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Showing 1 to 2 of 2 for “"GM2 Gangliosidosis"”.

  1. Preclinical Assessment of Intravenous Gene Therapy for GM2 Gangliosidosis

    GM2 Gangliosidoses are a group of severe neurodegenerative lysosomal storage disorders characterized by the inability to catabolize GM2 Gangliosides, leading to a neurotoxic accumulation of the GM2 lipids within the central nervous system. The Hexosaminidase A (HexA) enzyme is a heterodimeric …

    queens Repository record for Preclinical Assessment of Intravenous Gene Therapy for GM2 Gangliosidosis (opens in a new tab)

  2. Phenotypic characterisation of human iPSC neuronal models of GM2 gangliosidoses

    … lysosomal storage disorders (LSD). The GM2 gangliosidoses Tay-Sachs and Sandhoff disease are a type of LSD, resulting from the inability of the lysosome to catabolise the breakdown of the ganglioside GM2. This is due to a loss or mutation of either the HEXA or HEXB genes which form the …

    cambridge Repository record for Phenotypic characterisation of human iPSC neuronal models of GM2 gangliosidoses (opens in a new tab)