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Amyotrophic lateral sclerosis: Serum factors enhance spontaneous and evoked transmitter release at the neuromuscular junction
Author(s) -
O'Shaughnessy Thomas J.,
Yan Haidun,
Kim Jimok,
Middlekauff Eric H.,
Lee Kwang W.,
Phillips Lawrence H.,
Kim Jun,
Kim Yong I.
Publication year - 1998
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199801)21:1<81::aid-mus11>3.0.co;2-3
Subject(s) - amyotrophic lateral sclerosis , neuromuscular junction , motor nerve , population , intracellular , receptor , medicine , calcium , endocrinology , chemistry , neuroscience , biology , anatomy , biochemistry , disease , environmental health
Sera from 30 patients with sporadic amyotrophic lateral sclerosis (ALS) were tested to determine their effects at the neuromuscular junction. Spontaneous transmitter release was significantly increased, as evidenced by a 151% increase in MEPP frequency, by sera from 16 ALS patients. In addition, 16 patients' sera elevated EEP quantal content by an average of 89%. Eleven sera produced both effects. There was no consistent change in MEPP amplitude or time course, indicating the absence of a humoral effect on postjunctional ACh receptors or endplate membrane function. These results suggest that a portion of the sporadic ALS patient population possess serum factors that can alter presynaptic function of the motor nerve terminal. Evidence from the present experiments indicates that alterations at the neuromuscular junction are a result of a combination of increased Ca 2+ influx into the cell and an independent increase in intracellular calcium concentration. © 1998 John Wiley & Sons, Inc. Muscle Nerve, 21: 81–90, 1998.