Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Destm1Cap mutation
(0 available);
any
Des mutation
(34 available)
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Cardiac fibrosis and calcification seen in Destm1Cap/Destm1Cap mice is reduced in Destm1Cap/Destm1Cap Tg(Bcl2)ACap/Tg(Bcl2)ACap and Destm1Cap/Destm1Cap Tg(Bcl2)BCap/Tg(Bcl2)BCap mice
cardiovascular system
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• cardiomyocytes show disrupted contractile apparatus and numerous mitochondrial abnormalities, including loss of positioning, proliferation, clumping, and formation of cleared areas around mitochondria, and individual mitochondria appear swollen and misshapen
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• cardiac hypertrophy occurs in response to cardiac damage, with significant increase in heart size and distortion of the ventricles
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• fibrosis associated with calcium deposits is found throughout the left ventricle
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• impaired systolic function, showing decreased peak aortic velocity and mean acceleration
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• cardiomyocyte mitochondria exhibit increased sensitivity to calcium induced swelling
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muscle
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• cardiomyocytes show disrupted contractile apparatus and numerous mitochondrial abnormalities, including loss of positioning, proliferation, clumping, and formation of cleared areas around mitochondria, and individual mitochondria appear swollen and misshapen
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• impaired systolic function, showing decreased peak aortic velocity and mean acceleration
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growth/size/body
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• cardiac hypertrophy occurs in response to cardiac damage, with significant increase in heart size and distortion of the ventricles
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Allelic Composition |
Destm1Cap/Destm1Cap
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Genetic Background |
involves: 129S7/SvEvBrd * C57BL/6 |
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Destm1Cap mutation
(0 available);
any
Des mutation
(34 available)
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Myocardium degeneration and calcification in Destm1Cap/Destm1Cap mice
mortality/aging
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• 6% of homozygotes die suddenly before reaching 1 year of age
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cardiovascular system
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• loss of myocardial fiber tension
(J:35123)
• interstitial fibrosis and necrosis
(J:35123)
• detachment of fibers from the sarcolemma
(J:35123)
• disorganized mitochondria
(J:35123)
• a 24% increase in average cell volume of ventricular myocytes at 7 months, with no changes in cell length or profile area
(J:59831)
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• loss of lateral alignment
(J:35123)
• areas of disorganized and ragged myocytes, as commonly observed in hypertrophic myocardium
(J:59831)
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• moderate to severe thinning of myocardial wall after sudden death
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• mild heart enlargement is frequently observed in the absence of stress
• severe heart enlargement is noted after sudden death or after exercise-induced stress
• prominent heart enlargement and ventricular dilation also observed in aging homozygotes
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• a 20% increase in HW/BW ratio by 1 month of age or later
• concentric cellular hypertrophy of ventricular myocytes, with normal major diameters but a 30% increase in minor diameters at 7 months
• changes in expression pattern of hypertrophic markers associated with pressure overload hypertrophy at 1 and 6 months
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• slight dilation of left ventricle in the absence of stress, with no severe changes in myocardial wall thickness
• severe dilation of the left ventricle after sudden death, with moderate to severe thinning of myocardial wall
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• slight dilation of right ventricle in the absence of stress, with no severe changes in myocardial wall thickness
• severe dilation of the right ventricle after sudden death, with moderate to severe thinning of myocardial wall
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• progressive calcification and degeneration, particularly of the left ventricle, evident by 3 weeks of age
(J:35123)
• calcified lesions and areas of fibrous replacement of myocardial tissue as early as 1 week after birth; most prominent in the right ventricle and toward the inner and outer surfaces of the myocardium
(J:59831)
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• significantly impaired systolic function (reduced peak aortic flow velocity and average aortic acceleration) at 13-14 months of age, with a 20% reduction in peak aortic flow velocity evident at 3-13 months or later
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• enhanced diastolic function at 3 months of age (increased ratios of E/A peak velocities and areas), no longer evident after 13 months
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• progressive calcification and degeneration, particularly of the left ventricle, evident by 3 weeks of age
• lesions appeared to progress from the exterior of the myocardium inward
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muscle
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• loss of myocardial fiber tension
(J:35123)
• interstitial fibrosis and necrosis
(J:35123)
• detachment of fibers from the sarcolemma
(J:35123)
• disorganized mitochondria
(J:35123)
• a 24% increase in average cell volume of ventricular myocytes at 7 months, with no changes in cell length or profile area
(J:59831)
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• loss of lateral alignment
(J:35123)
• areas of disorganized and ragged myocytes, as commonly observed in hypertrophic myocardium
(J:59831)
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• moderate to severe thinning of myocardial wall after sudden death
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• significantly impaired systolic function (reduced peak aortic flow velocity and average aortic acceleration) at 13-14 months of age, with a 20% reduction in peak aortic flow velocity evident at 3-13 months or later
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• enhanced diastolic function at 3 months of age (increased ratios of E/A peak velocities and areas), no longer evident after 13 months
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• progressive calcification and degeneration, particularly of the left ventricle, evident by 3 weeks of age
• lesions appeared to progress from the exterior of the myocardium inward
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• similar abnormalities as observed in cardiac muscle, but to a lesser extent
• ragged, disorganized fibers with a loss of tension
• disorganized mitochondria
• increased severity of myofibril defects in muscles with high usage (e.g. tongue muscle)
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• defects similar to those observed in striated muscle, but less severe
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• degeneration of striated muscle
• more severe in cardiac muscle
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behavior/neurological
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• noted by 2 weeks of age
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• 50% of homozygotes show impaired excercise capacity and die during swimming
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homeostasis/metabolism
growth/size/body
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• mild heart enlargement is frequently observed in the absence of stress
• severe heart enlargement is noted after sudden death or after exercise-induced stress
• prominent heart enlargement and ventricular dilation also observed in aging homozygotes
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• a 20% increase in HW/BW ratio by 1 month of age or later
• concentric cellular hypertrophy of ventricular myocytes, with normal major diameters but a 30% increase in minor diameters at 7 months
• changes in expression pattern of hypertrophic markers associated with pressure overload hypertrophy at 1 and 6 months
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cellular
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Destm1Cap mutation
(0 available);
any
Des mutation
(34 available)
Tg(Bcl2)ACap mutation
(0 available)
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Cardiac fibrosis and calcification seen in Destm1Cap/Destm1Cap mice is reduced in Destm1Cap/Destm1Cap Tg(Bcl2)ACap/Tg(Bcl2)ACap and Destm1Cap/Destm1Cap Tg(Bcl2)BCap/Tg(Bcl2)BCap mice
cardiovascular system
N |
• the progressive calcification and degeneration observed in Destm1Cap homozygotes is ameliorated
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• some fibrosis is observed, althought there are fewer and smaller fibrotic lesions than in Destm1Cap homozygotes
• more fibrosis than with similar mice carrying the Tg(Bcl2)BCap, which overexpresses higher levels of BCL2
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• some calcification is observed, although much less than in Destm1Cap homozygotes
• more calcification than with similar mice carrying the Tg(Bcl2)BCap, which overexpresses higher levels of BCL2
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cellular
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• some fibrosis is observed, althought there are fewer and smaller fibrotic lesions than in Destm1Cap homozygotes
• more fibrosis than with similar mice carrying the Tg(Bcl2)BCap, which overexpresses higher levels of BCL2
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Destm1Cap mutation
(0 available);
any
Des mutation
(34 available)
Tg(Bcl2)BCap mutation
(0 available)
|
|
|
Cardiac fibrosis and calcification seen in Destm1Cap/Destm1Cap mice is reduced in Destm1Cap/Destm1Cap Tg(Bcl2)ACap/Tg(Bcl2)ACap and Destm1Cap/Destm1Cap Tg(Bcl2)BCap/Tg(Bcl2)BCap mice
cardiovascular system
N |
• the progressive calcification and degeneration observed in Destm1Cap homozygotes is ameliorated
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• although cadiomyocytes show a significant improvement from the damage seen in Destm1Cap homozygotes, some cardiomyocytes exhibit a loss of mitochondria alignment
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• some fibrosis is observed, although there are fewer and smaller fibrotic lesions than in both Destm1Cap homozygotes and Destm1Cap homozygotes carrying Tg(Bcl2)ACap
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• some calcification is observed, althogh less than in both Destm1Cap homozygotes and Destm1Cap homozygotes carrying Tg(Bcl2)ACap
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muscle
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• although cadiomyocytes show a significant improvement from the damage seen in Destm1Cap homozygotes, some cardiomyocytes exhibit a loss of mitochondria alignment
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cellular
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• some fibrosis is observed, although there are fewer and smaller fibrotic lesions than in both Destm1Cap homozygotes and Destm1Cap homozygotes carrying Tg(Bcl2)ACap
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