cellular
• primary neuronal cultures from the neocortex and cerebellum exhibit higher levels of apoptosis than wild-type cultures but not as high as in homozygous cultures
• mutants exhibit a higher number of TUNEL-positive neurons in the medial neocortex and cerebellumthan wild-type mice, although levels are much lower than in homozygotes
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• marker analysis indicates that mutants exhibit a delay in neuronal differentiation, but to a smaller extent than in homozygotes
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• mutants exhibit an increase in oxidative stress in brain sections and primary neural cultures, but less than in homozygotes
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nervous system
• primary neuronal cultures from the neocortex and cerebellum exhibit higher levels of apoptosis than wild-type cultures but not as high as in homozygous cultures
• mutants exhibit a higher number of TUNEL-positive neurons in the medial neocortex and cerebellumthan wild-type mice, although levels are much lower than in homozygotes
|
• marker analysis indicates that mutants exhibit a delay in neuronal differentiation, but to a smaller extent than in homozygotes
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• brain sections from E19 fetuses exhibit a 15% increase in the number of peroxisomes/area in the cerebellum and medial neocortex
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• brain sections from E19 fetuses exhibit a 15% increase in the number of peroxisomes/area in the cerebellum and medial neocortex
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Zellweger syndrome | DOID:905 | J:180632 |