Mouse Genome Informatics
ht
    Prph2tm1Nmc/Prph2+
involves: 129S1/Sv * 129X1/SvJ
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
vision/eye
• the outer segment of cells in the outer nuclear layer are disrupted in number and configuration compared to in wild-type mice
• whorl-like structures
• fewer phagosomes are observed than in wild-type mice despite ongoing phagocytosis of the membrane
• at 6 months, the outer nuclear layer (ONL) is only 4 or 5 nuclei thick and there is evidence of ongoing depletion of cells unlike in wild-type mice
• at 10 months, the ONL is only 3 or 4 nuclei thick unlike in wild-type mice
• mice exhibit 9 or 10 rows of photoreceptor nuclei at 2 months, 4 or 5 rows at 6 months, and 2 or 3 rows at 12 months of age indicating a faster degeneration than in Prph2Rd2 heterozygotes
• at 6 months, a- and b-wave of dark-adapted eyes are less than in wild-type mice
• at 10 months, a- and b-waves, maximal dark-adapted responses, and cone-isolated responses are further reduced

nervous system
• the outer segment of cells in the outer nuclear layer are disrupted in number and configuration compared to in wild-type mice
• whorl-like structures

pigmentation
• fewer phagosomes are observed than in wild-type mice despite ongoing phagocytosis of the membrane

Mouse Models of Human Disease
OMIM IDRef(s)
Retinitis Pigmentosa 7; RP7 608133 J:76490