Prolyl 3-hydroxylase 1 deficiency causes a recessive metabolic bone disorder resembling lethal/severe osteogenesis imperfecta.Cabral WA, Chang W, Barnes AM, Weis M, Scott MA, Leikin S, Makareeva E, Kuznetsova NV, Rosenbaum KN, Tifft CJ, Bulas DI, Kozma C, Smith PA, Eyre DR, Marini JC.Nat Genet. 2007 Mar;39(3):359-65. Epub 2007 Feb 4.
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