Mouse Genome Informatics
hm
    Zmpste24tm1Sgy/Zmpste24tm1Sgy
B6.129S4-Zmpste24tm1Sgy
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• treatment with an FTI increased the survival of both male and female mutants significantly compared with vehicle-treated mutant controls; by 20 weeks of age, 6 of 14 vehicle-treated mutants had died, but only 1 of 13 FTI-treated mutants was dead

growth/size
• male and female mutant mice treated with an FTI gain more weight than vehicle treated controls

skeleton
• at 20 weeks, the median number of rib fractures in FTO-treated mice is 2, compared to 14 in vehicle treated mice

behavior/neurological
• 100% of male and female mutant mice receiving vehicle exhibit abnormal grip strength by 16 weeks of age; only 28% of female and 33% of male mutants receiving a farnesyltransferase inhibitor (FTI) show a grip abnormality in a grid-hang test
• the delay in appearance of grip abnormalities is significant for male and female mutants
• the mean length of hang time of inhibitor-treated mice is longer than for vehicle treated mice

Mouse Models of Human Disease
OMIM IDRef(s)
Hutchinson-Gilford Progeria Syndrome; HGPS 176670 J:106706