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骨形态发生抑制蛋白SOST抗体

骨形态发生抑制蛋白SOST抗体
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  • 产品名称:骨形态发生抑制蛋白SOST抗体
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简单介绍
骨形态发��抑制蛋白SOST抗体应用于IHC、WB、 IF、IP、ELISA等科研实验,按理化性质和生物学功能IgM、IgG、IgA、IgE、IgD五类。按抗体的来源,可将其分为天然抗体和**抗体。骨形态发生抑制蛋白SOST抗体生产每个流程都执行严格的检测标准,保证蛋白抗原产品质量,质量稳定,实验效果明显。
产品描述

骨形态发生抑制蛋白SOST抗体

规格:1mg/1ml

英文名: Sclerostin

别名: BEER; Cortical hyperostosis with syndactyly; Sclerosteosis; Sclerostin; SOST; SOST_HUMAN; VBCH.

分子量: 21kDa

储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型:Polyclonal

亚型:IgG

纯化方法:affinity purified by Protein A

**原:KLH conjugated synthetic peptide derived from human Sclerost

交叉反应:Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,

细胞定位:细胞浆

骨形态发生抑制蛋白SOST抗体产品介绍:background: Negative regulator of bone growth.Sclerostin (SOST) is a bone morphogenetic protein (BMP) antagonist, leading to the activation of BMP signaling. It negatively regulates the formation of bone by repressing the differentiation and/or function of osteoblasts induced by BMPs. It has been shown that Sclerostin binds BMP-5, -6, and -7 with high affinity and BMP-2 and -4 with low affinity. The noggin-sclerostin protein complex represents a novel mechanism for the fine-tuning of BMP activity in bone homeostasis. Evidence is accumulating that one of the important mechanisms of bone regulation by sclerostin is the modulation of Wnt/Beta-catenin signaling. Sclerostin also rapidly activated ERK-1/2 MAPK signaling, indicating the involvement of additional signaling pathways Function: Negative regulator of bone growth that acts through inhibition of Wnt signaling and bone formation. Subunit: Interacts with LRP4 (via the extracellular domain); the interaction facilitates the inhibition of Wnt signaling. Interacts with LRP5 (via the first two YWTD-EGF repeat domains); the interaction inhibits Wnt-mediated 骨形态发生抑制蛋白SOST抗体signaling. Interacts with LRP6. Subcellular Location: Secreted. Tissue Specificity: Widely expressed at low levels with highest levels in bone, cartilage, kidney, liver, bone marrow and primary osteeoblasts differentiated for 21 days. DISEASE: Defects in SOST are the cause of sclerosteosis (SOST) [MIM:269500]; also known as cortical hyperostosis with syndactyly. SOST is an autosomal recessive sclerosing bone dysplasia characterized by a generalized hyperostosis and sclerosis leading to a markedly thickened skull, with mandible, ribs, clavicles and all long bones also being affected. Due to narrowing of the foramina of the cranial nerves, facial nerve palsy, hearing loss and atrophy of the optic nerves can occur. Sclerosteosis is clinically and radiologically very similar to van Buchem disease, mainly differentiated by hand malformations and a large stature in sclerosteosis patients. Note=A 52 kb deletion downstream of SOST results in SOST transcription suppression and is a cause of van Buchem disease (VBCH) [MIM:239100]; also known as hyperostosis corticalis generalisata. VBCH is an autosomal recessive sclerosing bone dysplasia characterized by endosteal hyperostosis of the mandible, skull, ribs, clavicles, and diaphyses of the long bones. Affected patients present a symmetrically increased thickness of bones, most骨形态发生抑制蛋白SOST抗体 frequently found as an enlarged jawbone, but also an enlargement of the skull, ribs, diaphysis of long bones, as well as tubular bones of hands and feet. The clinical consequence of increased thickness of the skull include facial nerve palsy causing hearing loss, visual problems, neurological pain, and, very rarely, blindness as a consequence of optic atrophy. Serum alkaline phosphatase levels are elevated. Similarity: Belongs to the sclerostin family. Contains 1 CTCK (C-terminal cystine knot-like) domain. Gene ID: 50964 Database links: Entrez Gene: 50964 Human Entrez Gene: 74499 Mouse Entrez Gene: 80722 Rat Omim: 605740 Human SwissProt: Q9BQB4 Human SwissProt: Q99P68 Mouse SwissProt: Q99P67 Rat Unigene: 349204 Human Unigene: 265602 Mouse Unigene: 95369 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

骨形态发生抑制蛋白SOST抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.

研究领域:细胞生物  信号转导  生长因子和**  

储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源: Rabbit

外观: Lyophilized or Liquid


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