ganglioside catabolic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways resulting in the breakdown of ganglioside, a ceramide oligosaccharide carrying, in addition to other sugar residues, one or more sialic residues. [ISBN:0198547684]

Ganglioside catabolic process is a complex and essential biological pathway responsible for the breakdown and removal of gangliosides, a class of complex glycosphingolipids found predominantly in the brain and other nervous tissues. Gangliosides play vital roles in neuronal signaling, cell adhesion, and membrane organization. Their catabolism involves a sequential enzymatic degradation of the molecule, starting with the removal of the terminal sialic acid residue by neuraminidase. The resulting product is then further hydrolyzed by a series of lysosomal enzymes, including hexosaminidase, β-galactosidase, and β-glucosidase. These enzymes progressively cleave off the sugar moieties of the ganglioside molecule until only the ceramide backbone remains. This backbone is then further degraded by ceramidase, generating sphingosine and a fatty acid. The final products of ganglioside catabolism are then recycled or excreted from the cell. Proper ganglioside catabolism is critical for maintaining neuronal function and integrity. Defects in this process can lead to the accumulation of gangliosides within cells, resulting in a variety of lysosomal storage disorders, such as Tay-Sachs disease, Sandhoff disease, and GM1 gangliosidosis. These disorders are characterized by severe neurological dysfunction and often result in premature death.'
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Proteins (6)

Compounds (36)