A ?-subunit mutation in the acetylcholine receptor channel gate causes severe slow-channel syndrome (original) (raw)

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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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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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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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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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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Slow-Channel Syndromes • review article • Decoding Pathogenesis of Slow-channel congenital Myasthenic Syndromes using recombinant expression and Mice Models

Emanuel Reyes

2010

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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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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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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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Acetylcholine receptor δ subunit mutations underlie a fast-channel myasthenic syndrome and arthrogryposis multiplex congenita

David Beeson

Journal of Clinical Investigation, 2001

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Serum choline activates mutant acetylcholine receptors that cause slow channel congenital myasthenic syndromes

Anthony Auerbach

Proceedings of the National Academy of Sciences, 1999

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

Andrew Engel

Muscle & Nerve, 1993

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Rare slow channel congenital myasthenic syndromes without repetitive compound muscle action potential and dramatic response to low dose fluoxetine

feza deymeer

Acta Neurologica Belgica, 2020

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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

Andrew Engel

Proceedings of the National Academy of Sciences, 1995

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Novel delta subunit mutation in slow-channel syndrome causes severe weakness by novel mechanisms

Legier Rojas

Annals of Neurology, 2002

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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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Decoding Pathogenesis of Slow-Channel Congenital Myasthenic Syndromes using Recombinant Expression and Mice Models

Carlos Luciano

Puerto Rico Health Sciences Journal, 2010

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Myasthenic syndrome AChRα C-loop mutant disrupts initiation of channel gating

Steven m. M Sine

Journal of Clinical Investigation, 2012

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Attributable to Mutation in the M 2 Domain of the Acetylcholine Receptor a Subunit

Steven m. M Sine

1997

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

Andrew Engel

Muscle & Nerve, 1993

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Mutations causing congenital myasthenia reveal principal coupling pathway in the acetylcholine receptor ε-subunit

Nur Yuceyar

JCI insight, 2018

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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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Congenital myasthenic syndromes: genetic defects of the neuromuscular junction

Andrew Engel

Current neurology and neuroscience reports, 2002

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Congenital Myasthenic Syndrome Caused by Novel Loss-of-Function Mutations in the Human AChR e Subunit Genea

Xin-ming Shen

Annals of the New York Academy of Sciences, 1998

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Myasthenic syndrome caused by mutation of the SCN4A sodium channel

Xin-ming Shen

Proceedings of the National Academy of Sciences, 2003

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Congenital myasthenic syndromes: Progress over the past decade

Andrew Engel

Muscle & Nerve, 2003

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