Answer
Cipaglucosidase alfa, stabilised by miglustat, binds mannose-6-phosphate receptors and enters lysosomes, where it is processed into active acid alpha-glucosidase to replace the absent or impaired enzyme, break down glycogen and reduce its accumulation in muscle.
Sources for this answer
- Pombiliti 105 mg powder for concentrate for solution for infusionAmicus Therapeutics UK Ltd · emc · SmPC §5.1 Pharmacodynamic properties · Version 3Document updated
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