Phenotypes associated with this allele

• Allele Symbol: Usp18^tm1Dez
• Allele Name: targeted mutation 1, Dong-Er Zhang
• Allele ID: MGI:2387336
• Summary: 4 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Usp18^tm1Dez/Usp18^tm1Dez	involves: 129S1/Sv * 129X1/SvJ	MGI:2450155
hm2	Usp18^tm1Dez/Usp18^tm1Dez	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:5908648
ht3	Usp18^ity9/Usp18^tm1Dez	involves: 129 * C57BL/6 * FVB/N	MGI:4830365
cx4	Uba7^tm1Dez/Uba7^tm1Dez Usp18^tm1Dez/Usp18^tm1Dez	involves: 129 * C57BL/6	MGI:3830335

Genotype
MGI:2450155

hm1

• Allelic Composition: Usp18^tm1Dez/Usp18^tm1Dez
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ

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

mortality/aging

premature death ( J:78821 )

behavior/neurological

tremors ( J:78821 )

circling ( J:78821 )

• tight circling

spinning ( J:78821 )

• described as rolling

convulsive seizures ( J:78821 )

cardiovascular system

intracerebral hemorrhage ( J:78821 )

• bloody cerebrospinal fluid was noted

nervous system

convulsive seizures ( J:78821 )

intracerebral hemorrhage ( J:78821 )

• bloody cerebrospinal fluid was noted

hydrocephaly ( J:78821 )

• hydroencephaly first appears at postnatal day 14

enlarged lateral ventricles ( J:78821 )

• bilateral enlargement of lateral ventricle and third ventricle

enlarged third ventricle ( J:78821 )

• bilateral enlargement of lateral ventricle and third ventricle

abnormal cerebral cortex morphology ( J:78821 )

• thinned cerebral cortex

Genotype
MGI:5908648

hm2

• Allelic Composition: Usp18^tm1Dez/Usp18^tm1Dez
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6

immune system

abnormal cytokine level ( J:214425 )

• MHV-3 infection does not induce type I or type II IFN induction in primary hepatocytes

• MHV-3-infected peritoneal exudative macrophages do not show increased type I IFN signaling

decreased circulating interferon-alpha level ( J:214425 )

• MHV-3-infected mice show no induction of IFN-alpha hepatic mRNA and serum protein levels as seen in wild-type mice

decreased circulating interferon-beta level ( J:214425 )

• MHV-3-infected mice show no induction of IFN-beta hepatic mRNA and serum protein levels as seen in wild-type mice

decreased circulating interferon-gamma level ( J:214425 )

• MHV-3-infected mice show no induction of IFN-gamma hepatic mRNA and serum protein levels as seen in wild-type mice

decreased susceptibility to Coronaviridae infection ( J:214425 )

• mice infected with the murine hepatitis virus strain 3 (MHV-3) coronavirus exhibit extended survival (up to 10 days compared to 4 in wild-type) and improved liver histology, decreased inflammatory response, and decreased viral titers than wild-type mice, however, infection gradually does lead to widespread liver damage of 40-50% focal necrosis but never as severe as in wild-type mice and mice eventually succumb to infection

homeostasis/metabolism

abnormal circulating enzyme level ( J:214425 )

• MHV-3-infected mice show a decrease in the levels of serum transaminases

abnormal cytokine level ( J:214425 )

• MHV-3 infection does not induce type I or type II IFN induction in primary hepatocytes

• MHV-3-infected peritoneal exudative macrophages do not show increased type I IFN signaling

decreased circulating interferon-alpha level ( J:214425 )

• MHV-3-infected mice show no induction of IFN-alpha hepatic mRNA and serum protein levels as seen in wild-type mice

decreased circulating interferon-beta level ( J:214425 )

• MHV-3-infected mice show no induction of IFN-beta hepatic mRNA and serum protein levels as seen in wild-type mice

decreased circulating interferon-gamma level ( J:214425 )

• MHV-3-infected mice show no induction of IFN-gamma hepatic mRNA and serum protein levels as seen in wild-type mice

Genotype
MGI:4830365

ht3

• Allelic Composition: Usp18^ity9/Usp18^tm1Dez
• Genetic Background: involves: 129 * C57BL/6 * FVB/N

mortality/aging

increased susceptibility to bacterial infection induced morbidity/mortality ( J:163843 )

• Salmonella-infected mice exhibit increased mortality compared with wild-type mice

immune system

increased susceptibility to bacterial infection ( J:163843 )

• Salmonella-infected mice exhibit increased mortality and organ bacterial loads (6-fold higher in the spleen and 28-fold higher in the liver) compared with wild-type mice

increased susceptibility to bacterial infection induced morbidity/mortality ( J:163843 )

• Salmonella-infected mice exhibit increased mortality compared with wild-type mice

Genotype
MGI:3830335

cx4

• Allelic Composition: Uba7^tm1Dez/Uba7^tm1Dez; Usp18^tm1Dez/Usp18^tm1Dez
• Genetic Background: involves: 129 * C57BL/6

cellular

increased sensitivity to induced cell death ( J:144149 )

• bone marrow cells have almost double the amount of apoptotic cells as controls when cultured with interferon-alpha/beta

• the homozygote null Uba7^tm1Dzh alleles do not rescue the increased sensitivity of Usp18^tm1Dzh homozygote cells to IFN-mediated apoptosis