Tj. Oshaughnessy et al., AMYOTROPHIC-LATERAL-SCLEROSIS - SERUM FACTORS ENHANCE SPONTANEOUS ANDEVOKED TRANSMITTER RELEASE AT THE NEUROMUSCULAR-JUNCTION, Muscle & nerve, 21(1), 1998, pp. 81-90
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 evid
enced by a 151% increase in MEPP frequency, by sera from 16 ALS patien
ts. In addition, 16 patients' sera elevated EEP quantal content by an
average of 89%. Eleven sera produced both effects. There was no consis
tent change in MEPP amplitude or time course, indicating the absence o
f a humoral effect on postjunctional ACh receptors or endplate membran
e function. These results suggest that a portion of the sporadic ALS p
atient population possess serum factors that can alter presynaptic fun
ction of the motor nerve terminal. Evidence from the present experimen
ts indicates that alterations at the neuromuscular junction are a resu
lt of a combination of increased Ca2+ influx into the cell and an inde
pendent increase in intracellular calcium concentration. (C) 1998 John
Wiley & Sons, Inc.