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Dev Dyn. 2019 Sep 11. doi: 10.1002/dvdy.114. [Epub ahead of print]

Kv2.1 voltage-gated potassium channels in developmental perspective.

Author information

1
International Institute of Molecular and Cell Biology in Warsaw, Warsaw, Poland.
2
Postgraduate School of Molecular Medicine, Warsaw Medical University, Warsaw, Poland.

Abstract

Kv2.1 voltage-gated potassium channels consist of two types of α-subunits: (a) electrically-active Kcnb1 α-subunits and (b) silent or modulatory α-subunits plus β-subunits that, similar to silent α-subunits, also regulate electrically-active subunits. Voltage-gated potassium channels were traditionally viewed, mainly by electrophysiologists, as regulators of the electrical activity of the plasma membrane in excitable cells, a role that is performed by transmembrane protein domains of α-subunits that form the electric pore. Genetic studies revealed a role for this region of α-subunits of voltage-gated potassium channels in human neurodevelopmental disorders, such as epileptic encephalopathy. The N- and C-terminal domains of α-subunits interact to form the cytoplasmic subunit of heterotetrameric potassium channels that regulate electric pores. Subsequent animal studies revealed the developmental functions of Kcnb1-containing voltage-gated potassium channels and illustrated their role during brain development and reproduction. These functions of potassium channels are discussed in this review in the context of regulatory interactions between electrically-active and regulatory subunits.

KEYWORDS:

Kcnb1; Kcng4; N- and C-terminals; brain ventricular system; hydrocephalus; microcephaly; neurodevelopmental diseases; zebrafish

PMID:
31512327
DOI:
10.1002/dvdy.114

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