Article
G(M1)-ganglioside degradation and biosynthesis in human and murine G(M1)-gangliosidosis.
Clinica chimica acta; international journal of clinical chemistry - 1 Apr 2005
Sano Renata, Trindade Vera M T, Tessitore Alessandra, d'Azzo Alessandra, Vieira Matheus B, Giugliani Roberto, Coelho Janice C
Abstract excerpt
BACKGROUND: Gangliosides are building blocks of cell membranes and their biosynthesis and degradation have been extensively studied in the past. Regulation of the metabolism of these glycolipids controls fundamental cell functions. G(M1)-gangliosidosis, a neurodegenerative glycosphingolipid storage disease, is caused by deficiency of lysosomal beta-galactosidase with consequent disruption of the normal...
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