Disease or Syndrome
gangliosidosis
Subclass of:
Sphingolipidoses
Definitions related to gangliosidoses:
  • A group of autosomal recessive lysosomal storage disorders marked by the accumulation of GANGLIOSIDES. They are caused by impaired enzymes or defective cofactors required for normal ganglioside degradation in the LYSOSOMES. Gangliosidoses are classified by the specific ganglioside accumulated in the defective degradation pathway.
    NLM Medical Subject Headings
    U.S. National Library of Medicine, 2021
  • (gangliosidosis) Group of often fatal inherited diseases marked by the accumulation of gangliosides in lysosomes secondary to enzymatic deficiency states; gangliosidoses include Tay-Sachs disease, gangliosidosis GM1, gangliosidoses GM2, and Sandhoff disease; which share the infantile or childhood onset of central nervous system deterioration.
    CRISP Thesaurus
    National Institutes of Health, 2006
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