MeSH

A subclass of ACIDIC GLYCOSPHINGOLIPIDS. They contain one or more sialic acid (N-ACETYLNEURAMINIC ACID) residues. Using the Svennerholm system of abbrevations, gangliosides are designated G for ganglioside, plus subscript M, D, or T for mono-, di-, or trisialo, respectively, the subscript letter being followed by a subscript arabic numeral to indicated sequence of migration in thin-layer chromatograms. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1997)

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.

Year introduced: 1992(1976)

A subclass of GLYCOSPHINGOLIPIDS containing large polar heads made up of several sugar units. One or more of their terminal sugar units are bound to a negatively charged molecule at pH 7. Members of this class include: GANGLIOSIDES, uronoglycosphingolipids, SULFOGLYCOSPHINGOLIPIDS, phosphoglycosphingolipids, and phosphonoglycosphingolipids.

Year introduced: 1999

A group of recessively inherited diseases characterized by the intralysosomal accumulation of G(M2) GANGLIOSIDE in the neuronal cells. Subtypes include mutations of enzymes in the BETA-N-ACETYLHEXOSAMINIDASES system or G(M2) ACTIVATOR PROTEIN leading to disruption of normal degradation of GANGLIOSIDES, a subclass of ACIDIC GLYCOSPHINGOLIPIDS.

Year introduced: 2007 (2000)

An autosomal recessive neurodegenerative disorder characterized by an accumulation of G(M2) GANGLIOSIDE in neurons and other tissues. It is caused by mutation in the common beta subunit of HEXOSAMINIDASE A and HEXOSAMINIDASE B. Thus this disease is also known as the O variant since both hexosaminidase A and B are missing. Clinically, it is indistinguishable from TAY-SACHS DISEASE.

Year introduced: 1979

Lipids containing at least one monosaccharide residue and either a sphingoid or a ceramide (CERAMIDES). They are subdivided into NEUTRAL GLYCOSPHINGOLIPIDS comprising monoglycosyl- and oligoglycosylsphingoids and monoglycosyl- and oligoglycosylceramides; and ACIDIC GLYCOSPHINGOLIPIDS which comprises sialosylglycosylsphingolipids (GANGLIOSIDES); SULFOGLYCOSPHINGOLIPIDS (formerly known as sulfatides), glycuronoglycosphingolipids, and phospho- and phosphonoglycosphingolipids. (From IUPAC's webpage)

Year introduced: 1975

An aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.

A ganglioside present in abnormally large amounts in the brain and liver due to a deficient biosynthetic enzyme, G(M3):UDP-N-acetylgalactosaminyltransferase. Deficiency of this enzyme prevents the formation of G(M2) ganglioside from G(M3) ganglioside and is the cause of an anabolic sphingolipidosis.

Year introduced: 1991(1977)

A glycosphingolipid that accumulates due to a deficiency of hexosaminidase A or B (BETA-N-ACETYLHEXOSAMINIDASES), or GM2 activator protein, resulting in GANGLIOSIDOSES, heredity metabolic disorders that include TAY-SACHS DISEASE and SANDHOFF DISEASE.

Year introduced: 1991(1975)

A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis.

Year introduced: 1986(1977)

catalyzes synthesis of GD1b from GD2, GM1 from GM2, and GA1 from GA2 gangliosides; amino acid sequence given in first source; GenBank AB003478

Date introduced: October 21, 1997

found in human meconium; member of the lactotetraose series of gangliosides

Date introduced: August 15, 1989

neuropathy antigenic glycolipid from human brain gangliosides

Date introduced: April 20, 1988

neuropathy antigenic glycolipid from human brain gangliosides

Date introduced: April 20, 1988

mixture of gangliosides extracted & purified from bovine brain cortex

Date introduced: April 15, 1983

gangliosides found in rabbit brain which contain fucose; often found in neoplasms, but absent in normal cells; see also specific fucogangliosides diasyl Le(a)

Date introduced: August 21, 1981

polyoma virus recognizes specific sialyloligosaccharide receptors on host cells; human influenza A virus hemagglutinin distinguishes sialyloligosaccharides in membrane-associated gangliosides

Date introduced: March 30, 1981