title: Morphological study of disordered myelination and the degeneration of nerve fibers in the spinal cord of mice lacking complex gangliosides.
abstract: Gangliosides, a family of glycosphingolipids that contain sialic acid, are abundant on the neuronal cell membranes, but their precise functions in the central nervous system remain largely undefined. In a previous study of GalNAc-T(-/-) mice engineered to lack beta1,4-N-acetylgalactos-aminyltransferase (GM2/GD2 synthase) to abolish any, complex gangliosides, we observed the reduction of nerve conduction velocity but did not find any obvious morphological change in the brain. In the present study, we observed morphological changes in the nerve fiber tracts of the spinal cord in these mice. In GalNAc-T(-/-) mice, the number of degenerated axons was markedly increased in the dorsal funiculus, tract of Lissauer, and dorsolateral funiculus of the cervical segment of the spinal cord as well as the dorsal funiculus and tract of Lissauer of the lumbar segment of the spinal cord. There were also increased numbers of unmyelinated fibers in GalNAc-T(-/-) mice. Loosened myelin sheaths and myelin sheaths separated from axons by wide spaces were also observed in GalNAc-T(-/-) mice. These results provide a morphological basis for the previously observed reduction in the nerve conduction velocity and suggest that complex gangliosides are essential for the maintenance of myelin and the integrity of nerve fibers of the spinal cord.
authors.affiliation: Department of Functional Anatomy and Neuroscience, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Qian
authors.initials: Q
authors.lastname: Ma
authors.forename: Miya
authors.initials: M
authors.lastname: Kobayashi
authors.forename: Midori
authors.initials: M
authors.lastname: Sugiura
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Ozaki
authors.forename: Koji
authors.initials: K
authors.lastname: Nishio
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Shiraishi
authors.forename: Keiko
authors.initials: K
authors.lastname: Furukawa
authors.forename: Koichi
authors.initials: K
authors.lastname: Furukawa
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Sugiura
mesh_terms: D000818:Animals; D002452:Cell Count; D051379:Mice; D008817:Mice, Mutant Strains; D017350:N-Acetylgalactosaminyltransferases / Q000235:genetics*; D009410:Nerve Degeneration / Q000473:pathology / Q000503:physiopathology*; D009413:Nerve Fibers, Myelinated / Q000473:pathology*; D009431:Neural Conduction; D010641:Phenotype; D013116:Spinal Cord / Q000473:pathology*
chemical_list: D017350:N-Acetylgalactosaminyltransferases; C023220:(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase