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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(DM15)26Bew
transgene insertion 26, Be Wieringa
MGI:3054417
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(DM15)26Bew/0 involves: FVB MGI:3054527


Genotype
MGI:3054527
tg1
Allelic
Composition
Tg(DM15)26Bew/0
Genetic
Background
involves: FVB
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phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• significant neonatal lethality was seen and no homozygous pups were ever produced
• occasional lethality is seen in pregnant female mutants
• this phenotype became less evident in later generations (F3 and later) when male carriers were used

behavior/neurological
• older mutants (11-15 months of age) perform at a significantly reduced level under stress testing, with tolerated workload 3-fold lower than in controls (J:93614)
• females display reduced food intake during and after pregnancy (J:33711)
• this phenotype became less evident in later generations (F3 and later) when male carriers were used (J:33711)
• older mice exhibit sporadic yet dramatic transient episodes of walking difficulties
• females develop a hunched posture during and after pregnancy
• this phenotype became less evident in later generations (F3 and later) when male carriers were used

cardiovascular system
• local whorling interrupts the regular pattern of the myocardium (J:33711)
• increased collagen is seen in the interstitium (J:33711)
• older mice display myocyte disarray, with subcellular degenerative changes, including deformity, multiple vaculolization and mitochondria with abnormal cristae (J:93614)
• interventricular spetal thickness is increased in older mutants
• seen in old mutants; left ventricular mass is doubled
• posterior wall is thicker in older mutants
• seen in older mutants
• older mutants exhibit ventricular tachyarrhythmias
• junctional rhythm occurs with significant greater frequency in older mutants under light sedation
• P wave occurs with significantly greater frequency in older mutants under light sedation
• older mice display depressed systemic systolic and diastolic arterial pressure
• obvious cardiomyopathy is seen at 7, 9, or 21 weeks of age (J:33711)
• hypertrophic maladaptive cardiomyopathy with dysrhythmia in older mutants, recapitulating typical features of myotonic dystrophy (J:93614)
• hypertrophic cardiomyopathy is characterized by overt intracellular calcium overload (J:93614)

muscle
• local whorling interrupts the regular pattern of the myocardium (J:33711)
• increased collagen is seen in the interstitium (J:33711)
• older mice display myocyte disarray, with subcellular degenerative changes, including deformity, multiple vaculolization and mitochondria with abnormal cristae (J:93614)
• obvious cardiomyopathy is seen at 7, 9, or 21 weeks of age (J:33711)
• hypertrophic maladaptive cardiomyopathy with dysrhythmia in older mutants, recapitulating typical features of myotonic dystrophy (J:93614)
• hypertrophic cardiomyopathy is characterized by overt intracellular calcium overload (J:93614)
• skeletal muscle of older mice demonstrates central nuclei, ringed fibers, ragged and torn fibers, hypotrophy with sarcomeric disorganization and sarcoplasmic masses, and accumulation of mitochondria
• skeletal muscle of older mice exhibits a broad spectrum of fiber sizes
• skeletal muscle of older mice demonstrates central nuclei
• myotonic dystrophy in older mutants
• older mutants exhibit prolonged myotonic discharges characterized by high frequency repetitive potentials in distal limb muscles mechanically stimulated with needle insertion
• skeletal muscles of older mutants show myopathy including central nuclei, ringed fibers, ragged and torn fibers, broad spectrum of fiber sizes, hypotrophy with sarcomeric disorganization and sarcoplasmic masses, and accumulation of mitochondria

integument
• females display bristling hair during and after pregnancy
• this phenotype became less evident in later generations (F3 and later) when male carriers were used

Mouse Models of Human Disease
OMIM ID Ref(s)
Myotonic Dystrophy 1; DM1 160900 J:93614





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last database update
06/22/2016
MGI 6.04
The Jackson Laboratory