Anthrax Toxin Receptor 2 (ANTXR2), Polyclonal Antibody

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

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

Polyclonal

Rabbit

200ug/ml (lot specific)

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 customer’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.

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

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)

53,184 Da

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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