The remaining debris generally stays within the lysosome and is called a residual body. |
Lysosomal diseases are rare genetic diseases which include about fifty handicapping affections of the child and the adult. Their common feature is a genetic deficiency inducing a malfunction of the lysosome. |
The site of action for idursulfase is the lysosome, and so the observed reductions in GAG levels were therefore considered to be indicative of lysosomal uptake of idursulfase. |
For example, the internal space of lysosomes is much more acidic than that of the cytosol pH 5 as opposed to pH 7 and is maintained by specific proton-pumping transport proteins in the lysosome membrane. |
Each enzyme in the lysosome is responsible for a certain step in the breakdown of the sugar chains. |
During the process, nutrients are recycled by the lysosome, an internal organelle, to produce metabolites that can be used by the cell. |