Abnormal glycosphingolipid metabolism in the nervous system of galactosialidosis

Journal of the Neurological Sciences
H YoshinoA Suzuki

Abstract

In an autopsy case of galactosialidosis, GM3, GM2, GM1, and GD1a were accumulated in sympathetic and spinal ganglia and grey matter of the spinal cord. Especially, the accumulations of GM3 and GM2 amounted to 41- and 86-fold increases in sympathetic ganglia, respectively, as compared to normal controls. In addition LacCer, GA2 and GA1 were accumulated in sympathetic and spinal ganglia. The accumulations of GM3 and GD1a are considered to be the result of defective lysosomal sialidase activity and the accumulation of GM1, LacCer and GA1 is also considered to be due to decreased beta-galactosidase activity in this disorder. To better understand the possible mechanism of GM2 accumulation, we determined the activity of GM2 synthesizing enzyme (GM3:UDP-GalNAc transferase), as well as hexosaminidase activity, in sympathetic ganglia, but they did not change. Abnormal ganglioside and neutral glycosphingolipid metabolism, as well as sialyloligosaccharide and sialylglycoprotein metabolism, may be involved in the pathogenesis of this disorder.

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Citations

Sep 20, 2011·Glycoconjugate Journal·Alexey V Pshezhetsky, Aleksander Hinek
Apr 20, 1999·Pediatric Neurology·O SohmaK Oyanagi
Sep 10, 2013·Biochemistry. Biokhimii︠a︡·A V Pshezhetsky, L I Ashmarina
Apr 1, 2000·Journal of Neuroimmunology·H YoshinoA Asano
Sep 3, 2004·Neuroscience Letters·Keiichiro SusukiNobuhiro Yuki
Oct 1, 2003·Human Mutation·Volkan SeyrantepeAlexey V Pshezhetsky
Sep 16, 2014·PloS One·Victoria SmutovaAlexey V Pshezhetsky
Apr 11, 2020·International Journal of Molecular Sciences·Bernadette Breiden, Konrad Sandhoff
Apr 24, 1995·Biochimica Et Biophysica Acta·V Gieselmann

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