Everything about Nebulin totally explained
Nebulin is an
actin-binding protein which is localized to the I-band the
sarcomeres in
skeletal muscle. It is a very large protein (600-900 kDa) and binds as many as 200 actin monomers. Because its length is proportional to
thin filament length, it's believed that nebulin acts as a thin filament "ruler" and regulates thin filament length during
sarcomere assembly. Other functions of nebulin, such as a role in
cell signaling, remain uncertain.
The interaction of nebulin with actin is
calcium-
calmodulin sensitive.
Mutations in nebulin cause some cases of the
autosomal recessive disorder
nemaline myopathy.
A second smaller
isoform of nebulin, termed
nebulette, is expressed in
cardiac muscle.
Knockout phenotype
As of 2007, two
knockout mouse models for nebulin have been developed to better understand its
in vivo function. Bang and colleagues demonstrated that nebulin-knockout mice die postnatally, have reduced thin filament length, and impaired
contractile function. Postnatal
sarcomere disorganization and degeneration occurred rapidly in these mice, indicating the nebulin is essential for maintaining the structural integrity of
myofibrils. Witt and colleagues had similar results in their mice, which also died postnatally with reduced thin filament length and contractile function. These nebulin-knockout mice are being investigated as
animal models of
nemaline myopathy.
Further Information
Get more info on 'Nebulin'.
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