Journal article

Effects of the beta 2-Adrenoceptor Agonist, Albuterol, in a Mouse Model of Anti-MuSK Myasthenia Gravis

Nazanin Ghazanfari, Marco Morsch, Nigel Tse, Stephen W Reddel, William D Phillips

PLOS ONE | PUBLIC LIBRARY SCIENCE | Published : 2014

Abstract

The β2-adrenergic receptor agonist, albuterol, has been reported beneficial in treating several forms of congenital myasthenia. Here, for the first time, we examined the potential benefit of albuterol in a mouse model of anti-Muscle Specific Kinase (MuSK) myasthenia gravis. Mice received 15 daily injections of IgG from anti-MuSK positive patients, which resulted in whole-body weakness. At neuromuscular junctions in the tibialis anterior and diaphragm muscles the autoantibodies caused loss of postsynaptic acetylcholine receptors, and reduced the amplitudes of the endplate potential and spontaneous miniature endplate potential in the diaphragm muscle. Treatment with albuterol (8 mg/kg/day) dur..

View full abstract

Grants

Awarded by Australian National Health and Medical Research Council


Funding Acknowledgements

The work presented was supported by a project grant from the Australian National Health and Medical Research Council (http://www.nhmrc.gov.au/; number 570930 to W. D. P & S. W. R.) and by the Brain Foundation, the Beeren Foundation and the Australian Myasthenic Association in New South Wales. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.