产品展示
Anti-ANTXR2炭疽毒素受体2抗体
点击次数：200发布时间：2012/12/26 4:48:01
更新日期：2024/9/5 14:43:27
所 在 地：其它
产品型号：BY-11071R
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详细内容
货号：BY-11071R
英文名称：Anti-ANTXR2
中文名称：炭疽毒素受体2抗体
其他名称：CMG2;ISH;JHF;anthrax toxin receptor 2;ANTR2_HUMAN;Antxr2;Capillary morphogenesis gene 2 protein;CMG 2;CMG-2.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 54kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, cow, dog
产品介绍：Function : Necessary for cellular interactions with laminin ａnd the extracellular matrix.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 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.
