Genetic modeling of Li-Fraumeni syndrome in zebrafish

• Published in: Disease models & mechanisms Vol. 3; no. 1-2; pp. 45 - 56
• Main Authors: Parant, John M, George, Stephen A, Holden, Joseph A, Yost, H Joseph
• Format: Journal Article
• Language: English
• Published: England The Company of Biologists Ltd 01.01.2010; The Company of Biologists Limited
• Subjects: Alleles; Animals; Apoptosis; Apoptosis - radiation effects; Cancer; Cell cycle; Disease Models, Animal; DNA Damage; Gene Knockdown Techniques; Genes, Dominant - genetics; Genetic engineering; Genetic Testing; Genotype & phenotype; Heterozygote; Insects; Kinases; Li-Fraumeni Syndrome - genetics; Loss of Heterozygosity - genetics; Models, Genetic; Mutation; Mutation - genetics; Neoplasms - genetics; Neoplasms - pathology; Protein Stability - radiation effects; Proteins; Proto-Oncogene Proteins c-mdm2 - metabolism; Radiation, Ionizing; Sarcoma; Signal Transduction - radiation effects; Transcriptional Activation - genetics; Transcriptional Activation - radiation effects; Tumor Suppressor Protein p53 - genetics; Tumor Suppressor Protein p53 - metabolism; Tumors; Zebrafish; Zebrafish - genetics

Li-Fraumeni syndrome (LFS) is a highly penetrant, autosomal dominant, human familial cancer predisposition. Although a key role for the tumor suppressor p53 has been implicated in LFS, the genetic and cellular mechanisms underpinning this disease remain unknown. Therefore, modeling LFS in a vertebra...