Globoside
Encyclopedia
A globoside is a type of glycosphingolipid with more than one sugar
s as the side chain (or R group
). The sugars are usually a combination of N-Acetylgalactosamine
, D-glucose or D-galactose. A glycosphingolipid that has only one sugar as the side chain is called a cerebroside
.
The side chain can be cleaved by beta-hexosaminidase
. If the enzyme is not functioning correctly, then globosides can accumulate, leading to Sandhoff disease
.
Sugar
Sugar is a class of edible crystalline carbohydrates, mainly sucrose, lactose, and fructose, characterized by a sweet flavor.Sucrose in its refined form primarily comes from sugar cane and sugar beet...
s as the side chain (or R group
R group
R group may refer to:* Side chain in chemistry* Tempered representation in mathematics...
). The sugars are usually a combination of N-Acetylgalactosamine
N-Acetylgalactosamine
N-Acetylgalactosamine , is an amino sugar derivative of galactose.-Function:In humans it is the terminal carbohydrate forming the antigen of blood group A....
, D-glucose or D-galactose. A glycosphingolipid that has only one sugar as the side chain is called a cerebroside
Cerebroside
Cerebrosides is the common name for a group of glycosphingolipids called monoglycosylceramides which are important components in animal muscle and nerve cell membranes.They consist of a ceramide with a single sugar residue at the 1-hydroxyl moiety...
.
The side chain can be cleaved by beta-hexosaminidase
Hexosaminidase
Hexosaminidase is an enzyme involved in the hydrolysis of terminal N-acetyl-D-hexosamine residues in N-acetyl-β-D-hexosaminides.- Lysosomal A, B, and S isozymes :Functional lysosomal β-hexosaminidase enzymes are dimeric in structure...
. If the enzyme is not functioning correctly, then globosides can accumulate, leading to Sandhoff disease
Sandhoff disease
Sandhoff disease, also known as Sandhoff-Jatzkewitz disease, variant 0 of GM2-Gangliosidosis or Hexosaminidase A and B deficiency, is a lysosomal genetic, lipid storage disorder caused by the inherited deficiency to create functional beta-hexosaminidases A and B...
.