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Phenotypes Associated with This Genotype
Genotype
MGI:6511077
Allelic
Composition
Nexnem1Chen/Nexnem1Chen
Genetic
Background
C57BL/6J-Nexnem1Chen
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nexnem1Chen mutation (0 available); any Nexn mutation (28 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• approximately 20% of mice exhibit preweaning mortality

cardiovascular system
• P10 cardiomyocytes show disorganization of the entire transverse-axial tubule system, with the T-tubule component decreased and the longitudinal component increased
• mice have a higher number of cardiomyocytes missing the transverse component of transverse-axial tubule system, suggesting delayed T-tubule network formation
• hearts show slightly enlarged left ventricle at P10
• mice exhibit progressive dilated cardiomyopathy
• hearts show a progressive chamber dilation that is visible at 3 months of age
• hearts show reduced percentage of fractional shortening at P10 and 3 months of age
• echocardiography shows increased left ventricular dimensions and reduced cardiac function at P10 and 3 months of age
• however, no alterations in thickness of left ventricular posterior wall end diastole, calculated ratio of left ventricular mass to body weight, or thickness of interventricular septal end diastole are seen

muscle
• P10 cardiomyocytes show disorganization of the entire transverse-axial tubule system, with the T-tubule component decreased and the longitudinal component increased
• mice have a higher number of cardiomyocytes missing the transverse component of transverse-axial tubule system, suggesting delayed T-tubule network formation
• mice exhibit progressive dilated cardiomyopathy
• hearts show a progressive chamber dilation that is visible at 3 months of age
• hearts show reduced percentage of fractional shortening at P10 and 3 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
dilated cardiomyopathy 1CC DOID:0110424 OMIM:613122
J:301807


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
10/29/2024
MGI 6.24
The Jackson Laboratory