Paired helical filament tau (PHFtau) in Niemann-Pick type C disease is similar to PHFtau in Alzheimer's disease |
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| Authors: | I. A. Auer M. L. Schmidt V. M. -Y. Lee B. Curry K. Suzuki R. -W. Shin P. G. Pentchev E. D. Carstea J. Q. Trojanowski |
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| Affiliation: | (1) Department of Histopathology, Foothills Hospital, University of Calgary, 1403 29 St. NW, T2N 2T9 Calgary, Alberta, Canada;(2) Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, USA;(3) Department of Pathology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA;(4) Department of Neuropathology, Kyushu University, Fukuoka, Japan;(5) Developmental and Metabolic neurology Branch, National Institute of Neurological Diseases and Stroke, NIH, Bethesda, MD, USA |
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| Abstract: | Niemann-Pick Type C disease (NPC) is a cholesterol storage disease with defects in the intracellular trafficking of exogenous cholesterol derived from low density lipoproteins. In NPC cases with a chronic progressive course, neurofibrillary tangles (NFTs) that consist of paired helical filaments (PHFs) have been reported. To determine if NPC tangles contain abnormal tau proteins (known as PHFtau) similar to those found in Alzheimer's disease (AD) tangles, we examined the brains of five NPC cases by immunohistochemical and Western blot methods using a library of antibodies to defined epitopes of PHFtau. We show here that PHFtau in tangle-rich NPC brains is indistinguishable from PHFtau in AD brains. We speculate, that the generation of PHFtau in NPC may induce a cascade of pathological events that contribute to the widespread degeneration of neurons, and that these events may be similar in NPC and AD. |
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| Keywords: | Cholesterol metabolism Cytoskeleton Dementia |
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