Alzheimer disease

Curly fibers and tangles in organs other than brain

J. Miklossy*, K. Taddei, R. Martins, G. Escher, R. Kraftsik, O. Pillevuit, D. Lepori, M. Campiche

*Corresponding author for this work

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

The filamentous brain lesions that define Alzheimer disease (AD) consist of senile plaques and neurofibrillary tangles. Undulated pathological filaments-curly fibers or neuropil threads-also occur in the neuropil. Beta- amyloid precursor proteins are synthesized by many cells outside-the central nervous system and recently, deposition of beta-amyloid-protein was reported to occur in non-neuronal tissues. In addition, increasing data claim the importance of chronic inflammation in the pathogenesis of AD. These observations suggest that AD may be a widespread systemic disorder. Here we report that pathological argyrophilic filaments with histochemical properties of amyloid showing striking morphological similarity to curly fibers and/or tangles accumulate not only in ependymal layer and in epithelial cells of choroid plexus, but also in several other organs (e.g. liver, pancreas, ovary, testis, thyroid) in AD. The ependyma, choroid plexus, and various organs of 39 autopsy cases were analyzed. In search of curly fiber and tangle-like changes in organs other than brain, 395 blocks from 21 different tissues of 24 AD Cases, 5 cases with discrete or moderate AD-type changes, and 10 control cases were investigated. We found in non-neuronal cells 'curly fibers' or 'tangles' immunoreactive with antibodies to P component, Tau- protein, ubiquitin, fibronectin, and Apolipoprotein-E, but lacking immunoreactivity with antibodies to neurofilament proteins. Ultrastructurally they consist of densely packed straight and paired helical filaments and closely resemble neurofibrillary tangles and neuropil threads. These observations indicate that the formation of 'curly fibers' and 'tangles' is not unique to the central nervous system. The results suggest that AD might be a systemic disorder or that similar fibrillary changes to tangles and curly fibers may also be associated with other amyloidosis than β- amyloidosis. Further investigations are necesSary to understand the pathogenetic interest of these fibrillary changes outside the CNS.

Original languageEnglish
Pages (from-to)803-814
Number of pages12
JournalJournal of Neuropathology and Experimental Neurology
Volume58
Issue number8
Publication statusPublished - Aug 1999
Externally publishedYes

Keywords

  • Alzheimer disease
  • Amyloid
  • Choroid plexus
  • Curly fibers
  • Ependyma
  • Neurofibrillary tangles
  • Systemic disorder

Fingerprint Dive into the research topics of 'Alzheimer disease: Curly fibers and tangles in organs other than brain'. Together they form a unique fingerprint.

  • Cite this

    Miklossy, J., Taddei, K., Martins, R., Escher, G., Kraftsik, R., Pillevuit, O., ... Campiche, M. (1999). Alzheimer disease: Curly fibers and tangles in organs other than brain. Journal of Neuropathology and Experimental Neurology, 58(8), 803-814.