Drost, M.R.

muscle patient disease fiber pompe disease pompe type 2 muscle fibers therapy type 1 enzyme replacement therapy enzyme biopsy study percentage lysosomal month group netherland fi bers muscle fi bers van der ploeg infantile-onset pompe disease 17 months infant muscle strength nonclassic patients enzyme therapy storage ivs 1 mutation lysosomal storage disorder lamp 1 signal lamp 1 intensity erasmus mc university erasmus mc damage strength muscle biopsies glycogen nonclassic distribution section replacement symptom adult fi ber types university mhc type 2 metab dis fig raben metab erasmu intensity supine position onset 3 months muscle weakness correlation van doorn pa response type 1 fibers van den berg fiber-type winkel difference protein analysis control value symptom onset non-contractile inclusions flexor center pompe patients ploeg acid maltase muscle damage membrane reuser maastricht