Alexander disease: Alzheimer disease of the developing brain?

R. J. Castellani*, G. Perry, D. S. Brenner, M. A. Smith

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

6 Scopus citations

Abstract

Alexander disease is a leukodystrophy-like neurodegenerative disease that typically presents in infancy or childhood. The disease is essentially a sporadic condition, and there is no known genetic predisposition or metabolic abnormality. The hallmark of the disease is the diffuse accumulation of Rosenthal fibers (RF) throughout the central nervous system. Although anetiological relationship of the RF to disease pathogenesis has been suspected since the initial description of Alexander disease, such a relationship has not been confirmed. We previously identified a number of oxidative post-translational modifications, including advanced glycation end products and lipid peroxidation adducts, in intimate association with the RF of Alexander disease. Such oxidative protein damage provides a mechanism, through protein crosslinking, for insolubility and accumulation of RF. Notably, these findings show a striking parallel with the biochemical features of age-related neurodegenerative diseases such as Alzheimer disease. Therefore, Alexander disease and Alzheimer disease likely share a common pathogenesis, namely oxidative injury as a potential primary process in the etiology and pathogenesis.

Original languageEnglish (US)
Pages (from-to)232-235
Number of pages4
JournalAlzheimer Disease and Associated Disorders
Volume13
Issue number4
DOIs
StatePublished - 1999
Externally publishedYes

Keywords

  • Alexander disease
  • Alzheimer disease
  • Oxidative injury
  • Rosenthal fibers

ASJC Scopus subject areas

  • Clinical Psychology
  • Gerontology
  • Geriatrics and Gerontology
  • Psychiatry and Mental health

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