Mouse Genome Informatics
ht
    Smarcb1tm1Sho/Smarcb1+
involves: 129S6/SvEvTac * C57BL/6J * DBA/2J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• 24% of mutants become terminal with malignant rhabdoid tumors within 8 to 10 months of age at a median age of 8.2 months

tumorigenesis
• mice develop malignant rhabdoid tumors, with a median latency of 8 and 10 months of age for spinal cord and soft tissue tumors, respectively
• malignant rhabdoid tumors present as soft tissue tumors mostly on the face and limbs (20%) with occasional paravertebral sites close to the distal end of spinal cord (4%)

Mouse Models of Human Disease
OMIM IDRef(s)
Rhabdoid Tumor Predisposition Syndrome 1; RTPS1 609322 J:120820