A SMARCB1-Deficient, Highly Penetrant Brain Tumour Mouse Model Recapitulates Human AT/RT

Heterozygous invalidation of SMARCB1 leads to soft-part tumours with long latency (9 months) and weak penetrance in mice (10%-30%). Very few brain tumours are observed in this model. A conditional SMARCB1 inversion gives rise to highly aggressive CD8 lymphomas with a short latency (12 weeks) and a full penetrance, but doesn’t recapitulate human rhabdoid tumours. Hence, a highly penetrant mouse model recapitulating human AT/RT is still awaited, for both cognitive purposes and preclinical therapeutics.