title: Polysaccharide (amylopectin-like) storage myopathy histochemical ultrastructural and biochemical studies.
abstract: A case of an adult polysaccharide myopathy is reported in a patient with progressive muscular atrophy and weakness of limb girdles. Histochemistry and electron microscopy showed in some muscle fibers, a storage material composed of amylopectin-like filaments. Biochemical results were normal and no enzyme deficiency was found. This case is compared with three other published cases. Pathological conditions with amylopectin or amylopectin-like storage material are reviewed.
mesh_terms: D000687:Amylopectin / Q000378:metabolism*; D006003:Glycogen / Q000378:metabolism; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009132:Muscles / Q000201:enzymology / Q000473:pathology; D009133:Muscular Atrophy / Q000201:enzymology / Q000473:pathology*; D011134:Polysaccharides / Q000378:metabolism*
chemical_list: D011134:Polysaccharides; D006003:Glycogen; D000687:Amylopectin