TY - JOUR
T1 - Amyotrophic lateral sclerosis associated with mutations in superoxide dismutase
T2 - A putative mechanism of degeneration
AU - Morrison, Brett M.
AU - Morrison, John H.
PY - 1999/1
Y1 - 1999/1
N2 - Amyotrophic lateral sclerosis (ALS) is a devastating neurologic disease that rapidly progresses from mild motor symptoms to severe motor paralysis and premature death. Until recently, there were few substantive studies conducted on the pathogenesis of the disease. With the genetic linkage of mutations in the superoxide dismutase (SOD-1) gene with familial ALS patients, new avenues for study have become available including transgenic mice and culture models. Although not yet providing a complete picture of the disease mechanism, studies utilizing these model systems have greatly advanced our understanding of the mechanism of degeneration and should eventually lead to putative therapeutic agents. In this review, we will present the important findings from these model systems, provide a framework in which to evaluate these findings, and speculate on the mechanism of degeneration initiated by the mutations in SOD-1.
AB - Amyotrophic lateral sclerosis (ALS) is a devastating neurologic disease that rapidly progresses from mild motor symptoms to severe motor paralysis and premature death. Until recently, there were few substantive studies conducted on the pathogenesis of the disease. With the genetic linkage of mutations in the superoxide dismutase (SOD-1) gene with familial ALS patients, new avenues for study have become available including transgenic mice and culture models. Although not yet providing a complete picture of the disease mechanism, studies utilizing these model systems have greatly advanced our understanding of the mechanism of degeneration and should eventually lead to putative therapeutic agents. In this review, we will present the important findings from these model systems, provide a framework in which to evaluate these findings, and speculate on the mechanism of degeneration initiated by the mutations in SOD-1.
KW - Excitotoxicity
KW - Neurofilament
KW - Transgenic
UR - http://www.scopus.com/inward/record.url?scp=0032908774&partnerID=8YFLogxK
U2 - 10.1016/S0165-0173(98)00049-6
DO - 10.1016/S0165-0173(98)00049-6
M3 - Article
C2 - 9974153
AN - SCOPUS:0032908774
SN - 0165-0173
VL - 29
SP - 121
EP - 135
JO - Brain Research Reviews
JF - Brain Research Reviews
IS - 1
ER -