Phenotypes Associated with This Genotype

Genotype
MGI:6383173

• Allelic Composition: Gpx4^tm1Yana/Gpx4^tm1Yana; Tg(Crx-cre)1Tfur/0; Tg(Gpx4)#Yana/0
• Genetic Background: involves: C57BL/6 * C57BL/6NCrlj * CBA/JNCrlj * DBA/2

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

vision/eye

abnormal retina morphology ( J:182776 )

• lipid peroxidation is increased in inner segment photoreceptor cells at P12, and a modest increase is seen in the other layers of the retina including the inner nuclear layer and ganglion cell layer

abnormal photoreceptor connecting cilium morphology ( J:182776 )

• 80% decrease in the number of connecting cilia in photoreceptor cells

abnormal photoreceptor inner segment morphology ( J:182776 )

• mitochondrial area in inner segment of photoreceptor cells is decreased indicating a decrease in mitochondrial biomass at P11

abnormal photoreceptor outer segment morphology ( J:182776 )

• the outer segment layer is very thin

disorganized photoreceptor outer segment ( J:182776 )

• the outer segment is disorganized and consists of cell membranes that branch and form a large web-like network structure without apparent disc rim and an abnormally wide intra- and inter-outer segment membrane space

thin retina inner nuclear layer ( J:182776 )

• milder thinning or degeneration of the inner nuclear layer

retina outer nuclear layer degeneration ( J:182776 )

• rapid loss of the outer nuclear layer

• an increasing number of TUNEL+ cells are seen in the outer nuclear layer after P12

retina degeneration ( J:182776 )

• mice show rapid and massive retinal degeneration after P12 with none of the photoreceptor cells retained by P21

• cone photoreceptor cells are lost earlier than rod photoreceptor cells

nervous system

abnormal photoreceptor connecting cilium morphology ( J:182776 )

• 80% decrease in the number of connecting cilia in photoreceptor cells

abnormal photoreceptor inner segment morphology ( J:182776 )

• mitochondrial area in inner segment of photoreceptor cells is decreased indicating a decrease in mitochondrial biomass at P11

abnormal photoreceptor outer segment morphology ( J:182776 )

• the outer segment layer is very thin

disorganized photoreceptor outer segment ( J:182776 )

• the outer segment is disorganized and consists of cell membranes that branch and form a large web-like network structure without apparent disc rim and an abnormally wide intra- and inter-outer segment membrane space

cellular

abnormal photoreceptor connecting cilium morphology ( J:182776 )

• 80% decrease in the number of connecting cilia in photoreceptor cells

decreased mitochondrial DNA content ( J:182776 )

• mitochondrial DNA replication in the whole retina is lower