Cell membrane repair is a critical process used to maintain cell integrity and survival from
potentially lethal chemical, and mechanical membrane injury. Rapid increases in local …

Recombinant adeno-associated virus (rAAV) is currently the best vector for gene delivery
into the skeletal muscle. However, the 5-kb packaging size of this virus is a major obstacle …

Dysferlin is mutated in a group of muscular dystrophies commonly referred to as
dysferlinopathies. It is highly expressed in skeletal muscle, where it is important for …

Objective—Ferlins are known to regulate plasma membrane repair in muscle cells and are
linked to muscular dystrophy and cardiomyopathy. Recently, using proteomic analysis of …

Dysferlinopathy is a progressive myopathy caused by mutations in the dysferlin (DYSF)
gene. Dysferlin protein plays a major role in plasma-membrane resealing. Some patients …

Myoblast fusion contributes to muscle growth in development and during regeneration of
mature muscle. Myoblasts fuse to each other as well as to multinucleate myotubes to …

Dysferlin is an integral membrane protein of the transverse tubules of skeletal muscle that is
mutated or absent in limb girdle muscular dystrophy 2B and Miyoshi myopathy. Here we …

Abstract Limb Girdle Muscular Dystrophies type 2I (LGMD2I), a recessive autosomal
muscular dystrophy, is caused by mutations in the Fukutin Related Protein (FKRP) gene. It …

Xenografts of human skeletal muscle generated in mice can be used to study muscle
pathology and to test drugs designed to treat myopathies and muscular dystrophies for their …

Background Mutations in the genes coding for either dystrophin or dysferlin cause distinct
forms of muscular dystrophy. Dystrophin links the cytoskeleton to the sarcolemma through …