The mammalian KIR2.x inward rectifier ion channel family: expression pattern and pathophysiology

• Published in: Acta Physiologica Vol. 199; no. 3; pp. 243 - 256
• Main Authors: De Boer, T. P., Houtman, M. J. C., Compier, M., Van Der Heyden, M. A. G.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.07.2010; Wiley-Blackwell
• Subjects: Andersen-Tawil syndrome; barium; Biological and medical sciences; expression pattern; Fundamental and applied biological sciences. Psychology; inward rectifier current; KIR2.x; thyrotoxic periodic paralysis; Vertebrates: anatomy and physiology, studies on body, several organs or systems; Barium; expression pattern; Pathophysiology; Ionic channel; K; Inflammatory disease; Vertebrata; Mammalia; thyrotoxic periodic paralysis; Periodic paralysis; Andersen-Tawil syndrome; 2.x; Inward current; inward rectifier current
• ISSN: 1748-1708; 1748-1716
• DOI: 10.1111/j.1748-1716.2010.02108.x

Inward rectifier currents based on KIR2.x subunits are regarded as essential components for establishing a stable and negative resting membrane potential in many excitable cell types. Pharmacological inhibition, null mutation in mice and dominant positive and negative mutations in patients reveal some of the important functions of these channels in their native tissues. Here we review the complex mammalian expression pattern of KIR2.x subunits and relate these to the outcomes of functional inhibition of the resultant channels. Correlations between expression and function in muscle and bone tissue are observed, while we recognize a discrepancy between neuronal expression and function.