Anti-ANTXR2 antibody (ab113523)
- Product nameAnti-ANTXR2 antibodySee all ANTXR2 primary antibodies ...
- DescriptionGoat polyclonal to ANTXR2
- Tested applicationsWB, IHC-P more details
- Species reactivityReacts with: Mouse, Rat, Human
Predicted to work with: Cow, Dog
Synthetic peptide conjugated to KLH, from an internal sequence of Human ANTXR2 (NP_477520.2; NP_001139266.1).
- Positive control
- Human Kidney and Liver tissues; Human, Mouse and Rat Kidney lysates.
- Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
- Storage bufferpH: 7,30
Preservative: 0.02% Sodium azide
Constituents: 98% Tris buffered saline, 0.5% BSA
- Concentration information loading...
- PurityProtein G purified
- Clonality Polyclonal
- Research Areas
Our Abpromise guarantee covers the use of ab113523 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||WB: Use a concentration of 0.3 - 1 µg/ml. Detects a band of approximately 60 kDa (predicted molecular weight: 54 kDa).|
|IHC-P||IHC-P: Use a concentration of 2.5 µg/ml.|
- FunctionNecessary for cellular interactions with laminin and the extracellular matrix.
- Tissue specificityExpressed in prostate, thymus, ovary, testis, pancreas, colon, heart, kidney, lung, liver, peripheral blood leukocytes, placenta, skeletal muscle, small intestine and spleen.
- Involvement in diseaseDefects in ANTXR2 are the cause of infantile systemic hyalinosis (ISH) [MIM:236490]. 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) [MIM:228600]. 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.
- Sequence similaritiesBelongs to the ATR family.
Contains 1 VWFA domain.
- DomainBinding to PA seems to be effected through the VWA domain.
- Cellular localizationSecreted; Cell membrane. Expressed at the cell surface and Endoplasmic reticulum membrane. Expressed predominantly within the endoplasmic reticulum and not at the plasma membrane.
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Anti-ANTXR2 antibody images
Anti-ANTXR2 antibody (ab113523) at 0.3 µg/ml + Mouse Kidney lysate (in RIPA buffer) at 35 µg
developed using the ECL technique
Predicted band size : 54 kDa
ab113523, at 2.5µg/ml, staining ANTXR2 in Formalin-fixed, Paraffin-embedded Human kidney tissue by Immunohistochemistry.
ab113523, at 2.5µg/ml, staining ANTXR2 in Formalin-fixed, Paraffin-embedded Human liver tissue by Immunohistochemistry.
References for Anti-ANTXR2 antibody (ab113523)
ab113523 has not yet been referenced specifically in any publications.