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Pubmed ID :12507422
Publication Date : //

The cardiac mechanical stretch sensor machinery involves a Z disc complex that is defective in a subset of human dilated cardiomyopathy.


Muscle cells respond to mechanical stretch stimuli by triggering downstream signals for myocyte growth and survival. The molecular components of the muscle stretch sensor are unknown, and their role in muscle disease is unclear. Here, we present biophysical/biochemical studies in muscle LIM protein (MLP) deficient cardiac muscle that support a selective role for this Z disc protein in mechanical stretch sensing. MLP interacts with and colocalizes with telethonin (T-cap), a titin interacting protein. Further, a human MLP mutation (W4R) associated with dilated cardiomyopathy (DCM) results in a marked defect in T-cap interaction/localization. We propose that a Z disc MLP/T-cap complex is a key component of the in vivo cardiomyocyte stretch sensor machinery, and that defects in the complex can lead to human DCM and associated heart failure.

Authors : Knöll Ralph , Hoshijima Masahiko , Hoffman Hal M , Person Veronika , Lorenzen-Schmidt Ilka , Bang Marie-Louise , Hayashi Takeharu , Shiga Nobuyuki , Yasukawa Hideo , Schaper Wolfgang , McKenna William , Yokoyama Mitsuhiro , Schork Nicholas J , Omens Jeffrey H , McCulloch Andrew D , Kimura Akinori , Gregorio Carol C , Poller Wolfgang , Schaper Jutta , Schultheiss Heinz P , Chien Kenneth R ,

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