All Phenotypes Pex11b<tm1Sjg> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Pex11b^tm1Sjg
targeted mutation 1, Stephen J Gould
MGI:2181008

Summary

4 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Pex11b^tm1Sjg/Pex11b^tm1Sjg	B6.129-Pex11b^tm1Sjg	MGI:5307126
hm2	Pex11b^tm1Sjg/Pex11b^tm1Sjg	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:3042895
ht3	Pex11b^tm1Sjg/Pex11b⁺	B6.129-Pex11b^tm1Sjg	MGI:5307125
cx4	Pex11a^tm1Sjg/Pex11a^tm1Sjg Pex11b^tm1Sjg/Pex11b^tm1Sjg	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:3042891

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pex11b^tm1Sjg mutation (0 available); any Pex11b mutation (14 available)

♀	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

Allelic Composition	Pex11b^tm1Sjg/Pex11b^tm1Sjg
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pex11b^tm1Sjg mutation (0 available); any Pex11b mutation (14 available)

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

mortality/aging

neonatal lethality, complete penetrance ( J:76782 )

• died within the first day after birth

behavior/neurological

abnormal suckling behavior ( J:76782 )

• suckled poorly

cellular

increased mitochondrial number ( J:76782 )

• some mitochondrial proliferation seen

abnormal peroxisome morphology ( J:76782 )

• peroxisome number about 1/2 normal

• increased clustering and elongation of peroxisomes

abnormal peroxisome physiology ( J:76782 )

• very mild defects in peroxisomal metabolic functions

• no abnormalities in peroxisomal protein import

craniofacial

craniofacial phenotype ( J:76782 )

N	• lack the facial dysmorphism expected in Zellweger Syndrome

abnormal neurocranium morphology ( J:76782 )

• delayed ossification of calvaria

growth/size/body

decreased body size ( J:76782 )

• mice were 80% of normal size at birth

decreased body weight ( J:76782 )

• mice were only 60% of normal body weight at birth

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:76782 )

N	• no very long chain fatty acid accumulation occurred as would be expected in Zellweger Syndrome

decreased liver glycogen level ( J:76782 )

• decreased levels of glycogen in the liver

liver/biliary system

decreased liver glycogen level ( J:76782 )

• decreased levels of glycogen in the liver

delayed hepatic development ( J:76782 )

• focal mosaic pattern of developmental delay

skeleton

abnormal neurocranium morphology ( J:76782 )

• delayed ossification of calvaria

nervous system

abnormal cerebral cortex morphology ( J:76782 )

• focal areas of decreased neuronal migration in neocortex

• increase in intermediate zone and layer V neurons

• reduced thickness of cortical plate with structural alterations as well

• abnormalities are seen embryonically as well (day not stated)

• enhanced neuronal apoptosis

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Zellweger syndrome		DOID:905		J:76782

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pex11b^tm1Sjg mutation (0 available); any Pex11b mutation (14 available)

♀	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 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

abnormal neuron differentiation ( J:180632 )

• marker analysis indicates that mutants exhibit a delay in neuronal differentiation, but to a smaller extent than in homozygotes

oxidative stress ( J:180632 )

• mutants exhibit an increase in oxidative stress in brain sections and primary neural cultures, but less than in homozygotes

nervous system

increased neuron apoptosis ( J:180632 )

• 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

abnormal neuron differentiation ( J:180632 )

• marker analysis indicates that mutants exhibit a delay in neuronal differentiation, but to a smaller extent than in homozygotes

abnormal neocortex morphology ( J:180632 )

• brain sections from E19 fetuses exhibit a 15% increase in the number of peroxisomes/area in the cerebellum and medial neocortex

abnormal cerebellum morphology ( J:180632 )

• brain sections from E19 fetuses exhibit a 15% increase in the number of peroxisomes/area in the cerebellum and medial neocortex

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

Allelic Composition	Pex11a^tm1Sjg/Pex11a^tm1Sjg Pex11b^tm1Sjg/Pex11b^tm1Sjg
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pex11a^tm1Sjg mutation (0 available); any Pex11a mutation (7 available)
Pex11b^tm1Sjg mutation (0 available); any Pex11b mutation (14 available)

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

mortality/aging

perinatal lethality ( J:80124 )

prenatal lethality ( J:80124 )

cellular

abnormal peroxisome morphology ( J:80124 )

• peroxisome number about 1/2 normal, about the same as in Pex11b^tm1Sjg homozygotes

abnormal peroxisome physiology ( J:80124 )

• very mild defects in peroxisomal metabolic functions

• no abnormalities in peroxisomal protein import

growth/size/body

abnormal embryonic growth/weight/body size ( J:80124 )

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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