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Calmodulin-dependent Signaling01:16

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Calmodulin (CaM) is a calcium-binding protein in eukaryotes that controls various calcium-regulated cellular processes. It has four calcium-binding sites that bind calcium to form the calcium-calmodulin ( Ca2+-CaM) complex. GPCR stimulation increases the calcium levels in the cells that bind to CaM and induces a conformational change.
The Ca2+-CaM complex does not have enzymatic activity by itself. Instead, the complex binds downstream target proteins, including membrane proteins or enzymes,...
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Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons
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Calcineurin Activity Is Increased in Charcot-Marie-Tooth 1B Demyelinating Neuropathy.

Mariapaola Sidoli1,2,3, Chelsey B Reed4,5, Cristina Scapin6

  • 1Hunter James Kelly Research Institute, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York 14203 mpsidoli@stanford.edu mlfeltri@buffalo.edu.

The Journal of Neuroscience : the Official Journal of the Society for Neuroscience
|April 21, 2021
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Summary

This study reveals calcineurin

Keywords:
Charcot-Marie-ToothPERKSchwann cellscalcineurindemyelinationmyelin

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Area of Science:

  • Neuroscience
  • Cell Biology
  • Biochemistry

Background:

  • Schwann cells synthesize myelin in the peripheral nervous system (PNS).
  • Misfolded P0 protein (P0S63del) causes Charcot-Marie-Tooth type 1B neuropathy by accumulating in the ER and activating the unfolded protein response (UPR).
  • The UPR transducer PERK normally reduces ER stress, but its ablation paradoxically improved myelin defects in P0S63del models.

Purpose of the Study:

  • To investigate the interaction between PERK and the calcineurin/NFATc4 pathway in Schwann cells under ER stress.
  • To determine the role of calcineurin in P0S63del-induced neuropathy.

Main Methods:

  • Utilized mouse genetics, including both female and male subjects.
  • Examined protein interactions between PERK and calcineurin in P0S63del nerves.
  • Assessed calcineurin activity and NFATc4 nuclear localization in Schwann cells.
  • Analyzed EGR2/KROX20 expression levels.

Main Results:

  • PERK and calcineurin interact in P0S63del nerves.
  • Calcineurin activity and NFATc4 nuclear localization were elevated in S63del Schwann cells, independent of EGR2/KROX20.
  • Genetic modulation of calcineurin subunits had variable effects, indicating Schwann cell sensitivity to its activity levels.

Conclusions:

  • Calcineurin plays a novel, potentially protective role in Schwann cells during ER stress and demyelinating diseases like Charcot-Marie-Tooth 1B.
  • Schwann cells exhibit high sensitivity to alterations in calcineurin activity.
  • This research uncovers a new function for calcineurin in the context of demyelinating neuropathies.