Why the study?
How do dystrophin and MLP gene mutations affect left-ventricular morphology, function, and cytoskeletal protein accumulation in mice?
Population
2- to 3-month old dystrophin-deficient (mdx) and MLP-null mice, and their respective controls
Comparison
Dystrophin deficiency or muscle LIM protein… vs Respective wild-type controls
Design
Preclinical
Authors
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MLP-null phenotypes caution against direct extrapolation to patients; leaves open MLP's compensatory role in human dystrophinopathies.
How do dystrophin and MLP gene mutations affect left-ventricular morphology, function, and cytoskeletal protein accumulation in mice?
MLP-null mice develop dilated cardiomyopathy and heart failure despite compensatory cytoskeletal protein accumulation, whereas mdx mice maintain normal LV function due to successful compensation by MLP and utrophin.
Wilding et al. (2004) studied this question.
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