Channelopathies comprise various diseases caused by defects of ion channels. Modifications of their biophysical properties are common and have been widely studied. However, ion channels are heterogeneous multi-molecular complexes that are extensively modulated and undergo a maturation process comprising numerous steps of structural modifications and intracellular trafficking. Perturbations of these processes can give rise to aberrant channels that cause pathologies. Here we review channelopathies of the nervous system associated with dysfunctions at the post-translational level (folding, trafficking, degradation, subcellular localization, interactions with associated proteins and structural post-translational modifications). We briefly outline the physiology of ion channels' maturation and discuss examples of defective mechanisms, focusing in particular on voltage-gated sodium channels, which are implicated in numerous neurological disorders. We also shortly introduce possible strategies to develop therapeutic approaches that target these processes. This article is part of the Special Issue entitled 'Channelopathies.'
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http://dx.doi.org/10.1016/j.neuropharm.2017.05.028 | DOI Listing |
Int J Mol Sci
December 2024
Laboratory of Neurobiology, Department of Neurology, Poznan University of Medical Sciences, 60-355 Poznan, Poland.
Calcium ions play a key role in the physiological processes of the central nervous system. The intracellular calcium signal, in nerve cells, is part of the neurotransmission mechanism. They are responsible for stabilizing membrane potential and controlling the excitability of neurons.
View Article and Find Full Text PDFJ Clin Exp Cardiolog
March 2024
Division of Thoracic Surgery, Johns Hopkins University School of Medicine, Baltimore, USA.
J Affect Disord
December 2024
The Affiliated Brain Hospital, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou Medical University, Guangzhou 510370, China. Electronic address:
Int Rev Neurobiol
November 2024
Department of Neurology, Mental Health and Neuroscience Research Institute, Maastricht University Medical Center+, Maastricht, Netherlands.
Small fiber neuropathy (SFN) is a condition involving the small nerve fibers of the peripheral nervous system, specifically the thinly myelinated Aδ and unmyelinated C fibers. It is an increasingly acknowledged condition within the spectrum of neuropathic pain disorders, leading to a rise in diagnosed patients. SFN is characterized by neuropathic pain, that is often described as burning, and typically presents in the hands and feet ascending proximally.
View Article and Find Full Text PDFEpidemiol Psychiatr Sci
November 2024
Department of Social Psychiatry, the Affiliated Brain Hospital, Guangzhou Medical University, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou Medical University, Guangzhou, China.
Aims: Psychotic-like experiences (PLEs), especially for persistent PLEs, are highly predictive of subsequent mental health problems. Hence, it is crucial to explore the psychopathological associations underlying the occurrence and persistence of PLEs. This study aimed to explore the above issues through a longitudinal dynamic network approach among PLEs and psychological and psychosocial factors.
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