Gangliosidosis

A group of inherited lipid storage disorders marked by accumulation of gangliosides in tissues due to an enzyme defect. Characterized by progressive neuromuscular dysfunction and impaired growth from an early age.

• GM₁ g. — a defect of β-galactosidase which causes accumulation of galactoside GM₁. Identified in Friesian cattle, dogs and cats.
• GM₂ g. — a defect of hexosaminidase A in dogs and pigs, and hexosaminidase A and B in cats. Analogous to the similar diseases in humans, which are called also Sandhoff's disease, Tay–Sachs disease and Bernheimer–Seitelberger disease.

Gangliosidosis
Classification and external resources
ICD-10	E75.0-E75.1
ICD-9	330.1
MeSH	[1]

Gangliosidosis is a lipid storage disorder caused by the accumulation of lipids known as gangliosides. There are two distinct genetic causes of the disease. Both are autosomal recessive and affect males and females equally.

Types

• GM1 gangliosidoses - GM1
• GM2 gangliosidoses - GM2

See also

• Sphingolipidoses#Overview for an overview table, including gangliosidosis

v • d • e (LSD) Inborn error of lipid metabolism: lipid storage disorders (E75, 272.7-272.8) Sphingolipidoses (to ceramide) From ganglioside (gangliosidoses) Ganglioside: GM1 gangliosidoses · GM2 gangliosidoses (Sandhoff disease, Tay-Sachs disease, AB variant) From globoside Globotriaosylceramide: Fabry's disease From sphingomyelin Sphingomyelin: phospholipid: Niemann-Pick disease (SMPD1-associated, type C) Glucocerebroside: Gaucher's disease From sulfatide (sulfatidoses, leukodystrophy) Sulfatide: Metachromatic leukodystrophy · Multiple sulfatase deficiency Galactocerebroside: Krabbe disease To sphingosine Ceramide: Farber disease NCL Infantile · Jansky-Bielschowsky disease · Batten disease Other Cerebrotendineous xanthomatosis · Cholesteryl ester storage disease (Wolman disease) · Sea-blue histiocyte syndrome see also enzymes, sphingolipids

External links

• Hide & Seek Foundation for Lysosomal Disease Research

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