Mouse Genome Informatics
ht1
     Del(5D5Mit73-D5Mit351)5Jcs/+
involves: 129S4/SvJae * C3H * C57BL/6J
Key:
♀ phenotype observed in females WTSI Wellcome Trust Sanger Institute
♂ phenotype observed in males Euro Europhenome
N normal phenotype
growth/size
decreased body weight ( J:67077 )
• Background Sensitivity: the severe runting is less severe as the deletion is backcrossed onto the C3H background


Mouse Genome Informatics
ht2
     Del(5D5Mit73-D5Mit351)5Jcs/+
involves: 129S4/SvJae * C57BL/6J
Key:
♀ phenotype observed in females WTSI Wellcome Trust Sanger Institute
♂ phenotype observed in males Euro Europhenome
N normal phenotype
mortality/aging

growth/size
decreased body weight ( J:67077 )
• Background Sensitivity: severe growth retardation averaging only 50-80% of the weight of same-sex wildtype littermates

craniofacial
abnormal craniofacial morphology ( J:67077 )
• Background Sensitivity: the 'Greek warrior helmet' phenotype of domed skull, small ears, and wide set eyes is found on a >50% C57BL/6J background
malocclusion ( J:67077 )
• in some
craniofacial asymmetry ( J:67077 )
• in the most severe cases the face is asymmetric and/or the jaw bones are asymmetric
abnormal palatal rugae morphology ( J:67077 )
• some have misshapened palatine ridges

hearing/vestibular/ear

limbs/digits/tail

skeleton
malocclusion ( J:67077 )
• in some

vision/eye
coloboma ( J:67077 )
• found in some

digestive/alimentary system
abnormal palatal rugae morphology ( J:67077 )
• some have misshapened palatine ridges

behavior/neurological
behavior/neurological phenotype ( J:67077 )
N
• Morris water maze performance normal (J:67077)
abnormal gait ( J:67077 )
• froglike gait with hindlimbs splayed outward during locomotion
seizures ( J:67077 )
• although there is a trend for increased seizure incidence, statistical significance was not found, but severity of seizure is much greater than in controls with half of the seizures in heterozygotes being maximal

nervous system
seizures ( J:67077 )
• although there is a trend for increased seizure incidence, statistical significance was not found, but severity of seizure is much greater than in controls with half of the seizures in heterozygotes being maximal
abnormal cerebellar lobule formation ( J:67077 )
• intraculminate fissure, posterior fissure, and uvular cus are lacking
abnormal cerebral cortex morphology ( J:67077 )
• hypoplasia of the cerebral cortex results in colliculi being more exposed

Mouse Models of Human Disease
 OMIM IDRef(s)
Wolf-Hirschhorn Syndrome; WHS 194190 J:67077