vision/eye
• reduction in the number of calbindin+ amacrine cells at P270, but not earlier time points
|
• rod bipolar cells show a progressive loss of dendrites that is first observed at P90 and is complete by P270
|
• progressive loss of photoreceptor rows is evident by P90, however the outer nuclear layer is not completely lost and at P270, 2-3 rows of photoreceptor cells are retained
|
• smaller terminal end-bulbs and varicosities in the sublamine 5 of the inner plexiform layer are seen from P90
• end-bulbs of axonal terminals are disorganized and smaller
|
• in the oldest mutants (P180 and P270) some horizontal cell somas are abnormally located in the outer nuclear layer
|
• progressive reduction in thickness due to photoreceptor death
|
• magnitude of age-related ERG amplitude reduction is more pronounced in mutants than in wild-type mice, especially at later ages
• ERG amplitudes evoked by high intensity stimuli presented to the dark-adapted eye are smaller than in wild-type at all ages tested for the a-wave and at all ages except P45 for the b-wave
• oscillatory potential amplitudes in response to a light flash are different from wild-type
|
• amplitudes of the cone ERG b-wave is decreased in mutants at P90 and older
• however, no differences from wild-type in cone flicker ERGs are seen
|
• amplitude of the rod b-wave is reduced in mutants for all postnatal days measured after P90
|
nervous system
• reduction in the number of calbindin+ amacrine cells at P270, but not earlier time points
|
• rod bipolar cells show a progressive loss of dendrites that is first observed at P90 and is complete by P270
|
• progressive loss of photoreceptor rows is evident by P90, however the outer nuclear layer is not completely lost and at P270, 2-3 rows of photoreceptor cells are retained
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
retinitis pigmentosa | DOID:10584 |
OMIM:PS268000 |
J:189268 |