2310046B19Rik; AW561899; cI-35; CMG-2; CMG2; Anthrax toxin receptor 2

For Research Use Only. Not for use in diagnostic procedures.

Baculovirus
Source: Mouse

> 95%

Liquid
In Phosphate Buffered Saline (pH 7.4) containing 10% glycerol.

Store at -20 degree C for 12 months.
Centrifuge the vial prior to opening.

Small volumes of ANTXR2 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ANTXR2; also known as anthrax toxin receptor 2; is a widely expressed anthrax toxin receptor family protein. The main functional domain of this protein is an extracellular integrinlike von Willebrand factor type A (VWA) domain with a metal ion dependent adhesion site; through which it adheres selectively to collagen type IV and laminin. This protein and the related protein ATR/TEM8 serve as receptors for the protective antigen of Bacillus anthracis. Recombinant mouse ANTXR2; fused to His-tag at C-terminus; was expressed in insect cell and purified by using conventional chromatography techniques.

SDS-PAGE

31.9 kDa

anthrax toxin receptor 2

anthrax toxin receptor 2

Anthrax toxin receptor 2

ANTXR2: Necessary for cellular interactions with laminin and the extracellular matrix. 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. Defects in ANTXR2 are the cause of juvenile hyaline fibromatosis (JHF). JHF is an autosomal recessive syndrome that is similar to ISH but takes a milder course. It is characterized by hyaline deposition in the dermis, multiple subcutaneous skin tumors and gingival hypertrophy, followed by progressive joint contractions, osteopenia and osteoporosis that may lead to a severe limitation of mobility. Belongs to the ATR family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Receptor, misc.

Chromosomal Location of Human Ortholog: 5|5 E3

Cellular Component: cell surface; external side of plasma membrane; integral to membrane

Biological Process: reproductive process

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