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- Title
Kv2.1 voltage‐gated potassium channels in developmental perspective.
- Authors
Jędrychowska, Justyna; Korzh, Vladimir
- 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. Key Findings: Genetic studies revealed a number of relatively weak human mutations in the voltage‐gated potassium channel Kv2.1 linked to epileptic encephalopathy.Experimental analysis in model animals such as the zebrafish demonstrated that the much more drastic change in the activity of Kv2.1/Kcnb1 results in microcephaly or hydrocephalus.
- Subjects
POTASSIUM channels; MEMBRANE proteins; PROTEIN domains; CELL membranes; NEURAL development
- Publication
Developmental Dynamics, 2019, Vol 248, Issue 12, p1180
- ISSN
1058-8388
- Publication type
Article
- DOI
10.1002/dvdy.114