Voltage-gated inwardly rectifying potassium channel KCNH2

UniProt Q12809 Organism Homo sapiens Gene ERG, ERG1, HERG, KCNH2

Also known as: ERG, ERG1, HERG, KCNH2

Function

Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel. Channel properties are modulated by cAMP and subunit assembly. Characterized by unusual gating kinetics by producing relatively small outward currents during membrane depolarization and large inward currents during subsequent repolarization which reflect a rapid inactivation during depolarization and quick recovery from inactivation but slow deactivation (closing) during repolarization. Forms a stable complex with KCNE1 or KCNE2, and that this heteromultimerization regulates inward rectifier potassium channel activity.

Classification

Family (Pfam): PF00027 cNMP_binding, PF00520 Ion_trans, PF13426 PAS_9
InterPro: cNMP-bd_dom, cNMP-bd_dom_sf, Ion_trans_dom, K_chnl_volt-dep_EAG/ELK/ERG, K_chnl_volt-dep_ERG, KCNH_animal-type, PAC, PAS, PAS-assoc_C, PAS-like_dom_sf, RmlC-like_jellyroll
Functional cluster: Serine/Threonine Protein Kinases

Experimental structures · PDB · 24

1BYW X-ray 2.60A
1UJL NMR
2L0W NMR
2L1M NMR
2L4R NMR
2LE7 NMR
2N7G NMR
4HP9 X-ray 2.12A
4HQA X-ray 1.96A
5VA1 EM 3.70A
5VA2 EM 3.80A
5VA3 EM 4.00A
… and 12 more

A predicted model is available from AlphaFold.

Gene Ontology · 37

Disease associations

short QT syndrome type 1 MONDO:0012312
long QT syndrome 2 MONDO:0013367

Drugs targeting this protein · 13

Neighborhood · nearest proteins

Nearest neighbours of Voltage-gated inwardly rectifying potassium channel KCNH2: 3 by sequence/function similarity (left); 3 co-cited in the literature (right).

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Voltage-gated inwardly rectifying potassium channel KCNH2 2 shared papers
Potassium voltage-gated channel subfamily KQT member 1 10 shared papers
Potassium voltage-gated channel subfamily E member 1 5 shared papers
Sodium channel protein type 5 subunit alpha 6 shared papers
Voltage-gated delayed rectifier potassium channel KCNH1 1 shared papers
Potassium voltage-gated channel subfamily E member 2 3 shared papers
Calnexin 1 shared papers
Protein-cysteine N-palmitoyltransferase HHAT-like protein 1 shared papers
Potassium voltage-gated channel subfamily E member 2 1 shared papers
DnaJ homolog subfamily B member 1 1 shared papers
RING finger protein 207 1 shared papers
RING finger protein 207 1 shared papers

Literature · 56 cited papers

Tetrameric assembly of K(+) channels requires ER-located chaperone proteins. Mol. Cell · 2017
Regulation of the human ether-a-go-go-related gene (hERG) potassium channel by Nedd4 family interacting proteins (Ndfips). Biochem. J. · 2015
RING finger protein RNF207, a novel regulator of cardiac excitation. J. Biol. Chem. · 2014
Toward a comprehensive characterization of a human cancer cell phosphoproteome. J. Proteome Res. · 2013
The S4-S5 linker acts as a signal integrator for HERG K+ channel activation and deactivation gating. PLoS ONE · 2012
Identification and functional characterization of the novel human ether-a-go-go-related gene (hERG) R744P mutant associated with hereditary long QT syndrome 2. Biochem. Biophys. Res. Commun. · 2012
Spectrum and prevalence of mutations from the first 2,500 consecutive unrelated patients referred for the FAMILION long QT syndrome genetic test. Heart Rhythm · 2009
Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions. Sci. Signal. · 2009
A primate-specific, brain isoform of KCNH2 affects cortical physiology, cognition, neuronal repolarization and risk of schizophrenia. Nat. Medicines · 2009
Identification of a posttranslational mechanism for the regulation of hERG1 K+ channel expression and hERG1 current density in tumor cells. Mol. Cell. Biol. · 2008
Spectrum of pathogenic mutations and associated polymorphisms in a cohort of 44 unrelated patients with long QT syndrome. Clin. Genet. · 2006
Genetic testing in the long QT syndrome: development and validation of an efficient approach to genotyping in clinical practice. JAMA · 2005
… and 44 more in the literature graph

A document in the lmmol reference corpus — open public data (UniProt, GO, PDB, the literature graph) rendered as a single cross-linked page. Hover any link to preview its target.