TY - JOUR
T1 - Amyotrophic lateral sclerosis
T2 - Serum factors enhance spontaneous and evoked transmitter release at the neuromuscular junction
AU - O'Shaughnessy, Thomas J.
AU - Yan, Haidun
AU - Kim, Jimok
AU - Middlekauff, Eric H.
AU - Lee, Kwang W.
AU - Phillips, Lawrence H.
AU - Kim, Jun
AU - Kim, Yong I.
PY - 1998
Y1 - 1998
N2 - 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 Ca2+ influx into the cell and an independent increase in intracellular calcium concentration.
AB - 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 Ca2+ influx into the cell and an independent increase in intracellular calcium concentration.
KW - Amyotrophic lateral sclerosis
KW - Calcium channels
KW - Evoked transmitter release
KW - Neuromuscular junction
KW - Quantal content
UR - http://www.scopus.com/inward/record.url?scp=0031982787&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0031982787&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1097-4598(199801)21:1<81::AID-MUS11>3.0.CO;2-3
DO - 10.1002/(SICI)1097-4598(199801)21:1<81::AID-MUS11>3.0.CO;2-3
M3 - Article
C2 - 9427227
AN - SCOPUS:0031982787
SN - 0148-639X
VL - 21
SP - 81
EP - 90
JO - Muscle and Nerve
JF - Muscle and Nerve
IS - 1
ER -