Product nameAnti-PAX3 antibody
See all PAX3 primary antibodies
DescriptionGoat polyclonal to PAX3
Tested applicationsSuitable for: WB, IHC-P, IPmore details
Species reactivityReacts with: Mouse, Human
Predicted to work with: Rat, Chicken, Dog
- Recombinant Human PAX3 protein (ab114320) can be used as a positive control in WB. Human squamous epithelium. Human duodenum lysate.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: Tris buffered saline, 0.5% BSA
Concentration information loading...
PurityImmunogen affinity purified
Purification notesPurified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Immunizing Peptide (Blocking)
Our Abpromise guarantee covers the use of ab15717 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.3 - 1 µg/ml. Predicted molecular weight: 53 kDa.Can be blocked with Human PAX3 peptide (ab23270).|
|IHC-P||Use a concentration of 3 µg/ml.|
|IP||Use a concentration of 2.5 µg/ml.|
FunctionProbable transcription factor associated with development of alveolar rhabdomyosarcoma.
Involvement in diseaseDefects in PAX3 are the cause of Waardenburg syndrome type 1 (WS1) [MIM:193500]. WS1 is an autosomal dominant disorder characterized by wide bridge of nose owing to lateral displacement of the inner canthus of each eye (dystopia canthorum), pigmentary disturbances such as frontal white blaze of hair, heterochromia of irides, white eyelashes, leukoderma and sensorineural deafness. The syndrome shows variable clinical expression and some affected individuals do not manifest hearing impairment.
Defects in PAX3 are the cause of Waardenburg syndrome type 3 (WS3) [MIM:148820]; also known as Klein-Waardenburg syndrome or Waardenburg syndrome with upper limb anomalies or white forelock with malformations. WS3 is a very rare autosomal dominant disorder, which shares many of the characteristics of WS1. Patients additionally present with musculoskeletal abnormalities.
Defects in PAX3 are the cause of craniofacial-deafness-hand syndrome (CDHS) [MIM:122880]. CDHS is thought to be an autosomal dominant disease which comprises absence or hypoplasia of the nasal bones, hypoplastic maxilla, small and short nose with thin nares, limited movement of the wrist, short palpebral fissures, ulnar deviation of the fingers, hypertelorism and profound sensory-neural deafness.
Defects in PAX3 are a cause of rhabdomyosarcoma type 2 (RMS2) [MIM:268220]. It is a form of rhabdomyosarcoma, a highly malignant tumor of striated muscle derived from primitive mesenchimal cells and exhibiting differentiation along rhabdomyoblastic lines. Rhabdomyosarcoma is one of the most frequently occurring soft tissue sarcomas and the most common in children. It occurs in four forms: alveolar, pleomorphic, embryonal and botryoidal rhabdomyosarcomas. Note=A chromosomal aberration involving PAX3 is found in rhabdomyosarcoma. Translocation (2;13)(q35;q14) with FOXO1. The resulting protein is a transcriptional activator.
Note=A chromosomal aberration involving PAX3 is a cause of rhabdomyosarcoma. Translocation t(2;2)(q35;p23) with NCOA1 generates the NCOA1-PAX3 oncogene consisting of the N-terminus part of PAX3 and the C-terminus part of NCOA1. The fusion protein acts as a transcriptional activator. Rhabdomyosarcoma is the most common soft tissue carcinoma in childhood, representing 5-8% of all malignancies in children.
Sequence similaritiesBelongs to the paired homeobox family.
Contains 1 homeobox DNA-binding domain.
Contains 1 paired domain.
- Information by UniProt
- CDHS antibody
- HUP 2 antibody
- HUP2 antibody
ab15717 staining PAX3 in murine striated muscle tissue by Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections).
Tissue was fixed in formaldehyde and a heat mediated antigen retrieval step was performed using citrated buffer pH 6. Samples were then blocked with 1% BSA for 30 minutes at 25°C and then incubated with ab15717 at a 1/100 dilution for 18 hours at 25°C. The secondary used was an undiluted HRP conjugated goat polyclonal.
ab15717 (3µg/ml) staining of paraffin embedded Human Oesophagus. The tissue sections subjected to antigen retrieval by microwave in Tris/EDTA buffer pH 9.0. The HRP-staining procedure was used for detection.
Anti-PAX3 antibody (ab15717) at 0.3 µg/ml + Human duodenum lysate in RIPA buffer at 35 µg
Developed using the ECL technique.
Predicted band size: 53 kDa
Observed band size: 45 kDa why is the actual band size different from the predicted?
This product has been referenced in:
- Sun L et al. Mir193b-365 is essential for brown fat differentiation. Nat Cell Biol 13:958-65 (2011). WB . Read more (PubMed: 21743466) »
- Dissanayake SK et al. Wnt5A regulates expression of tumor-associated antigens in melanoma via changes in signal transducers and activators of transcription 3 phosphorylation. Cancer Res 68:10205-14 (2008). WB ; Human . Read more (PubMed: 19074888) »