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Clinical Spectrum of KCNA1 Mutations: New Insights into Episodic Ataxia and Epilepsy Comorbidity
Review

Kv1.1 Channelopathies: Pathophysiological Mechanisms and Therapeutic Approaches

1
Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, Msida MDS-2080, Malta
2
Department of Pharmacy–Drug Sciences, University of Bari “Aldo Moro”, 70125 Bari, Italy
3
Electrophysiology Unit, Axxam SpA, Openzone, 20091 Bresso (Milan), Italy
4
Department of Physiology, College of Medicine and Health Sciences, United Arab Emirates University, Al Ain Po Box 17666, UAE
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2020, 21(8), 2935; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21082935
Received: 14 April 2020 / Revised: 19 April 2020 / Accepted: 20 April 2020 / Published: 22 April 2020
Kv1.1 belongs to the Shaker subfamily of voltage-gated potassium channels and acts as a critical regulator of neuronal excitability in the central and peripheral nervous systems. KCNA1 is the only gene that has been associated with episodic ataxia type 1 (EA1), an autosomal dominant disorder characterized by ataxia and myokymia and for which different and variable phenotypes have now been reported. The iterative characterization of channel defects at the molecular, network, and organismal levels contributed to elucidating the functional consequences of KCNA1 mutations and to demonstrate that ataxic attacks and neuromyotonia result from cerebellum and motor nerve alterations. Dysfunctions of the Kv1.1 channel have been also associated with epilepsy and kcna1 knock-out mouse is considered a model of sudden unexpected death in epilepsy. The tissue-specific association of Kv1.1 with other Kv1 members, auxiliary and interacting subunits amplifies Kv1.1 physiological roles and expands the pathogenesis of Kv1.1-associated diseases. In line with the current knowledge, Kv1.1 has been proposed as a novel and promising target for the treatment of brain disorders characterized by hyperexcitability, in the attempt to overcome limited response and side effects of available therapies. This review recounts past and current studies clarifying the roles of Kv1.1 in and beyond the nervous system and its contribution to EA1 and seizure susceptibility as well as its wide pharmacological potential. View Full-Text
Keywords: Kv1.1 potassium channel; episodic ataxia type 1; epilepsy; SUDEP; knock-out mouse; ataxic mouse; channel modulators; acetazolamide; sodium channel blockers Kv1.1 potassium channel; episodic ataxia type 1; epilepsy; SUDEP; knock-out mouse; ataxic mouse; channel modulators; acetazolamide; sodium channel blockers
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MDPI and ACS Style

D’Adamo, M.C.; Liantonio, A.; Rolland, J.-F.; Pessia, M.; Imbrici, P. Kv1.1 Channelopathies: Pathophysiological Mechanisms and Therapeutic Approaches. Int. J. Mol. Sci. 2020, 21, 2935. https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21082935

AMA Style

D’Adamo MC, Liantonio A, Rolland J-F, Pessia M, Imbrici P. Kv1.1 Channelopathies: Pathophysiological Mechanisms and Therapeutic Approaches. International Journal of Molecular Sciences. 2020; 21(8):2935. https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21082935

Chicago/Turabian Style

D’Adamo, Maria C., Antonella Liantonio, Jean-Francois Rolland, Mauro Pessia, and Paola Imbrici. 2020. "Kv1.1 Channelopathies: Pathophysiological Mechanisms and Therapeutic Approaches" International Journal of Molecular Sciences 21, no. 8: 2935. https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21082935

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