炭疽**受体2抗体
规格：1尘驳/1尘濒
英文名： ANTXR2
别名： CMG2; ISH; JHF; anthrax toxin receptor 2; ANTR2_HUMAN; Antxr2; Capillary morphogenesis gene 2 protein; CMG 2; CMG-2.
分子量： 50kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ANTXR2
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
细胞定位：细胞浆 细胞膜 细胞外基质 分泌型蛋白
炭疽**受体2抗体产物介绍：background: This gene encodes a receptor for anthrax toxin. The protein binds to collagen IV and laminin, suggesting that it may be involved in extracellular matrix adhesion. Mutations in this gene cause juvenile hyaline fibromatosis and infantile systemic hyalinosis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009]. Function: Necessary for cellular interactions with laminin and the extracellular matrix. Subunit: Binds laminin, and possibly also collagen type IV. Binds to the protective antigen (PA) of Bacillus anthracis in a divalent cation-dependent manner, with the following preference: calcium > manganese > 炭疽**受体2抗体magnesium > zinc. Binding of PA leads to heptamerization of the receptor-PA complex. Subcellular Location: Secreted; Cell membrane. Expressed at the cell surface and Endoplasmic reticulum membrane. Expressed predominantly within the endoplasmic reticulum and not at the plasma membrane. Tissue Specificity: Expressed in prostate, thymus, ovary, testis, pancreas, colon, heart, kidney, lung, liver, peripheral blood leukocytes, placenta, skeletal muscle, small intestine and spleen. DISEASE: Defects in ANTXR2 are the cause of infantile systemic hyalinosis (ISH). This autosomal recessive syndrome is similar to JHF, but has an earlier onset and a more severe course. Symptoms appear at birth or within the炭疽**受体2抗体 first months of life, with painful, swollen joint contractures, osteopenia, osteoporosis and livid red hyperpigmentation over bony prominences. Patients develop multiple subcutaneous skin tumors and gingival hypertrophy. Hyaline deposits in multiple organs, recurrent infections and intractable diarrhea often lead to death within the first 2 years of life. Surviving children may suffer from severely reduced mobility due to joint contractures. Similarity: Belongs to the ATR family. Contains 1 VWFA domain. Database links: UniProtKB/Swiss-Prot: P58335.5 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
炭疽**受体2抗体产物应用：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：神经生物学 信号转导 细胞膜受体 细胞粘附分子 细胞骨架 细胞外基质
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid