LEPRE1 purified MaxPab mouse polyclonal antibody (B01P)
产品名称： LEPRE1 purified MaxPab mouse polyclonal antibody (B01P)
英文名称： LEPRE1 purified MaxPab mouse polyclonal antibody (B01P)
产品编号： H00064175-B01P
产品价格： null
产品产地： 台湾
品牌商标： Abnova
更新时间： null
使用范围：
亚诺法生技股份有限公司（Abnova)
- 联系人 :
- 地址 : 台湾台北市内湖区洲子街 108 号 9 楼
- 邮编 : 11493
- 所在区域 : 台湾
- 电话 : +886-920**1152 点击查看
- 传真 : 点击查看
- 邮箱 : sales@abnova.com.tw
- Specification
- Product Description:
- Mouse polyclonal antibody raised against a full-length human LEPRE1 protein.
- Immunogen:
- LEPRE1 (AAH15309, 1 a.a. ~ 390 a.a) full-length human protein.
- Sequence:
- MLGEEHTRSIGPRESAKEYRQRSLLEKELLFFAYDVFGIPFVDPDSWTPEEVIPKRLQEKQKSERETAVRISQEIGNLMKEIETLVEEKTKESLDVSRLTREGGPLLYEGISLTMNSKLLNGSQRVVMDGVISDHECQELQRLTNVAATSGDGYRGQTSPHTPNEKFYGVTVFKALKLGQEGKVPLQSAHLYYNVTEKVRRIMESYFRLDTPLYFSYSHLVCRTAIEEVQAERKDDSHPVHVDNCILNAETLVCVKEPPAYTFRDYSAILYLNGDFDGGNFYFTELDAKTVTAEVQPQCGRAVGFSSGTENPHGVKAVTRGQRCAIALWFTLDPRHSERDRVQADDLVKMLFSPEEMDLSQEQPLDAQQGPPEPAQESLSGSESKPKDEL
- Host:
- Mouse
- Reactivity:
- Human
- Storage Buffer:
- In 1x PBS, pH 7.2
- Storage Instruction:
- Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
- Quality Control Testing:
- Antibody reactive against mammalian transfected lysate.
- MSDS:
Download
- Publication Reference
- 1.
- A Novel Mutation in LEPRE1 That Eliminates Only the KDEL ER- Retrieval Sequence Causes Non-Lethal Osteogenesis Imperfecta.
Takagi M, Ishii T, Barnes AM, Weis M, Amano N, Tanaka M, Fukuzawa R, Nishimura G, Eyre DR, Marini JC, Hasegawa T.PLoS One. 2012;7(5):e36809. Epub 2012 May 15.
- 2.
- Severe osteogenesis imperfecta caused by a small in-frame deletion in CRTAP.
Amor IM, Rauch F, Gruenwald K, Weis M, Eyre DR, Roughley P, Glorieux FH, Morello R.Am J Med Genet A. 2011 Nov;155(11):2865-70. doi: 10.1002/ajmg.a.34269. Epub 2011 Sep 30.
- 3.
- Mutations in PPIB (cyclophilin B) delay type I procollagen chain association and result in perinatal lethal to moderate osteogenesis imperfecta phenotypes.
Pyott SM, Schwarze U, Christiansen HE, Pepin MG, Leistritz DF, Dineen R, Harris C, Burton BK, Angle B, Kim K, Sussman MD, Weis M, Eyre DR, Russell DW, McCarthy KJ, Steiner RD, Byers PH.Hum Mol Genet. 2011 Feb 13. [Epub ahead of print]
- 4.
- Generalized connective tissue disease in Crtap-/- mouse.
Baldridge D, Lennington J, Weis M, Homan EP, Jiang MM, Munivez E, Keene DR, Hogue WR, Pyott S, Byers PH, Krakow D, Cohn DH, Eyre DR, Lee B, Morello R.PLoS One. 2010 May 11;5(5):e10560.
- 5.
- Prolyl 3-hydroxylase 1 and CRTAP are mutually stabilizing in the endoplasmic reticulum collagen prolyl 3-hydroxylation complex.
Chang W, Barnes AM, Cabral WA, Bodurtha JN, Marini JC.Hum Mol Genet. 2010 Jan 15;19(2):223-34. Epub 2009 Oct 21.
- 6.
- PPIB Mutations Cause Severe Osteogenesis Imperfecta.
van Dijk FS, Nesbitt IM, Zwikstra EH, Nikkels PG, Piersma SR, Fratantoni SA, Jimenez CR, Huizer M, Morsman AC, Cobben JM, van Roij MH, Elting MW, Verbeke JI, Wijnaendts LC, Shaw NJ, Hogler W, McKeown C, Sistermans EA, Dalton A, Meijers-Heijboer H, Pals G.Am J Hum Genet. 2009 Oct;85(4):521-7. Epub 2009 Sep 24.
- 7.
- Recessive Osteogenesis Imperfecta caused by LEPRE1 mutations: clinical documentation and identification of the splice form responsible for prolyl 3-hydroxylation.
Willaert A, Malfait F, Symoens S, Gevaert K, Kayserili H, Megarbane A, Mortier G, Leroy JG, Coucke PJ, De Paepe A.J Med Genet. 2009 Apr;46(4):233-41. Epub 2008 Dec 16.
- Applications
- Western Blot (Tissue lysate)
- LEPRE1 MaxPab polyclonal antibody. Western Blot analysis of LEPRE1 expression in human placenta.
- Protocol Download
- Western Blot (Transfected lysate)
- Western Blot analysis of LEPRE1 expression in transfected 293T cell line (H00064175-T01) by LEPRE1 MaxPab polyclonal antibody.
Lane 1: LEPRE1 transfected lysate(42.9 KDa).
Lane 2: Non-transfected lysate. - Protocol Download
- Entrez GeneID:
- 64175
- GeneBank Accession#:
- BC015309.1
- Protein Accession#:
- AAH15309
- Gene Name:
- LEPRE1
- Gene Alias:
- GROS1,MGC117314,P3H1
- Gene Description:
- leucine proline-enriched proteoglycan (leprecan) 1
- Gene Ontology:
- Hyperlink
- Gene Summary:
- 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. [provided by RefSeq
- Other Designations:
- OTTHUMP00000008736,growth suppressor 1,leprecan,leprecan 1,prolyl 3-hydroxylase 1