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Mapping the canine lumbosacral spinal cord neurons damaged by ischemia-reperfusion after high thoracic aorta ligation was performed using the Nauta degenerating method. Highly Nauta-positive perikarya of the long ascending projection systems in the 4th to 6th dorsal layer, interneurons in the 7th layer and motoneurons in the 8th and 9th layers in L3-S3 segments subjected to 30 min of ischemia and 30 min of reperfusion were localized and their laminar distribution was specified. Spastic paraplegia fully developed 2 days postoperatively after 30 min of aortic ligation is neurohistopathologically characterized by occurrence of enlarged Nauta-positive boutons with prevailing localization in the 4th to 8th layer of the gray matter.

SummaryThe transcriptional coactivator PGC-1α induces multiple effects on muscle, including increased mitochondrial mass and activity. Amyotrophic lateral sclerosis (ALS) is a progressive, fatal, adult-onset neurodegenerative disorder characterized by selective loss of motor neurons and skeletal muscle degeneration. An early event is thought to be denervation-induced muscle atrophy accompanied by alterations in mitochondrial activity and morphology within muscle. We now report that elevation of PGC-1α levels in muscles of mice that develop fatal paralysis from an ALS-causing SOD1 mutant elevates PGC-1α-dependent pathways throughout disease course. Mitochondrial biogenesis and activity are maintained through end-stage disease, accompanied by retention of muscle function, delayed muscle atrophy, and significantly improved muscle endurance even at late disease stages. However, survival was not extended. Therefore, muscle is not a primary target of mutant SOD1-mediated toxicity, but drugs increasing PGC-1α activity in muscle represent an attractive therapy for maintaining muscle function during progression of ALS.