PRE- AND POSTSYNAPTIC NEUROMUSCULAR JUNCTION ABNORMALITIES IN MuSK MYASTHENIA

Erik H. Niks; Jan B. M. Kuks; John H. J. Wokke; Henk Veldman; Egbert Bakker; Jan J. G. M. Verschuuren; Jaap J. Plomp

doi:10.1002/mus.21642

PRE- AND POSTSYNAPTIC NEUROMUSCULAR JUNCTION ABNORMALITIES IN MuSK MYASTHENIA

Erik H. Niks^*, Jan B. M. Kuks, John H. J. Wokke, Henk Veldman, Egbert Bakker, Jan J. G. M. Verschuuren, Jaap J. Plomp

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

55 Citations (Scopus)

Abstract

Autoantibodies to muscle-specific kinase (MuSK) can cause myasthenia gravis (MG). The pathophysiological mechanism remains unknown. We report in vitro electrophysiological and histological studies of the neuromuscular junction in a MuSK MG patient. Low levels of presynaptic acetylcholine release and small miniature endplate potentials were found. This combination of pre- and postsynaptic abnormalities was supported by histology, revealing partially denervated postsynaptic areas, and some degeneration of postsynaptic folds. Results suggest that anti-MuSK antibodies reduce the stability of muscle nerve contact. Muscle Nerve 42:283-288, 2010

Original language	English
Pages (from-to)	283-288
Number of pages	6
Journal	MUSCLE & NERVE
Volume	42
Issue number	2
DOIs	https://doi.org/10.1002/mus.21642
Publication status	Published - Aug-2010

Keywords

histology
in vitro electrophysiology
MuSK, myasthenia gravis
neuromuscular junction
quantal content
miniature end plate potential
MUSCLE-SPECIFIC KINASE
ACETYLCHOLINE-RECEPTOR ANTIBODIES
AMYOTROPHIC-LATERAL-SCLEROSIS
GRAVIS
AGRIN
LRP4
TRANSMISSION
RELEASE
COMPLEX
TID1

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title = "PRE- AND POSTSYNAPTIC NEUROMUSCULAR JUNCTION ABNORMALITIES IN MuSK MYASTHENIA",

abstract = "Autoantibodies to muscle-specific kinase (MuSK) can cause myasthenia gravis (MG). The pathophysiological mechanism remains unknown. We report in vitro electrophysiological and histological studies of the neuromuscular junction in a MuSK MG patient. Low levels of presynaptic acetylcholine release and small miniature endplate potentials were found. This combination of pre- and postsynaptic abnormalities was supported by histology, revealing partially denervated postsynaptic areas, and some degeneration of postsynaptic folds. Results suggest that anti-MuSK antibodies reduce the stability of muscle nerve contact. Muscle Nerve 42:283-288, 2010",

keywords = "histology, in vitro electrophysiology, MuSK, myasthenia gravis, neuromuscular junction, quantal content, miniature end plate potential, MUSCLE-SPECIFIC KINASE, ACETYLCHOLINE-RECEPTOR ANTIBODIES, AMYOTROPHIC-LATERAL-SCLEROSIS, GRAVIS, AGRIN, LRP4, TRANSMISSION, RELEASE, COMPLEX, TID1",

author = "Niks, {Erik H.} and Kuks, {Jan B. M.} and Wokke, {John H. J.} and Henk Veldman and Egbert Bakker and Verschuuren, {Jan J. G. M.} and Plomp, {Jaap J.}",

year = "2010",

month = aug,

doi = "10.1002/mus.21642",

language = "English",

volume = "42",

pages = "283--288",

journal = "MUSCLE & NERVE",

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T1 - PRE- AND POSTSYNAPTIC NEUROMUSCULAR JUNCTION ABNORMALITIES IN MuSK MYASTHENIA

AU - Niks, Erik H.

AU - Kuks, Jan B. M.

AU - Wokke, John H. J.

AU - Veldman, Henk

AU - Bakker, Egbert

AU - Verschuuren, Jan J. G. M.

AU - Plomp, Jaap J.

PY - 2010/8

Y1 - 2010/8

N2 - Autoantibodies to muscle-specific kinase (MuSK) can cause myasthenia gravis (MG). The pathophysiological mechanism remains unknown. We report in vitro electrophysiological and histological studies of the neuromuscular junction in a MuSK MG patient. Low levels of presynaptic acetylcholine release and small miniature endplate potentials were found. This combination of pre- and postsynaptic abnormalities was supported by histology, revealing partially denervated postsynaptic areas, and some degeneration of postsynaptic folds. Results suggest that anti-MuSK antibodies reduce the stability of muscle nerve contact. Muscle Nerve 42:283-288, 2010

AB - Autoantibodies to muscle-specific kinase (MuSK) can cause myasthenia gravis (MG). The pathophysiological mechanism remains unknown. We report in vitro electrophysiological and histological studies of the neuromuscular junction in a MuSK MG patient. Low levels of presynaptic acetylcholine release and small miniature endplate potentials were found. This combination of pre- and postsynaptic abnormalities was supported by histology, revealing partially denervated postsynaptic areas, and some degeneration of postsynaptic folds. Results suggest that anti-MuSK antibodies reduce the stability of muscle nerve contact. Muscle Nerve 42:283-288, 2010

KW - histology

KW - in vitro electrophysiology

KW - MuSK, myasthenia gravis

KW - neuromuscular junction

KW - quantal content

KW - miniature end plate potential

KW - MUSCLE-SPECIFIC KINASE

KW - ACETYLCHOLINE-RECEPTOR ANTIBODIES

KW - AMYOTROPHIC-LATERAL-SCLEROSIS

KW - GRAVIS

KW - AGRIN

KW - LRP4

KW - TRANSMISSION

KW - RELEASE

KW - COMPLEX

KW - TID1

U2 - 10.1002/mus.21642

DO - 10.1002/mus.21642

M3 - Article

SN - 0148-639X

VL - 42

SP - 283

EP - 288

JO - MUSCLE & NERVE

JF - MUSCLE & NERVE

IS - 2

ER -

PRE- AND POSTSYNAPTIC NEUROMUSCULAR JUNCTION ABNORMALITIES IN MuSK MYASTHENIA

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Keywords

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