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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Pparatm1Gonz
targeted mutation 1, Frank J Gonzalez
MGI:1857774
Summary 10 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Pparatm1Gonz/Pparatm1Gonz 129S4/SvJae-Pparatm1Gonz MGI:3037495
hm2
Pparatm1Gonz/Pparatm1Gonz B6.129S4-Pparatm1Gonz MGI:3037502
hm3
Pparatm1Gonz/Pparatm1Gonz involves: 129S4/SvJae MGI:3629413
hm4
Pparatm1Gonz/Pparatm1Gonz involves: 129S4/SvJae * C57BL/6 MGI:6273162
hm5
Pparatm1Gonz/Pparatm1Gonz involves: 129S4/SvJae * C57BL/6J MGI:4429479
hm6
Pparatm1Gonz/Pparatm1Gonz involves: 129S4/SvJae * C57BL/6N MGI:3037489
hm7
Pparatm1Gonz/Pparatm1Gonz involves: C57BL/6 MGI:3037531
cx8
Pkd1tm1.1Pcha/Pkd1tm1.1Pcha
Pparatm1Gonz/Pparatm1Gonz
involves: 129 * 129S4/SvJae * C57BL/6 MGI:6317342
cx9
Ldlrtm1Her/Ldlrtm1Her
Pparatm1Gonz/Pparatm1Gonz
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6 MGI:4367223
cx10
Pparatm1Gonz/Pparatm1Gonz
Tg(Ckm-Ppard)LEDpk/0
involves: 129S4/SvJae * C57BL/6 * C57BL/6J * CBA MGI:4429480


Genotype
MGI:3037495
hm1
Allelic
Composition
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
129S4/SvJae-Pparatm1Gonz
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Accumulation of intracellular lipid in the liver of fasted Pparatm1Gonz/Pparatm1Gonz mice

muscle
• patchy regions of neutral lipid accumulation are found in the myocardium after fasting

mortality/aging
• 2 males out of 32 (male and female) mutants did not survive a 48 hour fast

adipose tissue
• gonadal adipose stores are significantly larger in both female and male mutants

growth/size/body
• body weight is significantly elevated in male mutants on a 129S4/SvJae background compared to wild-type
• after a 48 hour fast livers in mutant mice are slightly enlarged and appear pale

homeostasis/metabolism
• fasting did not increase plasma ketone levels despite development of hypoglycemia
• serum concentrations of HDL cholesterol are significantly higher in 9 month-old mutants on a 129S4/SvJae background compared to wild-type
• serum concentrations of cholesterol are significantly higher in 9 month-old mutants on a 129S4/SvJae background compared to wild-type
• after fasting mutants exhibit abnormally high levels of circulating free fatty acids
• in fasted mutants the initial drop in mean blood glucose levels is significantly greater than in fasted wild-types
• mutants do not respond to high fat diet with an increase in plasma glucose
• mutants do not respond to high fat diet with an increase in insulin levels
• mutants on a 129S4/SvJae background fed a high fat diet do not develop insulin resistance

liver/biliary system
• after a 48 hour fast livers in mutant mice are slightly enlarged and appear pale
• 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)

cardiovascular system
• patchy regions of neutral lipid accumulation are found in the myocardium after fasting




Genotype
MGI:3037502
hm2
Allelic
Composition
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
B6.129S4-Pparatm1Gonz
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
adipose tissue
• gonadal adipose stores are significantly larger in both female and male mutants

homeostasis/metabolism
• mutants do not respond to high fat diet with an increase in plasma glucose
• mutants do not respond to high fat diet with an increase in insulin levels
• serum concentrations of triglycerides are significantly higher in 9 month-old mutants on a C57BL/6N background

liver/biliary system
• in 6 month old mutants hepatic accumulation of lipids is increased




Genotype
MGI:3629413
hm3
Allelic
Composition
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
involves: 129S4/SvJae
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Pparatm1Gonz/Pparatm1Gonz mice exhibit myocardial fibrosis and degeneration with age

cardiovascular system
• number of caveolae in the cardiac capillary endothelium is increased
• cristae of mitochondria increase in number and density in myocardial cells at 16 and 32 weeks of age
• hearts at 16 weeks of age show myocardial degeneration associated with contraction band necrosis
• exhibit contraction band necrosis
• 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
• 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
• 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
• seen at 16 and 32 weeks of age
• seen in older mutants
• exhibit age-dependent cardiac damage
• inflammatory infiltrates are predominantly composed of macrophages with few lymphocytes and neutrophils

immune system
• inflammatory infiltrates are predominantly composed of macrophages with few lymphocytes and neutrophils

muscle
• cristae of mitochondria increase in number and density in myocardial cells at 16 and 32 weeks of age
• hearts at 16 weeks of age show myocardial degeneration associated with contraction band necrosis
• exhibit contraction band necrosis
• exhibit age-dependent cardiac damage

homeostasis/metabolism
• there is a 25% reduction in fasting glucose levels
• fasting insulin leves tended to be higher regardless of diet
• over 60% increase in serum cholesterol levels
• 50% increase in nonesterifed fatty acids in serum of mice fed a zero fat diet

integument
N
• no epidermal phenotype is apparent; epidermal layer and surface lipid distribution appear normal

cellular
• exhibit contraction band necrosis




Genotype
MGI:6273162
hm4
Allelic
Composition
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
involves: 129S4/SvJae * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
N
• mice exhibit normal kidney histology




Genotype
MGI:4429479
hm5
Allelic
Composition
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
involves: 129S4/SvJae * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• in a hyperinsulinemic-euglycemic clamp study, peripheral glucose disposal in mice fed a high fat diet is improved compared to in similarly treated wild-type mice
• when fed a high fat diet compared with similarly treated wild-type mice
• when fed a high fat diet compared with similarly treated wild-type mice
• in the gastrocnemius when fed a high fat diet

muscle
• when fed a high fat diet, muscle glucose uptake is improved compared with similarly treated wild-type mice
• in the gastrocnemius when fed a high fat diet

growth/size/body

cellular
• when fed a high fat diet, muscle glucose uptake is improved compared with similarly treated wild-type mice




Genotype
MGI:3037489
hm6
Allelic
Composition
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
involves: 129S4/SvJae * C57BL/6N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• homozygotes do not develop liver enlargement following treatment with peroxisome proliferators

mortality/aging
• 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

cardiovascular system
• 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

homeostasis/metabolism
• male null mice develop severe hypoglycemia in response to CPT I inhibition
• estradiol rescues male null mice from the CPT I-induced death and impairment in lipid and glucose homeostasis
• in male mutants compared to female mutants inhibition of mitochondrial long chain fatty acid transport produces a marked decrease in hepatic glycogen levels
• wound healing is delayed during the first 4 days post wounding
• recruitment of neutrophils and monocytes is impaired in mutants on day 1 post wounding

liver/biliary system
• homozygotes do not develop liver enlargement following treatment with peroxisome proliferators
• in male mutants compared to female mutants inhibition of mitochondrial long chain fatty acid transport produces a marked decrease in hepatic glycogen levels
• the number of liver peroxisomes does not increase after treatment with peroxisome proliferators
• 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)

muscle
• 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

integument
• 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




Genotype
MGI:3037531
hm7
Allelic
Composition
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
integument
• foci of keratinocytes with nuclei are found in the stratum corneum
• mutants do not respond to peroxisome proliferators with thinning of the epidermis
• fewer keratohylin granules within the granular cells
• the granular layer is thinner

cellular
• foci of keratinocytes with nuclei are found in the stratum corneum
• mutants do not respond to peroxisome proliferators with thinning of the epidermis




Genotype
MGI:6317342
cx8
Allelic
Composition
Pkd1tm1.1Pcha/Pkd1tm1.1Pcha
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
involves: 129 * 129S4/SvJae * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm1.1Pcha mutation (0 available); any Pkd1 mutation (104 available)
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• all mice exhibit numerous kidney cysts
• kidney-weight to body-weight ratio is increased

renal/urinary system
• cyst epithelial proliferation is increased
• all mice exhibit numerous kidney cysts
• kidney-weight to body-weight ratio is increased

cellular
• cyst epithelial proliferation is increased




Genotype
MGI:4367223
cx9
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (64 available)
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
N
• fasting glucose levels and insulin levels are not increased after dexamethasone treatment

growth/size/body

adipose tissue
• after dexamethasone treatment




Genotype
MGI:4429480
cx10
Allelic
Composition
Pparatm1Gonz/Pparatm1Gonz
Tg(Ckm-Ppard)LEDpk/0
Genetic
Background
involves: 129S4/SvJae * C57BL/6 * C57BL/6J * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pparatm1Gonz mutation (5 available); any Ppara mutation (31 available)
Tg(Ckm-Ppard)LEDpk mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• when fed a high fat diet, mice exhibit greater glucose excursion compared with similarly treated Pparatm1Gonz homozygotes but improved glucose tolerance compared with similarly treated wild-type mice





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last database update
01/17/2023
MGI 6.22
The Jackson Laboratory