Overview

  • Product name
  • Description
    Rabbit polyclonal to PAX3
  • Host species
    Rabbit
  • Tested applications
    Suitable for: ICC/IF, WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
    Predicted to work with: Xenopus laevis
  • Immunogen

    Recombinant full length protein corresponding to Human PAX3 aa 1-206.
    Sequence:

    MTTLAGAVPRMMRPGPGQNYPRSGFPLEVSTPLGQGRVNQLGGVFINGRP LPNHIRHKIVEMAHHGIRPCVISRQLRVSHGCVSKILCRYQETGSIRPGA IGGSKPKQVTTPDVEKKIEEYKRENPGMFSWEIRDKLLKDAVCDRNTVPS VSSISRILRSKFGKGEEEEADLERKEAEESEKKAKHSIDGILSERGKALV SGVSSH


    Database link: P23760-3

  • Positive control
    • Extracts of A431 cell lines.

Properties

Applications

Our Abpromise guarantee covers the use of ab180754 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
ICC/IF Use at an assay dependent concentration.
WB 1/500 - 1/2000. Predicted molecular weight: 23 kDa.
IHC-P 1/50 - 1/200.

ab171870 - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody.

Target

  • Function
    Probable transcription factor associated with development of alveolar rhabdomyosarcoma.
  • Involvement in disease
    Defects 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 similarities
    Belongs to the paired homeobox family.
    Contains 1 homeobox DNA-binding domain.
    Contains 1 paired domain.
  • Cellular localization
    Nucleus.
  • Information by UniProt
  • Database links
  • Alternative names
    • CDHS antibody
    • HUP 2 antibody
    • HUP2 antibody
    • MGC120381 antibody
    • MGC120382 antibody
    • MGC120383 antibody
    • MGC120384 antibody
    • MGC134778 antibody
    • Paired box 3 antibody
    • Paired box gene 3 antibody
    • Paired box homeotic gene 3 antibody
    • Paired box protein Pax 3 antibody
    • Paired box protein Pax-3 antibody
    • Paired box protein Pax3 antibody
    • Paired domain gene 3 antibody
    • Paired domain gene HuP2 antibody
    • PAX 3 antibody
    • Pax3 antibody
    • PAX3/FKHR fusion gene antibody
    • PAX3_HUMAN antibody
    • Sp antibody
    • splotch antibody
    • Waardenburg syndrome 1 antibody
    • WS 1 antibody
    • WS1 antibody
    • WS3 antibody
    see all

Images

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of rat lung tissue labelling PAX3 with ab180754 at 1/200. Magnification: 400x.
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of rat liver tissue labelling PAX3 with ab180754 at 1/200. Magnification: 400x.
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human lung cancer tissue labelling PAX3 with ab180754 at 1/200. Magnification: 400x.
  • Anti-PAX3 antibody (ab180754) at 1/500 dilution + A431 cell lysate

    Predicted band size: 23 kDa

References

This product has been referenced in:
  • Kim CH  et al. Schisandrae fructus enhances myogenic differentiation and inhibits atrophy through protein synthesis in human myotubes. Int J Nanomedicine 11:2407-15 (2016). Read more (PubMed: 27330287) »

See 1 Publication for this product

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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