Substructure of 20 nm filaments of progressive supranuclear palsy.
In contrast to the ultrastructure of Alzheimer's neurofibrillary tangles (NFT), which has been well characterized as accumulations of paired helical 10-nm filaments (PHF) with 80-nm regular constrictions, the morphology of the neurofibrillary changes of PSP remains ill-defined. Until recently, the fine structure of PSP tangles was generally accepted as 15-nm straight filaments or tubules, although many reports describing different electron-microscopic findings have appeared in the literature. In this report, we present morphological data indicating a protofilamentous substructure present in straight filaments of PSP which has some points of similarity with the protofilamentous architectures which have been reported for paired helical filaments of Alzheimer's disease. The straight filaments were found to be composed of six or more helically symmetric 2-5-nm protofilaments. We conclude that despite the varied morphology of filaments in neurofibrillary tangles observed in PSP there may be some underlying identity at the molecular level with the PHF of neurofibrillary tangles of Alzheimer's disease.
D000368:Aged; D000544:Alzheimer Disease / Q000473:pathology; D001921:Brain / Q000473:pathology*; D010244:Bulbar Palsy, Progressive / Q000473:pathology*; D003599:Cytoskeleton / Q000648:ultrastructure*; D003711:Demyelinating Diseases / Q000473:pathology*; D006801:Humans; D007382:Intermediate Filaments / Q000648:ultrastructure*; D008297:Male; D008854:Microscopy, Electron; D009454:Neurofibrils / Q000648:ultrastructure*; D009886:Ophthalmoplegia / Q000473:pathology*; D013577:Syndrome