ANTXR2 Rabbit pAb
ANTXR2 Rabbit pAb
- 产品详情
- 实验流程
- 背景知识
Application
| WB |
|---|---|
| Primary Accession | P58335 |
| Reactivity | Mouse |
| Predicted | Rat, Pig, Dog, Human, Rabbit, Horse |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 53666 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human ANTXR2 |
| Epitope Specificity | 101-200/489 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 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. |
| 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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]. |
| Gene ID | 118429 |
|---|---|
| Other Names | Anthrax toxin receptor 2, Capillary morphogenesis gene 2 protein, CMG-2, ANTXR2 (HGNC:21732) |
| Dilution | WB=1:500-2000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | ANTXR2 (HGNC:21732) |
|---|---|
| Function | Necessary for cellular interactions with laminin and the extracellular matrix. |
| Cellular Location | [Isoform 1]: Cell membrane; Single-pass type I membrane protein. Note=Expressed at the cell surface [Isoform 3]: Secreted. |
| Tissue Location | Expressed in prostate, thymus, ovary, testis, pancreas, colon, heart, kidney, lung, liver, peripheral blood leukocytes, placenta, skeletal muscle, small intestine and spleen |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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