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: PMID:10219239   (View Abstract at PubMed)

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.

Gene Ontology Annotations    Click to see Annotation Detail View

Biological Process

Cellular Component
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
voltage-gated potassium channel complex part_ofIDA 633041PMID:10219239BHF-UCL 

Molecular Function

Objects Annotated

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

Objects referenced in this article
Gene KCNE2 potassium voltage-gated channel subfamily E regulatory subunit 2 Homo sapiens
Gene KCNE3 potassium voltage-gated channel subfamily E regulatory subunit 3 Homo sapiens
Gene KCNE4 potassium voltage-gated channel subfamily E regulatory subunit 4 Homo sapiens
Gene Kcne3 potassium voltage-gated channel, Isk-related subfamily, gene 3 Mus musculus
Gene Kcne4 potassium voltage-gated channel, Isk-related subfamily, gene 4 Mus musculus

Additional Information