Sign Up for a Free Account

This is an image preview.
Start a Free Account
to view the full image.

  • Nearly 3,000 illustrations, including video clips of neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of neurology in 1,200 comprehensive articles.

  • Listen to MedLink on the go with Audio versions of each article.

Biosynthetic and degradation pathways of glycosphingolipids in the brain

This diagram shows the metabolic loci for Fabry disease, metachromatic leukodystrophy, Krabbe disease, Gaucher disease, Sandhoff disease, Sialidosis, Tay Sachs disease/Sandhoff disease/GM2 gangliosidosis AB variant, and GM1 gangliosidosis. The nomenclature for gangliosides and the components are based on those of Svennerholm (1963) and IUPAC-IUBMB Joint Commission on Biochemical Nomenclature (IUPAC-IUB Joint Commission on Biochemical Nomenclature 1997; Chester 1997). Glycosphingolipids belonging to the asialo-series, ganglio-series, lacto/neo-lacto-series and globo/iso-globo-series are colored in brown, blue, purple and green, respectively. Lysosomal glycosphingolipid storage disorders resulting from an enzyme defect are indicated in brackets and in red in the figure.

Abbreviations and chromosomal locations of the responsible genes: A4GALT alpha 1,4- galactosyltransferase (22q13.2); ARSA arylsulfatase A (22q13.33); B3GALT4 beta-1,3-galactosyltransferase 4 (6p21.32); B4GALNT1 beta-1,4-N-acetyl- galactosaminyltransferase 1 (12q13.3); B4GALT6 beta-1,4-galactosyltransferase 6 (18q12.1); GAL3ST1 galactose-3-O-sulfotransferase 1 (22q12.2); GALC galactosylceramidase (14q31.3); GBA glucosylceramidase beta also named GBA1 (1q22); GBA2 glucosylceramidase beta 2 (9p13.3); GBA3 glucosylceramidase beta 3 (4p15.2); GLA galactosidase alpha (Xq22.1); GLB1 galactosidase beta 1 (3p22.3); HEXA hexosaminidase subunit alpha (15q23); HEXB hexosaminidase subunit beta (5q13.3); NEU1 neuraminidase 1 (6p21.33); NEU2 neuraminidase 2 (2q37.1); NEU3 neuraminidase 3 (11q13.5); NEU4 neuraminidase 4( 2q37.3); SM4s 3-sulfo-GalCer (also known as sulfatide, galactocerebroside, 3-O-sulfogalactosylceramide, SGalCer, and SGC); ST3GAL1 ST3 beta-galactoside alpha-2,3-sialyltransferase 1 (8q24.22); ST3GAL2 ST3 beta-galactoside alpha-2,3-sialyltransferase 2 (16q22.1); ST3GAL5 ST3 beta-galactoside alpha-2,3-sialyltransferase 5 (2p11.2); ST3GAL6 ST3 beta-galactoside alpha-2,3- sialyltransferase 6 (3q12.1); ST6GALNAC6 ST6 N-acetylgalactosaminide alpha-2,6-sialyltransferase 6 (9q34.11); ST8SIA1 ST8 alpha-N-acetyl- neuraminide alpha-2,8-sialyltransferase 1 (12p12.1); ST8SIA3 ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 3 (ST8SIA3, 18q21.31); ST8SIA5 ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 5 (18q21.1); UGCG UDP-glucose ceramide glucosyltransferase (9q31.3); UGT8 UDP glycosyltransferase 8 (4q26).

Chester MA. IUPAC-IUB Joint Commission on Biochemical Nomenclature (JCBN). Nomenclature of glycolipids--recommendations 1997. Eur J Biochem 1998;257(2):293-8.
IUPAC-IUB Joint Commission on Biochemical Nomenclature (JCBN). Nomenclature of glycolipids recommendations 1997. J Mol Biol. 1999;286(3):963-70.
Svennerholm L. Chromatographic separation of human brain gangliosides. J Neurochem 1963;10:613–23.

(Source: Belarbi K, Cuvelier E, Bonte MA, et al. Glycosphingolipids and neuroinflammation in Parkinson's disease. Mol Neurodegener 2020;15[1]:59. Creative Commons Attribution 4.0 International [CC BY 4.0] license,