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Rabbit Anti-PAX3 Recombinant Antibody (16H22L10) (CBMAB-1654-CN)


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Summary

Host Animal
Rabbit
Specificity
Human, Mouse
Clone
16H22L10
Antibody Isotype
IgG
Application
WB, IF, ICC, ELISA

Basic Information

Immunogen
Recombinant protein corresponding to aa. 34–276 of human Pax 3
Specificity
Human, Mouse
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS
Preservative
0.09% Sodium azide
Concentration
0.5 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
PAX3
Introduction
Members of the PAX family typically contain a paired box domain and a paired-type homeodomain and play critical roles during fetal development. PAX3 (paired box 3) is a member of the paired box (PAX) family of transcription factors. Mutations in PAX3 are associated with Waardenburg syndrome, craniofacial-deafness-hand syndrome, and alveolar rhabdomyosarcoma.
Entrez Gene ID
Human5077
Mouse18505
UniProt ID
HumanP23760
MouseP24610
Alternative Names
WS1; WS3; CDHS; HUP2
Function
Transcription factor that may regulate cell proliferation, migration and apoptosis. Involved in neural development and myogenesis. Transcriptional activator of MITF, acting synergistically with SOX10 (PubMed:21965087).
Biological Process
Anatomical structure developmentManual Assertion Based On ExperimentIBA:GO_Central
Animal organ morphogenesisManual Assertion Based On ExperimentTAS:ProtInc
Apoptotic processManual Assertion Based On ExperimentTAS:ProtInc
Muscle organ developmentIEA:UniProtKB-KW
Nervous system developmentIEA:UniProtKB-KW
Positive regulation of transcription by RNA polymerase IIManual Assertion Based On ExperimentIDA:MGI
Positive regulation of transcription, DNA-templatedManual Assertion Based On ExperimentIDA:MGI
Regulation of transcription by RNA polymerase IIManual Assertion Based On ExperimentIBA:GO_Central
Sensory perception of soundManual Assertion Based On ExperimentTAS:ProtInc
Cellular Location
Nucleus
Involvement in disease
Waardenburg syndrome 1 (WS1):
WS1 is an autosomal dominant disorder characterized by non-progressive sensorineural deafness, pigmentary disturbances such as frontal white blaze of hair, heterochromia of irides, white eyelashes, leukoderma, and wide bridge of nose owing to lateral displacement of the inner canthus of each eye (dystopia canthorum). WS1 shows variable clinical expression and some affected individuals do not manifest hearing impairment or iris pigmentation disturbances. Dystopia canthorum is the most consistent sign and is found in 98% of the patients.
Waardenburg syndrome 3 (WS3):
WS3 is an autosomal dominant disorder characterized by sensorineural deafness, pigmentary disturbances, dystopia canthorum and limb anomalies such as hypoplasia of the musculoskeletal system, flexion contractures, fusion of the carpal bones, syndactylies.
Craniofacial-deafness-hand syndrome (CDHS):
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.
Rhabdomyosarcoma 2 (RMS2):
A form of rhabdomyosarcoma, a highly malignant tumor of striated muscle derived from primitive mesenchymal 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.
More Infomation

Zhang, K., Yu, C., Tian, R., Zhang, W., Tang, S., & Wang, G. (2022). Downregulation of the paired box gene 3 inhibits the progression of skin cutaneous melanoma by inhibiting c-MET tyrosine kinase: PAX3 downregulation inhibits melanoma progression. Molecular Biology Reports, 49(10), 9137-9145.

Azhar, M., Wardhani, B. W. K., & Renesteen, E. (2022). The regenerative potential of Pax3/Pax7 on skeletal muscle injury. Journal of Genetic Engineering and Biotechnology, 20(1), 143.

Panagopoulos, I., Gorunova, L., Andersen, K., Lund-Iversen, M., Tafjord, S., Micci, F., & Heim, S. (2021). Fusion of the paired box 3 (PAX3) and myocardin (MYOCD) genes in pediatric rhabdomyosarcoma. Cancer Genomics & Proteomics, 18(6), 723-734.

Liu, Y., Cui, S., Li, W., Zhao, Y., Yan, X., & Xu, J. (2019). PAX3 is a biomarker and prognostic factor in melanoma: Database mining. Oncology Letters, 17(6), 4985-4993.

Chay, D. B., Han, G. H., Nam, S., Cho, H., Chung, J. Y., & Hewitt, S. M. (2019). Forkhead box protein O1 (FOXO1) and paired box gene 3 (PAX3) overexpression is associated with poor prognosis in patients with cervical cancer. International journal of clinical oncology, 24, 1429-1439.

Angelopoulou, E., Paudel, Y. N., & Piperi, C. (2019). Emerging pathogenic and prognostic significance of paired box 3 (PAX3) protein in adult gliomas. Translational Oncology, 12(10), 1357-1363.

Han, G. H., Chay, D. B., Nam, S., Cho, H., Chung, J. Y., & Kim, J. H. (2019). Prognostic implications of forkhead box protein O1 (FOXO1) and paired box 3 (PAX3) in epithelial ovarian cancer. BMC cancer, 19, 1-10.

Der Vartanian, A., Quétin, M., Michineau, S., Auradé, F., Hayashi, S., Dubois, C., ... & Relaix, F. (2019). PAX3 confers functional heterogeneity in skeletal muscle stem cell responses to environmental stress. Cell stem cell, 24(6), 958-973.

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For research use only. Not intended for any clinical use.

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