Product nameAnti-ANTXR2/CMG-2 antibody [EPR7717]
See all ANTXR2/CMG-2 primary antibodies
DescriptionRabbit monoclonal [EPR7717] to ANTXR2/CMG-2
Tested applicationsSuitable for: WB, IHC-P, ICC/IFmore details
Unsuitable for: Flow Cyt or IP
Species reactivityReacts with: Mouse, Rat, Human
Synthetic peptide within Human ANTXR2/CMG-2 aa 400-500. The exact sequence is proprietary.
- Human fetal heart, ECV304 and PC3 lysates; Human colon tissue; PC3 cells.
Storage instructionsShipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Storage bufferpH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol, 0.05% BSA, 50% Tissue culture supernatant
PurityTissue culture supernatant
Our Abpromise guarantee covers the use of ab129004 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000 - 1/10000. Detects a band of approximately 55 kDa (predicted molecular weight: 54 kDa).|
|IHC-P||1/250 - 1/500. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
Please note: ab129004 seems to be not suitable for the use in IHC-P with mouse samples.
|ICC/IF||1/250 - 1/500.|
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.
- Information by UniProt
- 2310046B19Rik antibody
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All lanes : Anti-ANTXR2/CMG-2 antibody [EPR7717] (ab129004) at 1/1000 dilution
Lane 1 : Human fetal heart lysate
Lane 2 : ECV304 cell lysate
Lane 3 : PC3 cell lysate
Lysates/proteins at 10 µg per lane.
All lanes : Goat anti-Rabbit HRP at 1/2000 dilution
Predicted band size: 54 kDa
ab129004, at 1/250 dilution, staining ANTXR2/CMG-2 in paraffin-embedded Human colon tissue by Immunohistochemistry.
ab129004, at 1/250 dilution staining ANTXR2/CMG-2 in PC3 cells by Immunofluorescence.
ab129004 has not yet been referenced specifically in any publications.