title: Comparative study of intraneuronal polyglucosan bodies in brains from patients with Lafora disease and aged dogs.
abstract: We compared intraneuronal polyglucosan body (PGB) in brains from two patients diagnosed as having Lafora disease and from 18 aged dogs (age range: 10 to 22 years, various breeds). PGBs appeared as various-sized spheroids intensely stained with periodic acid-Schiff (PAS) in both humans and aged dogs. Immunohistochemistry with a monoclonal antibody raised against human polyglucosan showed positive staining of the PGBs. Ultrastructurally, PGBs in both humans and aged dogs were composed of fibril-like structures 4 to 20 nm wide. Electron-dense material formed the central cores of the fibrils, but was also scattered in their peripheral areas. The fibril-like structures were intensely stained upon application of the Thiéry procedure. Immunoelectron microscopy showed that the fibril-like structures were specifically labeled with gold particles. The histological, immunohistochemical and ultrastructural features of the PGBs in humans and aged dogs were quite similar.
authors.affiliation: First Department of Pathology, Yamaguchi University School of Medicine, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Yamanami
authors.forename: T
authors.initials: T
authors.lastname: Ishihara
authors.forename: M
authors.initials: M
authors.lastname: Takahashi
authors.forename: F
authors.initials: F
authors.lastname: Uchino
mesh_terms: D000328:Adult; D000375:Aging / Q000473:pathology*; D000818:Animals; D001921:Brain / Q000473:pathology*; D004285:Dogs; D004831:Epilepsies, Myoclonic / Q000473:pathology*; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008297:Male; D008854:Microscopy, Electron; D016253:Microscopy, Immunoelectron; D009474:Neurons / Q000648:ultrastructure*