Mouse Genome Informatics
hm
    Csrp3tm1Crni/Csrp3tm1Crni
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• 50 to 70% of mice became fatigued between 5 and 10 days of age and died 20 to 30 hours after the onset of symptoms
• mice that did not during the second postnatal week survived to adulthood and displayed a different cardiac phenotype than those that died during the second postnatal
• the penetrance of death during the second postnatal week was higher in offspring from heterozygous crosses than in offspring of either homozygous or heterozygous/homozygous crosses indicating a genetic background effect

cardiovascular system
• pronounced convolution of the membrane
• myofibrils are somewhat disorganized
• increase in total heart size
• enlargement affected all 4 chambers equally and was predominantly observed mice that died during the second week of life
• increase in total heart weight
• observed in both mice that died during the second week of life and in mice that survived to adulthood
• observed in mice that survived to adulthood, but not in those that died during the second week of life
• disruption of cardiac myofibrillar organization observed (J:38213)
• disorganization of the actin cytoskeleton and myofibrillar apparatus was detected in newborns, prior to overt myopathy (J:38213)
• histologic and ultrastructural features similar to those observed in human dilated cardiomyopathy (J:38213)

muscle
• pronounced convolution of the membrane
• myofibrils are somewhat disorganized
• disruption of cardiac myofibrillar organization observed (J:38213)
• disorganization of the actin cytoskeleton and myofibrillar apparatus was detected in newborns, prior to overt myopathy (J:38213)
• histologic and ultrastructural features similar to those observed in human dilated cardiomyopathy (J:38213)

homeostasis/metabolism
• observed in mice that survived to adulthood, but not in those that died during the second week of life

Mouse Models of Human Disease
OMIM IDRef(s)
Cardiomyopathy, Dilated, 1M; CMD1M 607482 J:38213