Duchenne muscular dystrophy is a severe, progressive, muscle-wasting disease that leads
to difficulties with movement and, eventually, to the need for assisted ventilation and …

Decades of research in skeletal muscle physiology have provided multiscale insights into
the structural and functional complexity of this important anatomical tissue, designed to …

INTRODUCTION Loss-of-function mutations in one gene copy can lead to reduced amounts
of protein and, consequently, human disease, a condition termed haploinsufficiency. It is …

Skeletal muscle fibers are classified into fiber types, in particular, slow twitch versus fast
twitch. Muscle fiber types are generally defined by the particular myosin heavy chain …

Almost every muscle contains muscle spindles. These delicate sensory receptors inform the
central nervous system (CNS) about changes in the length of individual muscles and the …

Duchenne muscular dystrophy (DMD) is a common fatal heritable myopathy, with
cardiorespiratory failure occurring by the third decade of life. There is no specific treatment …

Increased utrophin expression is known to reduce pathology in dystrophin-deficient skeletal
muscles. Transgenic over-expression of PGC-1α has been shown to increase levels of …

Dystrophin and utrophin are highly similar proteins that both link cortical actin filaments with
a complex of sarcolemmal glycoproteins, yet localize to different subcellular domains within …

Background Duchenne muscular dystrophy (DMD) is a lethal, progressive muscle wasting
disease caused by a loss of sarcolemmal bound dystrophin, which results in the death of the …

The absence of dystrophin in Duchenne muscular dystrophy disrupts the dystrophin-
associated glycoprotein complex resulting in skeletal muscle fiber fragility and atrophy …