Presenilin is necessary for efficient proteolysis through the autophagy-lysosome system in a γ-secretase-independent manner

J Neurosci. 2011 Feb 23;31(8):2781-91. doi: 10.1523/JNEUROSCI.5156-10.2010.

Abstract

Presenilins are ubiquitous, intramembrane proteins that function in Alzheimer's disease (AD) as the catalytic component of the γ-secretase complex. Familial AD mutations in presenilin are known to exacerbate lysosomal pathology. Hence, we sought to elucidate the function endogenous, wild-type presenilins play in autophagy-mediated protein degradation. We report the finding that genetic deletion or knockdown of presenilins alters many autophagy-related proteins demonstrating a buildup of autophagosomes, indicative of dysfunction in the system. Presenilin-deficient cells inefficiently clear long-lived proteins and fail to build up autophagosomes when challenged with lysosomal inhibitors. Our studies further show that γ-secretase inhibitors do not adversely impact autophagy, indicating that the role of presenilins in autophagy is independent of γ-secretase activity. Based on our findings, we conclude that endogenous, wild-type presenilins are necessary for proper protein degradation through the autophagosome-lysosome system by functioning at the lysosomal level. The role of presenilins in autophagy has many implications for its function in neurological diseases such as AD.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Alzheimer Disease / enzymology
  • Alzheimer Disease / metabolism
  • Alzheimer Disease / pathology
  • Amyloid Precursor Protein Secretases / antagonists & inhibitors
  • Amyloid Precursor Protein Secretases / physiology*
  • Animals
  • Autophagy / genetics*
  • Cell Line, Tumor
  • Cells, Cultured
  • Lysosomes / enzymology
  • Lysosomes / metabolism*
  • Lysosomes / pathology
  • Mice
  • Neurons / enzymology
  • Neurons / metabolism
  • Neurons / pathology
  • Presenilin-1 / deficiency
  • Presenilin-1 / physiology*
  • Presenilin-2 / deficiency
  • Presenilin-2 / physiology*
  • Proteins / metabolism

Substances

  • Presenilin-1
  • Presenilin-2
  • Proteins
  • Amyloid Precursor Protein Secretases