All Phenotypes Cerkl<tm1.1Geno> MGI Mouse

decreased retina photoreceptor cell number ( J:292626 )

abnormal retina ganglion cell morphology ( J:292626 )

• retinal ganglion cells grown in culture exhibit abnormal neurite arborization, with shorter axons and increased numbers of neurites, although the length of the dendritic projections is unaltered

• however, no differences in retinal ganglion cell dendrite length, dendritic field area, or number of primary dendrites are seen in vitro

abnormal photoreceptor outer segment disc membrane morphology ( J:292626 )

• photoreceptors exhibit disordered and loosely stacked disks

• photoreceptor outer segments show double membrane vesicles that disrupt the stacking of disks

long photoreceptor outer segment ( J:292626 )

• photoreceptor outer segment length is longer

abnormal retina rod cell outer segment morphology ( J:292626 )

• retinas show longer rod outer segments along the dorsal retina of the retina

• however, the length of rod outer segments and cone sheaths are normal in the ventral retina

disorganized photoreceptor outer segment ( J:292626 )

• outer segments appear disarrayed

abnormal retina cone cell morphology ( J:292626 )

• S-opsin and M-opsin are detected up to the middle of the cone sheath

retina cone cell degeneration ( J:292626 )

• the number of cones is decreased in 2-month-old mice and is diminished in 11- to 18-month-old mice

abnormal retina pigment epithelium morphology ( J:292626 )

• retinal microvilli are disarrayed and do not wrap the outer segment tips, indicating defective phagocytosis

• distribution of phagosomes in retinal pigment cells of old mice is altered and the number of basal endosomes is increased

• total number of lysosomes is increased close to two-fold in the retinal pigment epithelium

cataract ( J:292626 )

• the lens of old, but not young, mice shows non-uniform calcified cataracts

abnormal retina outer nuclear layer morphology ( J:292626 )

• retinas exhibit a wavy outer nuclear layer, with zones with fewer nuclei and others with an increased number of nuclei

thin retina outer nuclear layer ( J:292626 )

• decrease in the number of the nuclei rows in the outer nuclear layer on both sides of the retina

disorganized retina layers ( J:292626 )

• mice show severe layer organization defects

increased susceptibility to age-related retinal degeneration ( J:292626 )

• the number of photoreceptor nuclei rows is decreased along the whole retina, indicating severe retinal degeneration

• retinal degeneration is seen by 18 months of age

abnormal total retina thickness ( J:292626 )

• retinas are thicker with enlargement of the inner nuclear and plexiform layers in half of mice and appear thinner in the other half of mice, indicating irregular thickness along the retina

retina gliosis ( J:292626 )

• retinas show an increase in GFAP expression indicating reactive gliosis

abnormal electroretinogram waveform feature ( J:292626 )

• oscillatory potential responses, reflecting the synaptic activity of retinal ganglion cells and amacrine cells, are decreased

decreased b-wave amplitude ( J:292626 )

• scotopic and photopic b-wave amplitudes are decreased and postsynaptic activity (b-wave mix) is decreased in mice over 18 months of age, but not in 6-10-month-old mice

abnormal cone electrophysiology ( J:292626 )

• ERG shows that cone activity is diminished in mice over 18 months of age

abnormal rod electrophysiology ( J:292626 )

• ERG shows that rod activity is diminished in mice over 18 months of age

abnormal vision ( J:292626 )

• progressive vision impairment