Guidelines for pre-clinical animal and cellular models of MuSK-myasthenia gravis.

Guidelines for pre-clinical animal and cellular models of MuSK-myasthenia gravis. Exp Neurol. 2014 Dec 24; Authors: Phillips WD, Christadoss P, Losen M, Punga A, Shigemoto K, Verschuuren J, Vincent A Abstract Muscle-specific tyrosine kinase (MuSK) autoantibodies are the hallmark of a form of myasthenia gravis (MG) that can challenge the neurologist and the experimentalist. The clinical disease can be difficult to treat effectively. MuSK autoantibodies affect the neuromuscular junction in several ways. When added to muscle cells in culture, MuSK antibodies disperse acetylcholine receptor clusters. Experimental animals actively immunized with MuSK develop MuSK autoantibodies and muscle weakness. Weakness is associated with reduced postsynaptic acetylcholine receptor numbers, reduced amplitudes of miniature endplate potentials and endplate potentials, and failure of neuromuscular transmission. Similar impairments have been found in mice injected with IgG from MG patients positive for MuSK autoantibody (MuSK-MG). The active and passive models have begun to reveal the mechanisms by which MuSK antibodies disrupt synaptic function at the neuromuscular junction, and should be valuable in developing therapies for MuSK-MG. However, translation into new and improved treatments for patients requires procedures that are not too cumbersome but suitable for examining different aspects of MuSK function and the effects of potential therapies. Study d...
Source: Experimental Neurology - Category: Neurology Authors: Tags: Exp Neurol Source Type: research