Product Name	ANTXR2
Chinese Name	炭疽毒素受体2抗体
Alias	CMG2; ISH; JHF; anthrax toxin receptor 2; ANTR2_HUMAN; Antxr2; Capillary morphogenesis gene 2 protein; CMG 2; CMG-2.
Research Area	Neurobiology  Signal transduction  The cell membrane受体  Cell adhesion molecule  Cytoskeleton  Extracellular matrix
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	50kDa
Cellular localization	cytoplasmic The cell membrane Extracellular matrix Secretory protein
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human ANTXR2: 101-200/489 <Extracellular>
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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

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