权益分类	功能权益	普通用户	{{item.name}}会员
{{category.name}}	{{benefitItem.name}}

A Mutational Model for Childhood Cancer

儿童癌症的突变模型

基本信息

批准号：
7680772
负责人：
LOUISE C STRONG
金额：
$ 4.57万
依托单位：
UNIVERSITY OF TX MD ANDERSON CAN CTR
依托单位国家：
美国
项目类别：
财政年份：
1997
资助国家：
美国
起止时间：
1997-08-01 至 2011-04-30
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/7680772
关键词：
Address Adolescent Adult Affect Anatomy Animal Model Cancer Model Cancer-Predisposing Gene Carcinogenesis Mechanism Cells Childhood Clinical Clonal Evolution Cohort Effect Collaborations Complex Critiques Data Detection Development Disruption Doctor of Medicine Environmental Carcinogens Epidemiologic Studies Family Funding Genes Genetic Genetic Heterogeneity Genetic Predisposition to Disease Genomic Instability Genomics Germ-Line Mutation Goals Guidelines High-Risk Cancer Human Individual Intervention Joints Knowledge Li-Fraumeni Syndrome Literature Malignant Childhood Neoplasm Malignant Neoplasms Methods Modeling Molecular Molecular Epidemiology Molecular Genetics Mutation Nephroblastoma Numbers Organ Pathway interactions Patients Peer Review Predisposition Process Progress Reports Publications Published Comment RB1 gene Regulation Research Research Personnel Resources Retinoblastoma Risk Role Sentinel Series Somatic Mutation Specificity Staging Susceptibility Gene Syndrome System Technology Time TimeLine Tissues Update Variant Work base body system cancer risk carcinogenesis forgetting genetic epidemiology insight kindred mouse model multidisciplinary mutation carrier neoplasm resource novel novel strategies programs prototype sarcoma simulation telomere tumor

项目摘要

A MUTATIONAL MODEL FOR CHILDHOOD CANCER Louise C. Strong, M.D. The overall goal of the program is to identify genes that predispose to childhood cancer, the molecular pathways to tumor development, and the clinical implications. We have focused on two model familial syndromes of childhood and adolescent cancers, sarcomas and Li Fraumeni syndrome (LFS) and its variants, and Wilms' tumor of the kidney. We have developed a multidisciplinary program to investigate genetic susceptibility to childhood and associated cancer using integrated technology of genetic epidemiology, molecular genetics and genomics applied to the rich resources of cancer prone families and mouse models developed in this program. The hypotheses are based on a multi-stage model for cancer. For each tumor type, genetic loci have been identified that may be altered both as germline mutations and as tumor-specific mutations. There is also significant evidence for additional cancer susceptibility genes and risk modifiers, including an effect of generation, at least for LFS. The underlying themes of the program include identification of the underlying cancer susceptibility genes and risk modifiers, analysis of germline and somatic mutations by type and mechanism, determination of the molecular genetic anatomy of the tumors, development of animal models for human cancer susceptibility syndromes, determination of the role of telomere function in cancer risk in LFS and mouse models, and determination of the implications of germline mutations for the patients and their families. The findings from this program should provide insights into the mechanisms of carcinogenesis as well as guidelines for clinical programs for patients at high risk of cancer.

儿童癌症的突变模型