Upregulation of the Sarco-Endoplasmic Reticulum Calcium ATPase 1 Truncated Isoform Plays a Pathogenic Role in Alzheimer's Disease

Cells. 2019 Nov 28;8(12):1539. doi: 10.3390/cells8121539.

Abstract

Dysregulation of the Endoplasmic Reticulum (ER) Ca2+ homeostasis and subsequent ER stress activation occur in Alzheimer Disease (AD). We studied the contribution of the human truncated isoform of the sarco-endoplasmic reticulum Ca2+ ATPase 1 (S1T) to AD. We examined S1T expression in human AD-affected brains and its functional consequences in cellular and transgenic mice AD models. S1T expression is increased in sporadic AD brains and correlates with amyloid β (Aβ) and ER stress chaperone protein levels. Increased S1T expression was also observed in human neuroblastoma cells expressing Swedish-mutated β-amyloid precursor protein (βAPP) or treated with Aβ oligomers. Lentiviral overexpression of S1T enhances in return the production of APP C-terminal fragments and Aβ through specific increases of β-secretase expression and activity, and triggers neuroinflammation. We describe a molecular interplay between S1T-dependent ER Ca2+ leak, ER stress and βAPP-derived fragments that could contribute to AD setting and/or progression.

Keywords: Alzheimer disease; BACE1; C83; C99; amyloid precursor protein; amyloid β; endoplasmic reticulum stress; neuroinflammation; truncated isoform of the sarco-endoplasmic reticulum Ca2+ ATPase 1 (S1T).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Alzheimer Disease / genetics*
  • Alzheimer Disease / metabolism*
  • Alzheimer Disease / pathology
  • Amyloid Precursor Protein Secretases / metabolism
  • Amyloid beta-Peptides / metabolism
  • Amyloid beta-Protein Precursor / metabolism
  • Animals
  • Biomarkers
  • Brain / metabolism
  • Brain / pathology
  • Cell Line
  • Disease Models, Animal
  • Disease Susceptibility*
  • Endoplasmic Reticulum Stress
  • Female
  • Gene Expression Regulation*
  • Humans
  • Immunohistochemistry
  • Inflammation Mediators / metabolism
  • Isoenzymes
  • Male
  • Mice
  • Mice, Transgenic
  • Middle Aged
  • Models, Biological
  • Protein Aggregation, Pathological
  • Sarcoplasmic Reticulum Calcium-Transporting ATPases / genetics*
  • Sarcoplasmic Reticulum Calcium-Transporting ATPases / metabolism
  • Signal Transduction

Substances

  • Amyloid beta-Peptides
  • Amyloid beta-Protein Precursor
  • Biomarkers
  • Inflammation Mediators
  • Isoenzymes
  • Amyloid Precursor Protein Secretases
  • Sarcoplasmic Reticulum Calcium-Transporting ATPases