All Phenotypes Mfrp<rdx> MGI Mouse

vision/eye phenotype ( J:181596 )

N	• retinal pigment epithelium phagocytosis is normal • mice exhibit normal apical retina pigment epithelium domain polarization • mice exhibit normal retinoid cycling as determined by paired-flash electroretinogram • photopic responses are largely preserved until at least 6 months

abnormal ocular fundus morphology ( J:181596 )

• by 4 months, white flecks are widespread and regularly spaced across the entire fundus

• by 21 months, large regions of the fundus are hypopigmented compared to in wild-type mice

abnormal retina morphology ( J:181596 )

• mice exhibit flecked retina disease beginning around 3 months

abnormal photoreceptor outer segment morphology ( J:181596 )

• as early as P10 and more prevalent at 3 months, mice exhibit aberrant pigmented cells among the distal tips of the outer segments unlike in wild-type mice

disorganized photoreceptor outer segment ( J:181596 )

• as early as 3 weeks

retina photoreceptor degeneration ( J:181596 )

• 80% loss of photoreceptors by 12 months

• no photoreceptors remain at 21 months

abnormal retina pigment epithelium morphology ( J:181596 )

• focal areas of degeneration in the retinal pigment layer

• mice exhibit an increase in the number of apical microvilli compared with wild-type mice

thin retina outer nuclear layer ( J:181596 )

• by 6 weeks and progressing slowly through 12 months

retina degeneration ( J:181596 )

• slow

abnormal rod electrophysiology ( J:181596 )

• at 10 weeks, mice exhibit a complete or near complete loss of a-wave and a modest attenuation of b-wave unlike wild-type mice