TY - JOUR
AR - JBN-2021-1-103
TI - Electrophysiological and Morphological Studies of SOD1 Transgenic Mice: An Animal Model of ALS
AU - N, Hori
AU - Y, Tan
AU - Z, Xu
AU - N, Akaike
AU - David O. , Carpenter
JO - Journal of Brain and Nerves
PY - 2021
DA - Sat 02, Oct 2021
SN - 2733-2284
DO - http://dx.doi.org/10.31487/j.JBN.2021.01.03
UR - https://www.sciencerepository.org/electrophysiological-and-morphological_JBN-2021-1-103 
KW - Lucifer yellow CH, ALS, membrane potential, membrane resistance, dendrites, glutamic-acid subtypes
AB - Amyotrophic lateral sclerosis (ALS) is a disease where upper and lower motor neurons die, and it is often associated with mutations of superoxide dismutase 1 (SOD1). We have used mouse models to compare physiologic and morphologic characteristics of cervical motor neurons in wild-type and mutant animals. Slices of the cervical spinal cord were prepared from old wild-type and mutant G93A and G85R mice, and intracellular recordings of membrane potential, resistance and responses to application of excitatory neurotransmitters were studied. Some motor neurons were injected with Lucifer Yellow for morphological analysis. There were no significant differences between membrane potential in the SOD1 mutants and aged wild-type mice, but membrane resistance was somewhat higher in the mutant motor neurons. Dendrites of the mutant motor neurons were not responsive to ionophoretic application of excitatory amino acids, although the cell body responded strongly. In Lucifer-filled cells, the dendrites were found to disappear. Mutant motor neurons were sometimes spontaneously active. Responses of mutant motor neurons to perfused glutamate with varying calcium concentrations in the Ringer’s solution were different from those of the wild-type cells.