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Phenotypes Associated with This Genotype
Genotype
MGI:3053060
Allelic
Composition
Trex1tm1Tld/Trex1tm1Tld
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Trex1tm1Tld mutation (1 available); any Trex1 mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• median survival time is around 6 months with about 60% of the deaths due to circulatory failure (J:92264)
• survival time for homozygotes born from heterozygous mothers is shorter than that of homozygotes born from homozygous mothers (J:92264)
• median survival time is around 6 months with about 60% of the deaths due to circulatory failure (J:92264)
• survival time for homozygotes born from heterozygous mothers is shorter than that of homozygotes born from homozygous mothers (J:92264)
• median survival is 7 weeks (J:202757)
• median survival is 7 weeks (J:202757)

cardiovascular system
• degeneration of myocytes with edema between the myofibers is seen (J:92264)
• degeneration of myocytes with edema between the myofibers is seen (J:92264)
• mutants that died due to circulatory failure displayed mild to severe cardiomyopathy with 1/3 having a heart 2 to 3 times larger than normal (J:92264)
• cardiomyopathy is not due to increased susceptibility to cardiotoxic viruses (J:92264)
• along with thrombosis one or both atria are enlarged (J:92264)
• mutants that died due to circulatory failure displayed mild to severe cardiomyopathy with 1/3 having a heart 2 to 3 times larger than normal (J:92264)
• cardiomyopathy is not due to increased susceptibility to cardiotoxic viruses (J:92264)
• along with thrombosis one or both atria are enlarged (J:92264)
• inflammatory changes in the heart (J:202757)
• inflammatory changes in the heart (J:202757)
• inflammation of the endothelium extending into the muscle wall is seen in mutant atria (J:92264)
• inflammation of the endothelium extending into the muscle wall is seen in mutant atria (J:92264)

hematopoietic system
• atrophy of the cortical area of the thymus is seen (J:92264)
• atrophy of the cortical area of the thymus is seen (J:92264)
• in less than 10% of older mice enlarged spleens are seen (J:92264)
• in less than 10% of older mice enlarged spleens are seen (J:92264)
• enlarged B-cell follicles are seen (J:92264)
• enlarged B-cell follicles are seen (J:92264)
• indistinct marginal zones are seen (J:92264)
• indistinct marginal zones are seen (J:92264)

immune system
• inflammatory changes in the heart (J:202757)
• inflammatory changes in the heart (J:202757)
• inflammation of the endothelium extending into the muscle wall is seen in mutant atria (J:92264)
• inflammation of the endothelium extending into the muscle wall is seen in mutant atria (J:92264)
• atrophy of the cortical area of the thymus is seen (J:92264)
• atrophy of the cortical area of the thymus is seen (J:92264)
• in less than 10% of older mice enlarged spleens are seen (J:92264)
• in less than 10% of older mice enlarged spleens are seen (J:92264)
• enlarged B-cell follicles are seen (J:92264)
• enlarged B-cell follicles are seen (J:92264)
• indistinct marginal zones are seen (J:92264)
• indistinct marginal zones are seen (J:92264)
• in less than 10% of older mice enlarged lymph nodes are seen (J:92264)
• in less than 10% of older mice enlarged lymph nodes are seen (J:92264)
• inflammatory changes in the skin (J:202757)
• inflammatory changes in the skin (J:202757)

liver/biliary system
• necrotic areas in the liver are occasionally seen (J:92264)
• necrotic areas in the liver are occasionally seen (J:92264)

muscle
• degeneration of myocytes with edema between the myofibers is seen (J:92264)
• degeneration of myocytes with edema between the myofibers is seen (J:92264)
• mutants that died due to circulatory failure displayed mild to severe cardiomyopathy with 1/3 having a heart 2 to 3 times larger than normal (J:92264)
• cardiomyopathy is not due to increased susceptibility to cardiotoxic viruses (J:92264)
• along with thrombosis one or both atria are enlarged (J:92264)
• mutants that died due to circulatory failure displayed mild to severe cardiomyopathy with 1/3 having a heart 2 to 3 times larger than normal (J:92264)
• cardiomyopathy is not due to increased susceptibility to cardiotoxic viruses (J:92264)
• along with thrombosis one or both atria are enlarged (J:92264)

renal/urinary system
• necrotic areas in the kidney are occasionally seen (J:92264)
• necrotic areas in the kidney are occasionally seen (J:92264)

homeostasis/metabolism
• thrombus formation is seen in one or both of the atria in mutants that died from circulatory failure (J:92264)
• thrombus formation is seen in one or both of the atria in mutants that died from circulatory failure (J:92264)

integument
• inflammatory changes in the skin (J:202757)
• inflammatory changes in the skin (J:202757)

endocrine/exocrine glands
• atrophy of the cortical area of the thymus is seen (J:92264)
• atrophy of the cortical area of the thymus is seen (J:92264)

Mouse Models of Human Disease
OMIM ID Ref(s)
Aicardi-Goutieres Syndrome 1; AGS1 225750 J:145466


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