The neuroendocrine protein 7B2 suppresses the aggregation of neurodegenerative disease-related proteins

J Biol Chem. 2013 Jan 11;288(2):1114-24. doi: 10.1074/jbc.M112.417071. Epub 2012 Nov 21.

Abstract

Neurodegenerative diseases such as Alzheimer (AD) and Parkinson (PD) are characterized by abnormal aggregation of misfolded β-sheet-rich proteins, including amyloid-β (Aβ)-derived peptides and tau in AD and α-synuclein in PD. Correct folding and assembly of these proteins are controlled by ubiquitously expressed molecular chaperones; however, our understanding of neuron-specific chaperones and their involvement in the pathogenesis of neurodegenerative diseases is limited. We here describe novel chaperone-like functions for the secretory protein 7B2, which is widely expressed in neuronal and endocrine tissues. In in vitro experiments, 7B2 efficiently prevented fibrillation and formation of Aβ(1-42), Aβ(1-40), and α-synuclein aggregates at a molar ratio of 1:10. In cell culture experiments, inclusion of recombinant 7B2, either in the medium of Neuro-2A cells or intracellularly via adenoviral 7B2 overexpression, blocked the neurocytotoxic effect of Aβ(1-42) and significantly increased cell viability. Conversely, knockdown of 7B2 by RNAi increased Aβ(1-42)-induced cytotoxicity. In the brains of APP/PSEN1 mice, a model of AD amyloidosis, immunoreactive 7B2 co-localized with aggregation-prone proteins and their respective aggregates. Furthermore, in the hippocampus and substantia nigra of human AD- and PD-affected brains, 7B2 was highly co-localized with Aβ plaques and α-synuclein deposits, strongly suggesting physiological association. Our data provide insight into novel functions of 7B2 and establish this neural protein as an anti-aggregation chaperone associated with neurodegenerative disease.

Publication types

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

MeSH terms

  • Aged
  • Amino Acid Sequence
  • Amyloid beta-Peptides / antagonists & inhibitors*
  • Amyloid beta-Peptides / metabolism
  • Animals
  • Cell Death / physiology
  • Female
  • Hippocampus / metabolism
  • Humans
  • Immunohistochemistry
  • Mice
  • Mice, Transgenic
  • Microscopy, Electron, Transmission
  • Molecular Sequence Data
  • Neurodegenerative Diseases / metabolism*
  • Neuroendocrine Secretory Protein 7B2 / chemistry
  • Neuroendocrine Secretory Protein 7B2 / metabolism
  • Neuroendocrine Secretory Protein 7B2 / physiology*
  • Substantia Nigra / metabolism
  • alpha-Synuclein / antagonists & inhibitors*
  • alpha-Synuclein / metabolism

Substances

  • Amyloid beta-Peptides
  • Neuroendocrine Secretory Protein 7B2
  • SCG5 protein, human
  • alpha-Synuclein