Nf1 expression is dependent on strain background: implications for tumor suppressor haploinsufficiency studies.

摘要: Neurofibromatosis type 1 (NF1) is the most common cancer predisposition syndrome affecting nervous system, with elevated risk for both astrocytoma and peripheral nerve sheath tumors. NF1 caused by a germline mutation in gene, tumors showing loss of wild copy NF1. In addition, heterozygosity surrounding stroma important tumor formation, suggesting an additional role haploinsufficiency Studies mouse models families have implicated modifier genes unlinked to severity disease susceptibility To determine if differences Nf1 expression may contribute strain-specific effects on predisposition, we examined levels gene strains using quantitative polymerase chain reaction. The data presented this paper demonstrate that strain background has as much effect one allele, indicating studies must be carefully interpreted respect background. Because do not correlate entirely or resistance observed strain, these suggest either variation responsible Nf1-/+;Trp53-/+cis mice, certain evolved compensatory mechanisms expression.