reproductive system
vision/eye
• the stacking of the discs in the outer segment (OS) is slightly disoriented and non-uniform at P20 and by 6 months of age, photoreceptors are greatly reduced and disorganized, with shortened, fragmented OS
|
• by 6 months of age, photoreceptors have shorted and fragmented outer segment
|
• expansion of the distal endoplasmic reticulum (near the base of the outer segment) in the rods
• increase in the number of mitochondria in the ellipsoid region of rods
|
• mice exhibit progressive photoreceptor degeneration from P20 onwards
|
• progressive thinning of the outer nuclear layer (ONL), with mild thinning at P20, severe thinning at 2 months, and only 40% of ONL rows remaining in the retina at 4 months of age
|
• electroretinograms show that photoreceptor dysfunction precedes degeneration
|
• full-field electroretinography of dark-adapted mice shows a reduction in the scotopic response to light as early as P20, with both the a-wave and b-wave amplitudes deceased and severely reduced amplitudes at 2 and 4 months of age
|
• full-field electroretinography of dark-adapted mice shows a reduction in the scotopic response to light as early as P20, with both the a-wave and b-wave amplitudes deceased and severely reduced amplitudes at 2 and 4 months of age
|
nervous system
• the stacking of the discs in the outer segment (OS) is slightly disoriented and non-uniform at P20 and by 6 months of age, photoreceptors are greatly reduced and disorganized, with shortened, fragmented OS
|
• by 6 months of age, photoreceptors have shorted and fragmented outer segment
|
• expansion of the distal endoplasmic reticulum (near the base of the outer segment) in the rods
• increase in the number of mitochondria in the ellipsoid region of rods
|
• mice exhibit progressive photoreceptor degeneration from P20 onwards
|
cellular
• marker analysis indicates ER stress in photoreceptor cells
|