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potassium voltage-gated channel, shaker-related subfamily, member 1 (episodic ataxia with myokymia)
Symbol KCNA1
Alt. Symbols Kv1.1, RBK1, HUK1, MBK1, AEMK
Entrez 3736
HUGO 6218
OMIM 176260
RefSeq NM_000217
UniProt Q09470
Other data
Locus Chr. 12 p13

Kv1.1 is a shaker related voltage-gated potassium channel encoded by the KCNA1 gene.[1][2][3] The Isaacs syndrome is a result of an autoimmune reaction against the Kv1.1 ion channel .[4]


  1. Curran ME, Landes GM, Keating MT (1992). "Molecular cloning, characterization, and genomic localization of a human potassium channel gene". Genomics. 12 (4): 729–37. PMID 1349297. doi:10.1016/0888-7543(92)90302-9 . 
  2. Albrecht B, Weber K, Pongs O (1995). "Characterization of a voltage-activated K-channel gene cluster on human chromosome 12p13". Recept. Channels. 3 (3): 213–20. PMID 8821794. 
  3. Gutman GA, Chandy KG, Grissmer S, Lazdunski M, McKinnon D, Pardo LA, Robertson GA, Rudy B, Sanguinetti MC, Stühmer W, Wang X (2005). "International Union of Pharmacology. LIII. Nomenclature and molecular relationships of voltage-gated potassium channels". Pharmacol. Rev. 57 (4): 473–508. PMID 16382104. doi:10.1124/pr.57.4.10. 
  4. Newsom-Davis J (1997). "Autoimmune neuromyotonia (Isaacs' syndrome): an antibody-mediated potassium channelopathy". Ann. N. Y. Acad. Sci. 835: 111–9. PMID 9616766. 

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