Richard L. Rotundo

TL;DR: The preservation of muscle integrity and function in MCK-PGC-1α animals resulted in significantly improved whole-body health; both the loss of bone mineral density and the increase of systemic chronic inflammation, observed during normal aging, were prevented.

TL;DR: The α-syn−/− mice showed no evidence of myopathy, despite reduced levels of α-dystrobrevin−2. as discussed by the authors showed that α-syntrophin null mice have shallow nerve gutters, abnormal distributions of acetylcholine receptors, and postjunctional folds that are generally less organized and have fewer openings to the synaptic cleft than controls.

TL;DR: It is shown that the collagenic-tailed form of the enzyme binds to the heparan-sulfate proteoglycan perlecan, which in turn binds toThe dystroglycan–perlecan complex serves as a cell surface acceptor for AChE, enabling it to be clustered at the synapse by lateral migration within the plane of the membrane.

TL;DR: It is shown that myotubes differentiated from dystroglycan−/− embryonic stem cells are responsive to agrin, but produce acetylcholine receptor (AChR) clusters which are two to three times larger in area, about half as dense, and significantly less stable than those on dystoglycan+/+ myot tubes.

TL;DR: Perlecan, a multifunctional heparan sulfate proteoglycan concentrated at the NMJ, is the unique acceptor molecule for collagen-tailed AChE at sites of nerve–muscle contact and is the principal mechanism for localizing A cholinesterase to the synaptic basal lamina.