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Telethonin and Other New Proteins of the Z‐Disc of Skeletal Muscle
Author(s) -
Faulkner Georgine,
Lanfranchi Gerolamo,
Valle Giorgio
Publication year - 2001
Publication title -
iubmb life
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.132
H-Index - 113
eISSN - 1521-6551
pISSN - 1521-6543
DOI - 10.1080/152165401317190761
Subject(s) - titin , filamin , nebulin , actin , pdz domain , microbiology and biotechnology , myofibril , biology , sarcomere , chemistry , biophysics , cytoskeleton , biochemistry , myocyte , cell
This 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.