Occludin deficiency with BACE1 elevation in cerebral amyloid angiopathy

Neurology. 2014 May 13;82(19):1707-15. doi: 10.1212/WNL.0000000000000403. Epub 2014 Apr 16.

Abstract

Objective: A significant cause of spontaneous hemorrhages in the elderly is cerebral amyloid angiopathy (CAA), which causes degeneration of cerebral vessels, but the mechanisms are unclear.

Methods: We isolated leptomeningeal vessels from rapidly autopsied brains (the average of postmortem intervals was 3.28 hours) from 9 patients with CAA and 10 age-matched controls, and used molecular, cell biology, and immunohistochemical approaches to examine β-site APP-cleaving enzyme 1 (BACE1) protein expression and enzymatic activities as well as tight junction molecular components in small- and medium-sized arteries of the cerebral cortex and leptomeninges.

Results: We not only identified that the cerebral vessels, including leptomeningeal and cortical vessels, synthesize and express BACE1, but also found a significant elevation of both BACE1 protein levels and enzymatic activities in leptomeningeal vessels from patients with CAA. Moreover, overexpression of BACE1 in endothelial cells resulted in a significant reduction of occludin, a tight junction protein in blood vessels.

Conclusion: These findings suggest that in addition to neurons, cerebral vascular cells express functional BACE1. Moreover, elevated vascular BACE1 may contribute to deficiency of occludin in cerebral vessels, which ultimately has a critical role in pathogenesis of CAA and its related hemorrhage.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Amyloid Precursor Protein Secretases / metabolism*
  • Aspartic Acid Endopeptidases / metabolism*
  • Case-Control Studies
  • Cerebral Amyloid Angiopathy / enzymology*
  • Cerebral Amyloid Angiopathy / metabolism
  • Cerebral Arteries / enzymology
  • Cerebral Arteries / metabolism
  • Cerebral Cortex / blood supply
  • Cerebral Cortex / enzymology
  • Female
  • Humans
  • Male
  • Meninges / blood supply
  • Meninges / enzymology
  • Occludin / deficiency*

Substances

  • Occludin
  • Amyloid Precursor Protein Secretases
  • Aspartic Acid Endopeptidases
  • BACE1 protein, human