About   Help   FAQ
Phenotypes Associated with This Genotype
Genotype
MGI:3042895
Allelic
Composition
Pex11btm1Sjg/Pex11btm1Sjg
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pex11btm1Sjg mutation (0 available); any Pex11b mutation (10 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• died within the first day after birth

behavior/neurological
• suckled poorly

cellular
• peroxisome number about 1/2 normal
• increased clustering and elongation of peroxisomes
• some mitochondrial proliferation seen
• very mild defects in peroxisomal metabolic functions
• no abnormalities in peroxisomal protein import

craniofacial
N
• lack the facial dysmorphism expected in Zellweger Syndrome
• delayed ossification of calvaria

growth/size/body
• mice were 80% of normal size at birth
• mice were only 60% of normal body weight at birth

homeostasis/metabolism
N
• no very long chain fatty acid accumulation occurred as would be expected in Zellweger Syndrome
• decreased levels of glycogen in the liver

liver/biliary system
• decreased levels of glycogen in the liver
• focal mosaic pattern of developmental delay

skeleton
• delayed ossification of calvaria

nervous system
• 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


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)
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
01/12/2022
MGI 6.17
The Jackson Laboratory