CMG-2; CMG2; ISH; JHF; Capillary Morphogenesis Protein 2

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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against ANTXR2. It has been selected for its ability to recognize ANTXR2 in immunohistochemical staining and western blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4 containing 0.02% NaN3, 50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-ANTXR2 antibody 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.

Knock-Out Validation Antibody

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western Blot: 1:50-400
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200

Western Blot: Sample: Lane1: Human PC-3 Cells; Lane2: Porcine Intestine Tissue; Lane3: Rat Prostate Gland Tissue.

Western Blot: Sample: Recombinant ANTXR2, Human.

DAB staining on IHC-P; Samples: Human Stomach Tissue.

DAB staining on IHC-P; Samples: Human Liver Tissue

DAB staining on IHC-P; Samples: Human Esophagus Cancer Tissue

Knockout Validation: Lane 1: Wild-type PC3 cell lysate; Lane 2: ANTXR2 knockout PC3 cell lysate; Predicted MW: 54,53,43,35kd Observed MW: 58kd Primary Ab: 1ug/ml Rabbit Anti-Human ANTXR2 Antibody Second Ab: 0.2ug/mL?HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047)

53,493 Da

anthrax toxin receptor 2

anthrax toxin receptor 2

Anthrax toxin receptor 2

CMG2; CMG-2??[Similar Products]

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]

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: 4q21.21

Cellular Component: endoplasmic reticulum membrane; endosome membrane; external side of plasma membrane; extracellular region; integral component of membrane; plasma membrane

Molecular Function: metal ion binding; protein binding; receptor activity

Biological Process: reproductive process

Disease: Hyaline Fibromatosis Syndrome

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.