Cyclophilin D deficiency rescues Aβ-impaired PKA/CREB signaling and alleviates synaptic degeneration

Biochim Biophys Acta. 2014 Dec;1842(12 Pt A):2517-27. doi: 10.1016/j.bbadis.2013.03.004. Epub 2013 Mar 16.

Abstract

The coexistence of neuronal mitochondrial pathology and synaptic dysfunction is an early pathological feature of Alzheimer's disease (AD). Cyclophilin D (CypD), an integral part of mitochondrial permeability transition pore (mPTP), is involved in amyloid beta (Aβ)-instigated mitochondrial dysfunction. Blockade of CypD prevents Aβ-induced mitochondrial malfunction and the consequent cognitive impairments. Here, we showed the elimination of reactive oxygen species (ROS) by antioxidants probucol or superoxide dismutase (SOD)/catalase blocks Aβ-mediated inactivation of protein kinase A (PKA)/cAMP regulatory-element-binding (CREB) signal transduction pathway and loss of synapse, suggesting the detrimental effects of oxidative stress on neuronal PKA/CREB activity. Notably, neurons lacking CypD significantly attenuate Aβ-induced ROS. Consequently, CypD-deficient neurons are resistant to Aβ-disrupted PKA/CREB signaling by increased PKA activity, phosphorylation of PKA catalytic subunit (PKA C), and CREB. In parallel, lack of CypD protects neurons from Aβ-induced loss of synapses and synaptic dysfunction. Furthermore, compared to the mAPP mice, CypD-deficient mAPP mice reveal less inactivation of PKA-CREB activity and increased synaptic density, attenuate abnormalities in dendritic spine maturation, and improve spontaneous synaptic activity. These findings provide new insights into a mechanism in the crosstalk between the CypD-dependent mitochondrial oxidative stress and signaling cascade, leading to synaptic injury, functioning through the PKA/CREB signal transduction pathway.

Keywords: Alzheimer's disease; Amyloid beta; Mitochondrial permeability transition; Oxidative stress; PKA/CREB signaling; Synaptic alteration.

Publication types

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

MeSH terms

  • Amyloid beta-Peptides / pharmacology*
  • Amyloid beta-Protein Precursor / genetics
  • Amyloid beta-Protein Precursor / metabolism
  • Animals
  • Antioxidants / pharmacology
  • Catalase / pharmacology
  • Cells, Cultured
  • Cyclic AMP Response Element-Binding Protein / metabolism*
  • Cyclic AMP-Dependent Protein Kinases / metabolism*
  • Cyclophilins / genetics
  • Cyclophilins / metabolism*
  • Immunoblotting
  • Mice, Knockout
  • Mice, Transgenic
  • Mitochondria / drug effects
  • Mitochondria / metabolism
  • Neurons / drug effects
  • Neurons / metabolism
  • Neurons / physiology
  • Oxidative Stress / drug effects
  • Patch-Clamp Techniques
  • Peptidyl-Prolyl Isomerase F
  • Probucol / pharmacology
  • Reactive Oxygen Species / metabolism
  • Signal Transduction / drug effects*
  • Superoxide Dismutase / pharmacology
  • Synaptic Transmission / drug effects*

Substances

  • Amyloid beta-Peptides
  • Amyloid beta-Protein Precursor
  • Antioxidants
  • Creb1 protein, mouse
  • Cyclic AMP Response Element-Binding Protein
  • Peptidyl-Prolyl Isomerase F
  • PPIF protein, mouse
  • Reactive Oxygen Species
  • Catalase
  • Superoxide Dismutase
  • Cyclic AMP-Dependent Protein Kinases
  • Cyclophilins
  • Probucol