RGD Reference Report - MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmia. - Rat Genome Database

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MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmia.

Authors: Abbott, GW  Sesti, F  Splawski, I  Buck, ME  Lehmann, MH  Timothy, KW  Keating, MT  Goldstein, SA 
Citation: Abbott GW, etal., Cell 1999 Apr 16;97(2):175-87.
RGD ID: 633041
Pubmed: (View Article at PubMed) PMID:10219239

A novel potassium channel gene has been cloned, characterized, and associated with cardiac arrhythmia. The gene encodes MinK-related peptide 1 (MiRP1), a small integral membrane subunit that assembles with HERG, a pore-forming protein, to alter its function. Unlike channels formed only with HERG, mixed complexes resemble native cardiac IKr channels in their gating, unitary conductance, regulation by potassium, and distinctive biphasic inhibition by the class III antiarrhythmic E-4031. Three missense mutations associated with long QT syndrome and ventricular fibrillation are identified in the gene for MiRP1. Mutants form channels that open slowly and close rapidly, thereby diminishing potassium currents. One variant, associated with clarithromycin-induced arrhythmia, increases channel blockade by the antibiotic. A mechanism for acquired arrhythmia is revealed: genetically based reduction in potassium currents that remains clinically silent until combined with additional stressors.

Annotation

Objects Annotated

Genes (Rattus norvegicus)
Kcne2  (potassium voltage-gated channel subfamily E regulatory subunit 2)

Objects referenced in this article
0 KCNE2 potassium voltage-gated channel subfamily E regulatory subunit 2 All species
0 Kcne3 potassium voltage-gated channel, Isk-related subfamily, gene 3 All species
0 KCNE3 potassium voltage-gated channel subfamily E regulatory subunit 3 All species
0 KCNE4 potassium voltage-gated channel subfamily E regulatory subunit 4 All species
0 Kcne4 potassium voltage-gated channel, Isk-related subfamily, gene 4 All species

Additional Information