Eag domain-CNBHD complex of the mouse EAG1

Introduction:

The KCNH voltage-gated channels are transmembrane channels highly selective for potassium and sensitive to voltage changes in the cells’ membrane potential.

They play a crucial roles in repolarization of cells to their resting state and in various diseases such as cardiac long QT syndrome type 2 (LQT2)[1], epilepsy[2], schizophrenia[3] and cancer[4]. The KCNH channels family has unique intracellular domains that distinguish them from other voltage-gated channels. The amino terminal contains an eag domain and the C-terminus contains a cyclic nucleotide binding homology domain (CNBDH).


Reference:

  1. Sanguinetti, M. C. & Tristani-Firouzi, M. hERG potassium channels and cardiac arrhythmia. Nature 440, 463–469 (2006).
  2. Zhang, X. et al. Deletion of the potassium channel Kv12.2 causes hippocampal hyperexcitability and epilepsy. Nature Neurosci. 13, 1056–1058 (2010).
  3. Huffaker, S. J. et al. A primate-specific, brain isoform of KCNH2 affects cortical physiology, cognition, neuronal repolarization and risk of schizophrenia. Nature Med. 15, 509–518 (2009).
  4. Camacho, J. Ether a` go-go potassium channels and cancer. Cancer Lett. 233, 1–9 (2006).

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