Congenital myasthenic syndromes: II. Syndrome attributed to abnormal interaction of acetylcholine with its receptor (original) (raw)

Congenital myasthenic syndromes: I. Deficiency and short open-time of the acetylcholine receptor

Andrew Engel

Muscle & Nerve, 1993

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Newly recognized congenital myasthenic syndrome associated with high conductance and fast closure of the acetylcholine receptor channel

John Bodensteiner, Andrew Engel

Annals of Neurology, 1993

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Myasthenic Syndromes Attributed to Mutations Affecting the Epsilon Subunit of the Acetylcholine Receptor

Andrew Engel

Annals of the New York Academy of Sciences, 1993

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Electrophysiological study in synaptic congenital myasthenic syndrome: end-plate acetylcholinesterase deficiency

Lineu Werneck

Arquivos de Neuro-Psiquiatria, 2009

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Congenital myasthenic syndromes: genetic defects of the neuromuscular junction

Andrew Engel

Current neurology and neuroscience reports, 2002

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Mutation of the acetylcholine receptor α subunit causes a slow-channel myasthenic syndrome by enhancing agonist binding affinity

Andrew Engel

Neuron, 1995

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Congenital myasthenic syndrome caused by prolonged acetylcholine receptor channel openings due to a mutation in the M2 domain of the epsilon subunit

Steven m. M Sine

Proceedings of the National Academy of Sciences, 1995

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New mutations in acetylcholine receptor subunit genes reveal heterogeneity in the slow-channel congenital myasthenic syndrome

Nina Bren

Human Molecular Genetics, 1996

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Congenital Myasthenic Syndrome Caused by Decreased Agonist Binding Affinity Due to a Mutation in the Acetylcholine Receptor ε Subunit

Nina Bren

Neuron, 1996

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Slow-Channel Congenital Myasthenic Syndrome due to a Novel Mutation in the Acetylcholine Receptor Alpha Subunit in a South Asian: A Case Report

Sunethra Senanayake

Journal of neuromuscular diseases, 2021

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Congenital myasthenic syndromes: Experiments of nature

Andrew Engel

Journal of Physiology-Paris, 1998

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Slow-Channel Myasthenic Syndrome Caused By Enhanced Activation, Desensitization, and Agonist Binding Affinity Attributable to Mutation in the M2 Domain of the Acetylcholine Receptor α Subunit

Steven m. M Sine

The Journal of Neuroscience, 1997

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The Electrophysiology of Presynaptic Congenital Myasthenic Syndromes With and Without Facilitation: From Electrodiagnostic Findings to Molecular Mechanisms

Stefan Nicolau

Frontiers in Neurology

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Slow-Channel Myasthenic Syndrome Caused By Enhanced Activation, Desensitization, and Agonist Binding Affinity Attributable to Mutation in the M2 Domain of the Acetylcholine Receptor a Subunit

Nina Bren

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Congenital Myasthenic Syndromes due to Heteroallelic Nonsense/Missense Mutations in the Acetylcholine Receptor Subunit Gene: Identification and Functional Characterization of Six New Mutations

Andrew Engel

Human Molecular Genetics, 1997

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The Spectrum of Congenital Myasthenic Syndromes

Andrew Engel

Molecular Neurobiology, 2002

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Electrophysiological and morphological characterization of a case of autosomal recessive congenital myasthenic syndrome with acetylcholine receptor deficiency due to a N88K rapsyn homozygous mutation

Daniel Hantai

Neuromuscular Disorders, 2004

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Congenital myasthenic syndromes: Progress over the past decade

Andrew Engel

Muscle & Nerve, 2003

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Congenital Myasthenic Syndrome Associated with Paucity of Synaptic Vesicles and Reduced Quantal Release

Andrew Engel

Annals of the New York Academy of Sciences, 1993

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The therapy of congenital myasthenic syndromes

Andrew Engel

Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics, 2007

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Abnormal neuromuscular transmission in an infantile myasthenic syndrome

Katerina Dorovini-zis

Annals of Neurology, 1984

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A novel c.973G>T mutation in the ε-subunit of the acetylcholine receptor causing congenital myasthenic syndrome in an iranian family

Parvaneh Karimzadeh

Balkan Journal of Medical Genetics, 2019

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Congenital myasthenic syndrome caused by decreased receptor channel openings due to a novel mutation in the nAChR ε-subunit

Daniel Hantaï

Journal of Physiology-Paris, 2006

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Studies of human myasthenia gravis: electrophysiological and ultrastructural evidence compatible with antibody attachment to acetylcholine receptor complex

John Rash

Proceedings of the National Academy of Sciences, 1976

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What Have We Learned from the Congenital Myasthenic Syndromes

Andrew Engel

Journal of Molecular Neuroscience, 2010

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Current understanding of congenital myasthenic syndromes

Andrew Engel

Current Opinion in Pharmacology, 2005

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Mutations in Different Functional Domains of the Human Muscle Acetylcholine Receptor Subunit in Patients with the Slow-Channel congenital Myasthenic Syndrome

David Beeson

Human Molecular Genetics, 1997

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Repetitive compound muscle action potentials in electrophysiological diagnosis of congenital myasthenic syndromes: A case report and review of literature

Abraham Kuruvilla

Annals of Indian Academy of Neurology, 2010

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