產(chǎn)品編號 | bs-18222R |
英文名稱 | LEPRE1 Rabbit pAb |
中文名稱 | 生長抑制因子1抗體 |
別 名 | GROS 1; GROS1; Growth suppressor 1; LEPRE 1; Lepre1; Leprecan 1; Leprecan; Leprecan-1; Leprecan1; Leucine proline enriched proteoglycan(leprecan) 1; Leucine proline enriched proteoglycan 1; Leucine-and proline-enriched proteoglycan 1; MGC117314; OI8; P3H1; P3H1_HUMAN; Prolyl 3 hydroxylase 1; Prolyl 3-hydroxylase 1. |
研究領(lǐng)域 | 細(xì)胞生物 信號轉(zhuǎn)導(dǎo) |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
克 隆 號 | |
交叉反應(yīng) | (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse) |
產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
理論分子量 | 81 kDa |
檢測分子量 | |
細(xì)胞定位 | 分泌型蛋白 |
性 狀 | Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human LEPRE1: 351-450/736 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
PubMed | PubMed |
產(chǎn)品介紹 |
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. Three 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, Aug 2011] Function: Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts. Subcellular Location: Endoplasmic reticulum. Secreted > extracellular space > extracellular matrix. Secreted into the extracellular matrix as a chondroitin sulfate proteoglycan. Post-translational modifications: O-glycosylated; chondroitin sulfate. DISEASE: Defects in LEPRE1 are the cause of osteogenesis imperfecta type 8 (OI8) [MIM:610915]. A connective tissue disorder characterized by disproportionate short stature, severe osteoporosis, shortening of the long bones, white sclerae, a round face and a short barrel-shaped chest. Similarity: Belongs to the leprecan family. Contains 1 Fe2OG dioxygenase domain. Contains 4 TPR repeats. SWISS: Q32P28 Gene ID: 64175 Database links: Entrez Gene: 64175 Human Entrez Gene: 56401 Mouse Omim: 610339 Human SwissProt: Q32P28 Human SwissProt: Q3V1T4 Mouse Unigene: 720014 Human Unigene: 27961 Mouse Unigene: 13741 Rat |