Western Blot (WB)
(Detection of Mouse CMG‑2/ANTXR2 by Western Blot.)
Goat anti-Mouse Capillary Morphogenesis Gene 2 Polyclonal Antibody | anti-ANTXR2 antibody
Capillary Morphogenesis Gene 2 (CMG-2, Anthrax Toxin Receptor 2, ANTXR2)
Purified by mouse CMG-2 affinity chromatography.
Purified by mouse CMG-2 affinity chromatography.
Dilution: Western Blot: 0.1-0.2ug/ml. The detection limit for rmCMG-2 is approximately 5ng/lane under non-reducing and reducing conditions.
ELISA: 0.5-1ug/ml. The detection limit for rmCMG-2 is approximately 1.5ng/well.
Western Blot (WB)
(Detection of Mouse CMG‑2/ANTXR2 by Western Blot.)
Western Blot (WB)
(Detection of Mouse CMG‑2/ANTXR2 by Western Blot.)
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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: Receptor, misc.; Membrane protein, integral
Chromosomal Location of Human Ortholog: 4q21.21
Cellular Component: endoplasmic reticulum membrane; extracellular region; plasma membrane; integral to membrane; endosome membrane; external side of plasma membrane
Molecular Function: protein binding; metal ion binding; receptor activity
Biological Process: reproductive process; pathogenesis
Disease: Hyaline Fibromatosis Syndrome
Research Articles on ANTXR2
2. Cytoplasmic delivery of lethal factor by anthrax toxin receptor 2 was mediated by cathepsin B.
3. Observational study and genome-wide association study of gene-disease association. (HuGE Navigator)
4. Results suggest that, in vivo, slow folding, rather than misfolding, is responsible for ER retention, and that systemic hyalinosis can be qualified as a conformational disease.
5. Observational study of gene-disease association. (HuGE Navigator)
6. structures provide a template to begin probing the high-affinity capillary morphogenesis protein 2-protective antigen interaction and may facilitate understanding of toxin assembly/internalization
7. Data show that cells expressing palmitoylation-defective mutant receptors are less sensitive to anthrax toxin due to a lower number of surface receptors as well as premature internalization of protective antigen without a requirement for heptamerization.
8. identification and characterization of juvenile hyaline fibromatosis and infantile systemic hyalinosis disease-causing mutations in the capillary morphogenesis factor-2 gene
9. Molecular dynamics simulations explain the great strength that the protective antigen-capillary morphogenesis gene 2 complex achieves through extraordinary coordination of a divalent cation.
10. identification of 15 different mutations in the gene encoding capillary morphogenesis protein 2 in 17 families with juvenile hyaline fibromatosis or infantile systemic hyalinosis
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Product Notes
The ANTXR2 antxr2 (Catalog #AAA610618) is an Antibody produced from Goat and is intended for research purposes only. The product is available for immediate purchase. The Capillary Morphogenesis Gene 2 (CMG-2, Anthrax Toxin Receptor 2, ANTXR2) reacts with Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's Capillary Morphogenesis Gene 2 can be used in a range of immunoassay formats including, but not limited to, ELISA (EL/EIA), Western Blot (WB). Suitable for use in ELISA and Western Blot., Dilution: Western Blot: 0.1-0.2ug/ml. The detection limit for rmCMG-2 is approximately 5ng/lane under non-reducing and reducing conditions. ELISA: 0.5-1ug/ml. The detection limit for rmCMG-2 is approximately 1.5ng/well. Researchers should empirically determine the suitability of the ANTXR2 antxr2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Capillary Morphogenesis Gene 2, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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