TY - JOUR
T1 - Role of TGF-β signaling in inherited and acquired myopathies
AU - Burks, Tyesha N.
AU - Cohn, Ronald D.
N1 - Funding Information:
This project was funded by the National Institute on Aging, Claude D. Pepper Older Americans Independence Center, parent grant P30AG021334 to RDC and supplemental grant P30AG021334-08S1 to TNB. RDC is also supported by the NIH Director’s New Innovator Award DP2 OD004515, by NIH 5K08NS055879 award, and MDA #101938.
PY - 2011/5/4
Y1 - 2011/5/4
N2 - The transforming growth factor-beta (TGF-β) superfamily consists of a variety of cytokines expressed in many different cell types including skeletal muscle. Members of this superfamily that are of particular importance in skeletal muscle are TGF-β1, mitogen-activated protein kinases (MAPKs), and myostatin. These signaling molecules play important roles in skeletal muscle homeostasis and in a variety of inherited and acquired neuromuscular disorders. Expression of these molecules is linked to normal processes in skeletal muscle such as growth, differentiation, regeneration, and stress response. However, chronic elevation of TGF-β1, MAPKs, and myostatin is linked to various features of muscle pathology, including impaired regeneration and atrophy. In this review, we focus on the aberrant signaling of TGF-β in various disorders such as Marfan syndrome, muscular dystrophies, sarcopenia, and critical illness myopathy. We also discuss how the inhibition of several members of the TGF-β signaling pathway has been implicated in ameliorating disease phenotypes, opening up novel therapeutic avenues for a large group of neuromuscular disorders.
AB - The transforming growth factor-beta (TGF-β) superfamily consists of a variety of cytokines expressed in many different cell types including skeletal muscle. Members of this superfamily that are of particular importance in skeletal muscle are TGF-β1, mitogen-activated protein kinases (MAPKs), and myostatin. These signaling molecules play important roles in skeletal muscle homeostasis and in a variety of inherited and acquired neuromuscular disorders. Expression of these molecules is linked to normal processes in skeletal muscle such as growth, differentiation, regeneration, and stress response. However, chronic elevation of TGF-β1, MAPKs, and myostatin is linked to various features of muscle pathology, including impaired regeneration and atrophy. In this review, we focus on the aberrant signaling of TGF-β in various disorders such as Marfan syndrome, muscular dystrophies, sarcopenia, and critical illness myopathy. We also discuss how the inhibition of several members of the TGF-β signaling pathway has been implicated in ameliorating disease phenotypes, opening up novel therapeutic avenues for a large group of neuromuscular disorders.
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U2 - 10.1186/2044-5040-1-19
DO - 10.1186/2044-5040-1-19
M3 - Review article
C2 - 21798096
AN - SCOPUS:84857460345
SN - 2044-5040
VL - 1
JO - Skeletal Muscle
JF - Skeletal Muscle
IS - 1
M1 - 19
ER -