Unknown

Dataset Information

0

The occurrence of intracranial rhabdoid tumours in mice depends on temporal control of Smarcb1 inactivation.


ABSTRACT: Rhabdoid tumours (RTs) are highly aggressive tumours of infancy, frequently localized in the central nervous system (CNS) where they are termed atypical teratoid/rhabdoid tumours (AT/RTs) and characterized by bi-allelic inactivation of the SMARCB1 tumour suppressor gene. In this study, by temporal control of tamoxifen injection in Smarcb1(flox/flox);Rosa26-Cre(ERT2) mice, we explore the phenotypes associated with Smarcb1 inactivation at different developmental stages. Injection before E6, at birth or at 2 months of age recapitulates previously described phenotypes including embryonic lethality, hepatic toxicity or development of T-cell lymphomas, respectively. Injection between E6 and E10 leads to high penetrance tumours, mainly intra-cranial, with short delays (median: 3 months). These tumours demonstrate anatomical, morphological and gene expression profiles consistent with those of human AT/RTs. Moreover, intra- and inter-species comparisons of tumours reveal that human and mouse RTs can be split into different entities that may underline the variety of RT cells of origin.

SUBMITTER: Han ZY 

PROVIDER: S-EPMC4738337 | biostudies-literature | 2016 Jan

REPOSITORIES: biostudies-literature

altmetric image

Publications


Rhabdoid tumours (RTs) are highly aggressive tumours of infancy, frequently localized in the central nervous system (CNS) where they are termed atypical teratoid/rhabdoid tumours (AT/RTs) and characterized by bi-allelic inactivation of the SMARCB1 tumour suppressor gene. In this study, by temporal control of tamoxifen injection in Smarcb1(flox/flox);Rosa26-Cre(ERT2) mice, we explore the phenotypes associated with Smarcb1 inactivation at different developmental stages. Injection before E6, at bir  ...[more]

Similar Datasets

| S-EPMC5563506 | biostudies-literature
| S-EPMC2843150 | biostudies-literature
| S-EPMC8197137 | biostudies-literature
| S-EPMC3075385 | biostudies-literature
| S-EPMC4195815 | biostudies-literature
2021-06-02 | GSE175892 | GEO
| S-EPMC5722562 | biostudies-literature
| S-EPMC7381660 | biostudies-literature
| S-EPMC7876656 | biostudies-literature