Mouse Genome Informatics
hm1
    Myo7ash1-11J/Myo7ash1-11J
129.B6-Myo7ash1-11J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
• mutants exposed to moderate intensity light show an accumulation of superoxide in rods at a level 3.5 times greater than in wild-type mice
• mutants exhibit rod photoreceptor degeneration in response to moderate intensity light exposure that is not seen in wild-type mice
• mutants reared under a moderate light/dark cycle, but not dim/dark cycle conditions, develop severe retinal degeneration in less than 6 months

vision/eye
• mutants exposed to moderate intensity light show an accumulation of superoxide in rods at a level 3.5 times greater than in wild-type mice
• mutants exhibit rod photoreceptor degeneration in response to moderate intensity light exposure that is not seen in wild-type mice
• mutants reared under a moderate light/dark cycle, but not dim/dark cycle conditions, develop severe retinal degeneration in less than 6 months

Mouse Models of Human Disease
OMIM IDRef(s)
Usher Syndrome, Type I; USH1 276900 J:181430


Mouse Genome Informatics
hm2
    Myo7ash1-11J/Myo7ash1-11J
C57BL/6J-Myo7ash1-11J/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
behavior/neurological
• mutants exhibit a head tilt from weaning age, approximately 3-4 weeks
• mutant mice exhibit bidirectional circling behavior from weaning age, at approximately 3-4 weeks