Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
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Accumulation of intracellular lipid in the liver of fasted Pparatm1Gonz/Pparatm1Gonz mice
mortality/aging
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• 2 males out of 32 (male and female) mutants did not survive a 48 hour fast
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adipose tissue
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• gonadal adipose stores are significantly larger in both female and male mutants
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growth/size/body
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• body weight is significantly elevated in male mutants on a 129S4/SvJae background compared to wild-type
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• after a 48 hour fast livers in mutant mice are slightly enlarged and appear pale
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homeostasis/metabolism
liver/biliary system
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• after a 48 hour fast livers in mutant mice are slightly enlarged and appear pale
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• lipid accumulation in the hepatocytes of fasted mutants is massive and homogeneous rather than exhibiting a regional pattern as in wild-types
(J:56056)
• the majority of the lipid accumulation is in the triglyceride fraction
(J:56056)
• in 6 month old mutants hepatic accumulation of lipids is increased
(J:72185)
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cardiovascular system
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• patchy regions of neutral lipid accumulation are found in the myocardium after fasting
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muscle
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• patchy regions of neutral lipid accumulation are found in the myocardium after fasting
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
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adipose tissue
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• gonadal adipose stores are significantly larger in both female and male mutants
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homeostasis/metabolism
liver/biliary system
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• in 6 month old mutants hepatic accumulation of lipids is increased
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
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Pparatm1Gonz/Pparatm1Gonz mice exhibit myocardial fibrosis and degeneration with age
cardiovascular system
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• number of caveolae in the cardiac capillary endothelium is increased
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• cristae of mitochondria increase in number and density in myocardial cells at 16 and 32 weeks of age
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• hearts at 16 weeks of age show myocardial degeneration associated with contraction band necrosis
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• exhibit contraction band necrosis
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• hearts at 16 weeks of age show a little focal fibrosis and by 32 weeks of age, see diffuse fibrosis occupying 1/3 of the inner wall of the myocardium
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• starvation stress and starvation plus high temperature stress significantly decrease the ATP concentration and increase the calcium concentration in hearts of mutants but not controls
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• the capacity for constitutive myocardial beta-oxidation of the medium and long chain fatty acids, octanoic acid and palmitic acid, is reduced compared to wild-type, indicating impaired mitochondrial fatty acid catabolism
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• seen at 16 and 32 weeks of age
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• exhibit age-dependent cardiac damage
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• inflammatory infiltrates are predominantly composed of macrophages with few lymphocytes and neutrophils
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immune system
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• inflammatory infiltrates are predominantly composed of macrophages with few lymphocytes and neutrophils
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muscle
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• cristae of mitochondria increase in number and density in myocardial cells at 16 and 32 weeks of age
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• hearts at 16 weeks of age show myocardial degeneration associated with contraction band necrosis
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• exhibit contraction band necrosis
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• exhibit age-dependent cardiac damage
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homeostasis/metabolism
integument
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N |
• no epidermal phenotype is apparent; epidermal layer and surface lipid distribution appear normal
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cellular
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• exhibit contraction band necrosis
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
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renal/urinary system
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N |
• mice exhibit normal kidney histology
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
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homeostasis/metabolism
muscle
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• when fed a high fat diet, muscle glucose uptake is improved compared with similarly treated wild-type mice
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• in the gastrocnemius when fed a high fat diet
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growth/size/body
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• when fed a high fat diet
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cellular
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• when fed a high fat diet, muscle glucose uptake is improved compared with similarly treated wild-type mice
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
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mortality/aging
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• inhibition of mitochondrial long chain fatty acid transport results the death of all male mutants but only 25% (2/8) of female mutants, no wild-types died as a result of this treatment
• intraperitoneal injection of 100ul of a 50% dextrose solution rescues these mice
• estradiol pretreatment rescues these male mice
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cardiovascular system
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• the cardiomyopathic effects (increase in biventricular weight to body weight ratio, increase in left ventricular mass index, hypertrophy) of diabetes are not seen in insulin deficient mutants
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homeostasis/metabolism
liver/biliary system
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• homozygotes do not develop liver enlargement following treatment with peroxisome proliferators
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• in male mutants compared to female mutants inhibition of mitochondrial long chain fatty acid transport produces a marked decrease in hepatic glycogen levels
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• the number of liver peroxisomes does not increase after treatment with peroxisome proliferators
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• lipid droplets accumulate in livers of mutant mice treated with peroxisome proliferators
(J:25516)
• inhibition of mitochondrial long chain fatty acid transport resulted in fatty degeneration of the liver
(J:50024)
• in male mutants almost all the accumulated lipid is triglyceride
(J:50024)
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muscle
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• the cardiomyopathic effects (increase in biventricular weight to body weight ratio, increase in left ventricular mass index, hypertrophy) of diabetes are not seen in insulin deficient mutants
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integument
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• between E18.5 and birth mutants display a delay in cornified layer development with a significant reduction in the number of cell layers
• processing of lamellar bilayers at the level of the first extracellular space above the stratum granulosum- cornified layer interface is delayed at E18.5
• no defect in lamellar bilayers is seen in adults
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growth/size/body
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• homozygotes do not develop liver enlargement following treatment with peroxisome proliferators
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
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integument
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• foci of keratinocytes with nuclei are found in the stratum corneum
• mutants do not respond to peroxisome proliferators with thinning of the epidermis
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• fewer keratohylin granules within the granular cells
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• the granular layer is thinner
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cellular
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• foci of keratinocytes with nuclei are found in the stratum corneum
• mutants do not respond to peroxisome proliferators with thinning of the epidermis
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm1.1Pcha mutation
(0 available);
any
Pkd1 mutation
(153 available)
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
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renal/urinary system
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• cyst epithelial proliferation is increased
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• all mice exhibit numerous kidney cysts
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• kidney-weight to body-weight ratio is increased
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cellular
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• cyst epithelial proliferation is increased
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growth/size/body
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• all mice exhibit numerous kidney cysts
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• kidney-weight to body-weight ratio is increased
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homeostasis/metabolism
growth/size/body
adipose tissue
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• after dexamethasone treatment
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation
(5 available);
any
Ppara mutation
(42 available)
Tg(Ckm-Ppard)LEDpk mutation
(0 available)
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Analysis Tools