Question

Given that sphingolipids represent less than 15% of the membrane lipids in eukaryotic cells, why are defects in lysosomal hydrolases that catalyze their degradation so deleterious?

Answer 1

Answer- Sphingolipids are a class of sphingosine-based lipids, found abundantly in the cell membranes in brain and other neural tissues. They help in signal transduction and cell recognition. Lysosomal hydrolases are responsible for the degradation of Sphingolipids; defects in these enzymes causes accumulation of non-biodegradable material in the cells, causing mitochondrial dysregulation and ultimately, cell death. Such defects in the metabolism of brain cells is disastrous for the body. Examples of such inheritable disorders include Fabry disease, Krabbe disease, Gaucher disease, Tay–Sachs disease, etc.