Chapter

Endplate Acetylcholine Receptor, a Target for Myasthenic Disorders: Structural and Mechanistic Essentials

Steven M. Sine and Andrew G. Engel

in Myasthenia Gravis and Myasthenic Disorders

Second edition

Published on behalf of © 2012 by Mayo Foundation for Medical Education and Research

Published in print March 2012 | ISBN: 9780199738670
Published online April 2013 | e-ISBN: 9780199322848 | DOI: http://dx.doi.org/10.1093/med/9780199738670.003.0009

Series: Contemporary Neurology Series

Endplate Acetylcholine Receptor, a Target for Myasthenic Disorders: Structural and Mechanistic Essentials

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The acetylcholine receptor (AChR) at the motor endplate is a frequent target for myasthenic disorders. Understanding both the structure and mechanism of action of the AChR is therefore essential to understanding disease mechanisms and developing rational therapy. Toward this end, this chapter develops present-day concepts of AChR structure and mechanism through a historical account of key advances over the past half century. Our understanding of AChR structure has advanced from a low-resolution profile of the receptor oligomer and its overall chemical composition to a near atomic resolution image of the majority of the protein backbone and location of more than 80% of the residue side chains. Mechanistic understanding has advanced from relatively simple state diagrams based on measurements of currents through AChR ensembles to more complex state diagrams based on measurements of currents through single AChR channels. The emerging structural and mechanistic concepts are then used to understand structural and mechanistic bases of several exemplar cases of congenital myasthenic syndromes (CMS).

Chapter.  13355 words.  Illustrated.

Subjects: Neurology

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