Affiliation
School of Biology, Chemistry and Health Science, Manchester Metropolitan University, and Department of Pathology Sciences, Christie Hospital, Manchester, United Kingdom.Issue Date
2008-12
Metadata
Show full item recordAbstract
The paired box genes are a family of nine developmental control genes, which in human beings (PAX) and mice (Pax) encode nuclear transcription factors. The temporal and spatial expressions of these highly conserved genes are tightly regulated during foetal development including organogenesis. PAX/Pax genes are switched off during the terminal differentiation of most structures. Specific mutations within a number of PAX/Pax genes lead to developmental abnormalities in both human beings and mice. Mutation in PAX3 causes Waardenburg syndrome, and craniofacial-deafness-hand syndrome. The Splotch phenotype in mouse exhibits defects in neural crest derivatives such as, pigment cells, sympathetic ganglia and cardiac neural crest-derived structures. The PAX family also plays key roles in several human malignancies. In particular, PAX3 is involved in rhabdomyosarcoma and tumours of neural crest origin, including melanoma and neuroblastoma. This review critically evaluates the roles of PAX/Pax in oncogenesis. It especially highlights recent advances in knowledge of how their genetic alterations directly interfere in the transcriptional networks that regulate cell differentiation, proliferation, migration and survival and may contribute to oncogenesis.Citation
Pax genes in embryogenesis and oncogenesis. 2008, 12 (6A):2281-94 J. Cell. Mol. Med.Journal
Journal of Cellular and Molecular MedicineDOI
10.1111/j.1582-4934.2008.00427.xPubMed ID
18627422Type
ArticleLanguage
enISSN
1582-1838ae974a485f413a2113503eed53cd6c53
10.1111/j.1582-4934.2008.00427.x
Scopus Count
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