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Phenotypes Associated with This Genotype
Genotype
MGI:3722830
Allelic
Composition
Pex13tm1.1Crne/Pex13tm1.1Crne
Genetic
Background
involves: 129T2/SvEms * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pex13tm1.1Crne mutation (0 available); any Pex13 mutation (21 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• pups die soon after birth (6-12 hours) (J:84795)
• pups die soon after birth (6-12 hours) (J:84795)

growth/size/body
• body weight of pups is 73% of wild-type (J:84795)
• body weight of pups is 73% of wild-type (J:84795)

behavior/neurological
• pups fail to feed (J:84795)
• pups fail to feed (J:84795)
• pups are hypoactive (J:84795)
• pups are hypoactive (J:84795)

cellular
• lack morphologically intact peroxisomes (J:84795)
• lack morphologically intact peroxisomes (J:84795)
• structure of mitochondria is abnormal (J:84795)
• structure of mitochondria is abnormal (J:84795)
• abnormal cristae (J:84795)
• abnormal cristae (J:84795)
• analysis of tissue and cultured skin fibroblasts indicate severe impairment of peroxisomal fatty acid oxidation and plasmalogen synthesis (J:84795)
• analysis of tissue and cultured skin fibroblasts indicate severe impairment of peroxisomal fatty acid oxidation and plasmalogen synthesis (J:84795)
• cultured cerebellar neurons exhibit a 40% decrease in mitochondrial dehydrogenase activity (J:167578)
• cultured cerebellar neurons exhibit a 40% decrease in mitochondrial dehydrogenase activity (J:167578)
• cultured cerebellar neurons from E19 mice show increased oxidative stress and apoptosis (J:167578)
• cultured cerebellar neurons from E19 mice show increased oxidative stress and apoptosis (J:167578)

endocrine/exocrine glands
• myelin-like lipid structures are seen in some adrenocortical cells (J:84795)
• myelin-like lipid structures are seen in some adrenocortical cells (J:84795)

liver/biliary system
• hepatocytes exhibit an abundance of large lipid droplets (J:84795)
• hepatocytes exhibit an abundance of large lipid droplets (J:84795)

muscle
• severe hypotonia, with pups maintaining a contracted C posture (J:84795)
• severe hypotonia, with pups maintaining a contracted C posture (J:84795)

nervous system
• brains show disordered lamination in the cerebral cortex (J:84795)
• brains show disordered lamination in the cerebral cortex (J:84795)
• much of the cortical mantle is densely populated by degenerating neurons characterized by small, round, hyperchromatic nuclei, often with a thin rim of amphophilic cytoplasm (J:84795)
• much of the cortical mantle is densely populated by degenerating neurons characterized by small, round, hyperchromatic nuclei, often with a thin rim of amphophilic cytoplasm (J:84795)

renal/urinary system
• podocyte foot processes are scarce (J:84795)
• podocyte foot processes are scarce (J:84795)
• delayed differentiation of glomeruli as evidenced by the reduced size and capillary structure and paucity of podocyte foot processes (J:84795)
• delayed differentiation of glomeruli as evidenced by the reduced size and capillary structure and paucity of podocyte foot processes (J:84795)

respiratory system
• seen in a few rare cases (J:84795)
• seen in a few rare cases (J:84795)

Mouse Models of Human Disease
OMIM ID Ref(s)
Peroxisome Biogenesis Disorder 11a (zellweger); PBD11A 614883 J:84795


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory