hERG

hERG (the human Ether-à-go-go-Related Gene) is a gene (KCNH2) that codes for a protein known as Kv11.1, the alpha subunit of a potassium ion channel. This ion channel (sometimes simply denoted as 'hERG') is best known for its contribution to the electrical activity of the heart: the hERG channel mediates the repolarizing IKr current in the cardiac action potential, which helps coordinate the heart's beating.

KCNH2
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesKCNH2, ERG-1, ERG1, H-ERG, HERG, HERG1, Kv11.1, LQT2, SQT1, potassium voltage-gated channel subfamily H member 2
External IDsOMIM: 152427 MGI: 1341722 HomoloGene: 201 GeneCards: KCNH2
Orthologs
SpeciesHumanMouse
Entrez

3757

16511

Ensembl

ENSG00000055118

ENSMUSG00000038319

UniProt

Q12809

O35219

RefSeq (mRNA)

NM_000238
NM_001204798
NM_172056
NM_172057

NM_001294162
NM_013569

RefSeq (protein)

NP_000229
NP_001191727
NP_742053
NP_742054

NP_001281091
NP_038597

Location (UCSC)Chr 7: 150.94 – 150.98 MbChr 5: 24.52 – 24.56 Mb
PubMed search
Wikidata
View/Edit HumanView/Edit Mouse

When this channel's ability to conduct electrical current across the cell membrane is inhibited or compromised, either by application of drugs or by rare mutations in some families, it can result in a potentially fatal disorder called long QT syndrome. Conversely, genetic mutations that increase the current through these channels can lead to the related inherited heart rhythm disorder Short QT syndrome. A number of clinically successful drugs in the market have had the tendency to inhibit hERG, lengthening the QT and potentially leading to a fatal irregularity of the heartbeat (a ventricular tachyarrhythmia called torsades de pointes). This has made hERG inhibition an important antitarget that must be avoided during drug development.

hERG has also been associated with modulating the functions of some cells of the nervous system and with establishing and maintaining cancer-like features in leukemic cells.

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