PAX3 antibody, paired box gene 3 (Waardenburg syndrome 1) (PAX3) Antibody, Mouse Monoclonal Antibody

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paired box gene 3 (Waardenburg syndrome 1) (PAX3) Antibody, Mouse Monoclonal Antibody

Catalog Number: 20-372-60266

Buy PAX3 antibody -

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Related Product Names:

- PAX3 antibody; PAX3; WS1; CDHS; HUP2; MGC120381; MGC120382; MGC120383; MGC120384; MGC134778; paired box gene 3 (Waardenburg syndrome 1) (PAX3)
- Mouse monoclonal anti-human PAX3 antibody; HUP2; Homo sapiens paired box 3 (PAX3), transcript variant PAX3A, mRNA; Paired box protein Pax-3

- Gene Information - Information in yellow represents specific gene information and does not necessarily represent specific product details. For more information please contact sales@genwaybio.com.

Gene Name: PAX3	Gene Name Synonym: WS1; CDHS; HUP2; MGC120381; MGC120382; MGC120383; MGC120384; MGC134778
Gi #: 31563350	NCBI Acc #: NM_000438.3	Swiss Prot Acc #: P23760
Length (aa): N/A	Mol. Weight (Da): 52968	Chrom Location: N/A

Clone: 6288D4a

IGG Subclass: IgG1

Preparation: This antibody was purified using protein G column chromatography from culture supernatant of hybridoma cultured in a medium containing bovine IgG-depleted (approximately 95%) fetal bovine serum.

Sterility: Filtered through a 0.22 um membrane.

Source/Host: Mouse

Clonality: Monoclonal

Crossreactivity: Human

Format: Mouse monoclonal anti-human PAX3 antibody in PBS (3.0 mM KCl, 1.5 mM K2HPO4, 140 mM NaCl, 8.0 mM Na2HPO4 (pH 7.4)) containing 1% bovine serum albumin (BSA) and 0.05% sodium azide (NaN3)

Storage: Store at 2-8 C for up to one year. We recommend storing at –20 C for long-term storage. Avoid repeat freezing and thawing cycles.

Shipping: Products may be shipped on ice pack or dry ice.

APPLICATIONS for PAX3 ANTIBODY:

DB: Tested

TESTING: (secondary reagents and protocols )

Western Blot ( anti-PAX3 )

PAX3 antibody tern B image
PAX3

PAX3 ANTIBODY TARGET DESCRIPTION:

Synonym Names for PAX3 antibody: PAX3; WS1; CDHS; HUP2; MGC120381; MGC120382; MGC120383; MGC120384; MGC134778; Mouse monoclonal anti-human PAX3 antibody; HUP2; Homo sapiens paired box 3 (PAX3), transcript variant PAX3A, mRNA; Paired box protein Pax-3

Function: Probable transcription factor associated with development of alveolar rhabdomyosarcoma.

Subunit: Can bind to DNA as a heterodimer with PAX7. Interacts with DAXX.

Subcellular Location: Nucleus.

Disease: Defects in PAX3 are the cause of Waardenburg syndrome type I (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.

Disease: Defects in PAX3 are the cause of Waardenburg syndrome type III (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.

Disease: 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.

Disease: A chromosomal aberration involving PAX3 is a cause of rhabdomyosarcoma 2 (RMS2) [MIM:268220]; also known as alveolar rhabdomyosarcoma. Translocation (2;13)(q35;q14) with FOXO1A. The resulting protein is a transcriptional activator.

Disease: 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.

Similarity: Belongs to the paired homeobox family.

Similarity: Contains 1 homeobox DNA-binding domain.

Similarity: Contains 1 paired domain.

Summary: This protein is a member of the paired box (PAX) family of transcription factors. Members of the PAX family typically contain a paired box domain and a paired-type homeodomain. These genes play critical roles during fetal development. Mutations in paired box gene 3 are associated with Waardenburg syndrome, craniofacial-deafness-hand syndrome, and alveolar rhabdomyosarcoma. The translocation t(2;13)(q35;q14), which represents a fusion between PAX3 and the forkhead gene, is a frequent finding in alveolar rhabdomyosarcoma. Alternative splicing results in transcripts encoding isoforms with different C-termini.

paired box gene 3 (Waardenburg syndrome 1) (PAX3) reacts with human.

OMIM: 122880; phenotype. [NCBI / EBI]
148820; phenotype. [NCBI / EBI]
193500; phenotype. [NCBI / EBI]
268220; phenotype. [NCBI / EBI]
606597; gene. [NCBI / EBI]

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SUGGESTED ANITBODY REAGENTS - GenWay has an extensive selection of reagents useful for antibody applications. Find blocking buffers, stop reagents, chemiluminescent substrates, diluents, Immunohistochemistry reagents & more.

BACKGROUND REFERENCES for PAX3 ANTIBODY:

Background references for antibody target are not specific to GenWay products

[1] Scuoppo,C., Riess,I., Schmitt-Ney,M., Allegra,P., Forni,P.E., Bersani,F., Taulli,R., Accornero,P., Crepaldi,T. and Ponzetto,C.
     The oncogenic transcription factor PAX3-FKHR can convert fibroblasts into contractile myotubes
[2] Wang,Q., Kumar,S., Mitsios,N., Slevin,M. and Kumar,P., et al.
     Investigation of downstream target genes of PAX3c, PAX3e and PAX3g isoforms in melanocytes by microarray analysis
[3] Kazanowska,B., Reich,A., Stegmaier,S., Bekassy,A.N., Leuschner,I., Chybicka,A. and Koscielniak,E.
     Pax3-fkhr and pax7-fkhr fusion genes impact outcome of alveolar rhabdomyosarcoma in children
[4] Lu,W., Zhu,H., Wen,S., Laurent,C., Shaw,G.M., Lammer,E.J. and Finnell,R.H.
     Screening for novel PAX3 polymorphisms and risks of spina bifida
[5] Qin,W., Shu,A., Qian,X., Gao,J., Xing,Q., Zhang,J., Zheng,Y., Li,X., Li,S., Feng,G. and He,L.
     A novel mutation of PAX3 in a Chinese family with Waardenburg syndrome
[6] Macina R.A., Barr F.G., Galili N., Riethman H.C.
     Genomic organization of the human PAX3 gene: DNA sequence analysis of the region disrupted in alveolar rhabdomyosarcoma.
[7] Burri M., Tromvoukis Y., Bopp D., Frigerio G., Noll M.
     Conservation of the paired domain in metazoans and its structure in three isolated human genes.
[8] Tassabehji M., Newton V.E., Leverton K., Turnbull K., Seemanova E., Kunze J., Sperling K., Strachan T., Read A.P.
     PAX3 gene structure and mutations: close analogies between Waardenburg syndrome and the Splotch mouse.
[9] Tsukamoto K., Nakamura Y., Niikawa N.
     Isolation of two isoforms of the PAX3 gene transcripts and their tissue-specific alternative expression in human adult tissues.
[10] Wachtel M., Dettling M., Koscielniak E., Stegmaier S., Treuner J., Simon-Klingenstein K., Buehlmann P., Niggli F.K., Schaefer B.W.
     Gene expression signatures identify rhabdomyosarcoma subtypes and detect a novel t(2;2)(q35;p23) translocation fusing PAX3 to NCOA1.

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Precautions: PAX3 antibody is for in vitro research use only. Not for use in diagnostics or therapeutic procedures.

Important Notes: During shipment, small volumes of PAX3 antibody vial. For products with volumes of 200 µL or less, we recommend gently tapping the vial on a hard surface or briefly centrifuging the vial in a tabletop centrifuge to dislodge any liquid in the container’s cap. Actual concentration, volume and quantity will be printed on the vial's label. Please refer to the vials label for this information.

Copyright: This GenWay TDS is copyrighted. This datasheet is produced based partially on data from Swiss-Prot/TrEMBL and NCBI. To better serve our clients with everything we know about PAX3 antibody, all related information, articles, resources about PAX3 antibody are being stored on our online database. Let us know if you have questions regarding this product.

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