Combined deficiencies of endosomal ganglioside neuraminidases 3 and 4 in mice cause lysosomal GM3 gangliosidosis

The sialylated glycosphingolipids gangliosides are essential to myelination, axon integrity, transmission of nervous impulses and memory consolidation. Ganglioside expression patterns in the brain are cell-specific, dynamic and maintained by highly coordinated processes of biosynthesis, trafficking,...

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Bibliographic Details
Main Authors: Pshezhetsky, A, Pan, X, De Britto Pará De Aragão, C, Velasco-Martin, J, Priestman, D, Platt, F, Lamarche-Vaned, N, Morales, C, Miyagi, T
Format: Conference item
Published: Elsevier 2017
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Summary:The sialylated glycosphingolipids gangliosides are essential to myelination, axon integrity, transmission of nervous impulses and memory consolidation. Ganglioside expression patterns in the brain are cell-specific, dynamic and maintained by highly coordinated processes of biosynthesis, trafficking, processing and catabolism. The changes in the composition of gangliosides during development and aging cannot be explained based solely on the regulation of the ganglioside-synthesizing enzymes, sialyltransferases, suggesting that neuraminidases play essential role.