lysosomal disease
Last reviewed 01/2018
Lysosomes are subcellular organelles which are bounded by a single phospholipid bilayer membrane and contain one or more acid hydrolase enzyme.
Lysosome are involved in:
- biosynthesis
- exocytic pathways
- endocytic pathways
Lysosomes are the site of metabolism of many macromolecules which are:
- presented to the cell from the external environment
- synthesized within the cell
Absence of an enzymic activity causes accumulation of precursor chemicals in the lysosome. It is often the pathological storage of material in lysosomes which causes organ damage.
clinical features of lysosomal disease