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ANTXR2 Rabbit pAb (bs-11071R)  
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產品編號 bs-11071R
英文名稱 ANTXR2 Rabbit pAb
中文名稱 炭疽毒素受體2抗體
別    名 CMG2; ISH; JHF; anthrax toxin receptor 2; ANTR2_HUMAN; Antxr2; Capillary morphogenesis gene 2 protein; CMG 2; CMG-2.  
研究領域 神經生物學  信號轉導  細胞膜受體  細胞粘附分子  細胞骨架  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse (predicted: Human,Rat,Rabbit,Pig,Cow,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 50 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 細胞外基質 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ANTXR2: 101-200/489 <Extracellular>
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
產品介紹 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



產品圖片
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 size: 50 kDa Observed band size: 52 kDa
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