產(chǎn)品編號 yb-11071R
英文名稱 Anti-ANTXR2抗體
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.
Anti-ANTXR2抗體
說 明 書 0.1ml  0.2ml  
研究領(lǐng)域 神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  細(xì)胞膜受體  細(xì)胞粘附分子  細(xì)胞骨架  細(xì)胞外基質(zhì)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, 
產(chǎn)品應(yīng)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 （石蠟切片需做抗原修復(fù)） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 50kDa
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 細(xì)胞外基質(zhì) 分泌型蛋白 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ANTXR2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
產(chǎn)品介紹 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 > 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. 


Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.