Analysis Tools
renal/urinary system
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• dilated tubules exhibit longer cilium
• Alprostadil treatment shortens cilia length
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• kidneys show the presence of tubular dilatations in the cortex as early as 2 months of age and more evident at 5 months of age
• however, renal function is not impaired in 5-month-old mice
• renal tubular dilatations mainly affect the connecting tubules as well as the junction between the connecting tubules and the distal convoluted tubules
• male mice treated with the prostaglandin E1 agonist Alprostadil daily for 4 months show reduced number and total area of tubular dilatations
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vision/eye
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• retinopathy, with a drastic loss of photoreceptors seen as early as P21
• treatment with the prostaglandin E2 agonist Taprenepag every 3 days from P6 to P21 partially preserves the outer nuclear layer of the photoreceptor
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• scotopic a-wave amplitude is completely absent
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• mice exhibit complete loss of the scotopic response
• Taprenepag treatment leads to improvement of the light-detection capacity of the photoreceptors with an increased magnitude of the b-wave
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cellular
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• dilated tubules exhibit longer cilium
• Alprostadil treatment shortens cilia length
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nervous system
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• retinopathy, with a drastic loss of photoreceptors seen as early as P21
• treatment with the prostaglandin E2 agonist Taprenepag every 3 days from P6 to P21 partially preserves the outer nuclear layer of the photoreceptor
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| nephronophthisis 1 | DOID:0111112 |
OMIM:256100 |
J:360848 | |
