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Telethonin and Other New Proteins of the Z‐Disc of Skeletal Muscle

Authors: FAULKNER G.; LANFRANCHI, GEROLAMO; VALLE, GIORGIO;

Telethonin and Other New Proteins of the Z‐Disc of Skeletal Muscle

Abstract

AbstractThis brief review outlines some of the most relevant proteins of the Z‐disc and the complex network of interactions that link them together in a stable structure. Apart from the well‐known Z‐disc proteins such as actin, cap‐Z, titin, nebulin, and α‐actinin 2, several other Z‐disc proteins have been recently discovered, including telethonin and myotilin that have been linked to limb girdle muscular dystrophies. Some proteins including ALP and ZASP have known interaction domains (PDZ and LIM motifs), whereas others like FATZ have no canonical interaction domains, although they are known to bind several proteins. Another new Z‐disc protein is γ‐filamin that could provide a link between the plasma membrane and myofibrils because it binds directly to γ‐ and δ‐sarcoglycans and indirectly to α‐actinin 2 via FATZ and myotilin. A greater knowledge of Z‐disc proteins and their interactions is essential for understanding their role in the structure and function of muscle.

Keywords

Models, Molecular, Macromolecular Substances, Mutation, Animals, Humans, Muscle Proteins, Connectin, Cardiomyopathies, Muscle, Skeletal, Muscular Dystrophies

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
96
Top 10%
Top 10%
Top 10%
bronze