The Mannose-6-Phosphate Receptor (M6PR) is a crucial component in the cellular machinery, primarily involved in the targeting and transport of lysosomal enzymes. These receptors are transmembrane glycoproteins that play a significant role in the biogenesis of lysosomes by ensuring that newly synthesized hydrolytic enzymes are correctly delivered to the lysosome .
There are two main types of M6PRs:
The discovery of M6PRs is closely linked to the study of I-cell disease, a lysosomal storage disorder. Elizabeth Neufeld’s research on patients with multiple inclusion bodies led to the identification of the deficiency in the enzymes responsible for adding the M6P tag to lysosomal enzymes. This deficiency resulted in the mis-targeting of these enzymes, which were secreted instead of being delivered to lysosomes .
Recombinant M6PRs are produced using recombinant DNA technology, allowing for the study and utilization of these receptors in various research and therapeutic applications. These recombinant receptors retain the functional properties of their natural counterparts, making them valuable tools in understanding lysosomal enzyme targeting and transport mechanisms .