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Phenotypes Associated with This Genotype
Genotype
MGI:3804449
Allelic
Composition
Ercc1tm1Jhjh/Ercc1tm1Jhjh
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ercc1tm1Jhjh mutation (0 available); any Ercc1 mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• most die by four weeks after birth (J:117488)
• most die by four weeks after birth (J:117488)

growth/size/body
• mildly retarded (J:117488)
• mildly retarded (J:117488)
• growth arrests in the second week of life (J:117488)
• growth arrests in the second week of life (J:117488)
• mildly retarded (J:117488)
• mildly retarded (J:117488)
• mildly retarded (J:117488)
• mildly retarded (J:117488)

embryogenesis
• mildly retarded (J:117488)
• mildly retarded (J:117488)

cellular
• apoptotic cells are increased in mutant liver compared to age-matched controls (J:117488)
• apoptotic cells are increased in mutant liver compared to age-matched controls (J:117488)
• mutant ES cells and MEFs are mildly, but significantly, hypersensitive to oxidative damage compared to wild-type (J:117488)
• mutant ES cells and MEFs are mildly, but significantly, hypersensitive to oxidative damage compared to wild-type (J:117488)
• cellular proliferation is decreased compared to age matched controls (J:117488)
• cellular proliferation is decreased compared to age matched controls (J:117488)
• senescent, polyploid hepatocytes are prominent in mutants but not age-matched controls (J:117488)
• senescent, polyploid hepatocytes are prominent in mutants but not age-matched controls (J:117488)
• MEFS exhibit premature replicative senescence (J:117488)
• MEFS exhibit premature replicative senescence (J:117488)

behavior/neurological
• progressive ataxia (J:117488)
• progressive ataxia (J:117488)

homeostasis/metabolism
• triglyceride accumulation is observed in the liver in young mutants as is seen in older controls (J:117488)
• triglyceride accumulation is observed in the liver in young mutants as is seen in older controls (J:117488)

liver/biliary system
• apoptotic cells are increased in mutant liver compared to age-matched controls (J:117488)
• apoptotic cells are increased in mutant liver compared to age-matched controls (J:117488)
• senescent, polyploid hepatocytes are prominent in mutants but not age-matched controls (J:117488)
• senescent, polyploid hepatocytes are prominent in mutants but not age-matched controls (J:117488)
• triglyceride accumulation is observed in the liver in young mutants as is seen in older controls (J:117488)
• triglyceride accumulation is observed in the liver in young mutants as is seen in older controls (J:117488)
• progressive steatosis (J:117488)
• progressive steatosis (J:117488)

muscle
• sarcopenia (J:117488)
• sarcopenia (J:117488)

renal/urinary system
• renal insufficiency (J:117488)
• renal insufficiency (J:117488)

skeleton

Mouse Models of Human Disease
OMIM ID Ref(s)
Xfe Progeroid Syndrome; XFEPS 610965 J:117488


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