Phenotypes Associated with This Genotype

Genotype
MGI:5307126

• Allelic Composition: Pex11b^tm1Sjg/Pex11b^tm1Sjg
• Genetic Background: B6.129-Pex11b^tm1Sjg

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

cellular

increased neuron apoptosis ( J:180632 )

• primary neuronal cultures from the neocortex and cerebellum exhibit higher levels of apoptosis than wild-type cultures

• mutants exhibit a higher number of TUNEL-positive neurons in the medial neocortex and cerebellum than wild-type mice

abnormal neuron differentiation ( J:180632 )

• marker analysis indicates that mutants exhibit a delay in neuronal differentiation

oxidative stress ( J:180632 )

• mutants exhibit an increase in oxidative stress in brain sections and primary neural cultures

nervous system

increased neuron apoptosis ( J:180632 )

• primary neuronal cultures from the neocortex and cerebellum exhibit higher levels of apoptosis than wild-type cultures

• mutants exhibit a higher number of TUNEL-positive neurons in the medial neocortex and cerebellum than wild-type mice

abnormal neuron differentiation ( J:180632 )

• marker analysis indicates that mutants exhibit a delay in neuronal differentiation

abnormal neocortex morphology ( J:180632 )

• brain sections from E19 fetuses exhibit a 50% reduction in the number of peroxisomes/area in the medial and lateral neocortex compared to wild-type mice

abnormal cerebellum morphology ( J:180632 )

• brain sections from E19 fetuses exhibit a 30% reduction in the number of peroxisomes/area in the cerebellum compared to wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Zellweger syndrome		DOID:905		J:180632