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ANTXR2 Rabbit pAb (bs-11071R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-11071R
英文名称 ANTXR2 Rabbit pAb
中文名称 炭疽毒素受体2抗体
别    名 CMG2; ISH; JHF; anthrax toxin receptor 2; ANTR2_HUMAN; Antxr2; Capillary morphogenesis gene 2 protein; CMG 2; CMG-2.  
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  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse (predicted: Human,Rat,Rabbit,Pig,Cow,Dog,Horse)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 50 kDa
检测分子量
细胞定位 细胞浆 细胞膜 细胞外基质 分泌型蛋白 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ANTXR2: 101-200/489 <Extracellular>
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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 > 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 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.

SWISS:
P58335

Gene ID:
118429

Database links:

Entrez Gene: 118429 Human

Entrez Gene: 71914 Mouse

Entrez Gene: 305633 Rat

Omim: 608041 Human

SwissProt: P58335 Human

SwissProt: Q6DFX2 Mouse

Unigene: 162963 Human

Unigene: 24842 Mouse

Unigene: 229072 Rat



产品图片
Sample: Lane 1: Mouse Lung tissue lysates Lane 2: Mouse Ovary tissue lysates Lane 3: Mouse Large intestine tissue lysates Primary: Anti-ANTXR2 (bs-11071R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band siz
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