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Kv11.1 the ether-a-go-go related gene is a pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel is associated with cardiac arrhythmias and rhythmic excitability of the pituitary. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr). The potassium channel is probably composed of a homo- or heterotetrameric complex of pore-forming alpha subunits that can associate with modulating beta subunits. Heteromultimer with Kv11.2 and Kv11.3. Interacts with ALG10B. Heteromultimer with KCNE1 and KCNE2. Defects in Kv11.1 are the cause of long QT syndrome type 2 (LQT2), a heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress. Deafness is often associated with LQT2. Defects in Kv11.1 are the cause of short QT syndrome type 1 (SQT1), a heart disorder characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. They cause syncope and sudden death. Four isoforms of the human protein are produced by alternative splicing. Isoform 3 has no channel activity by itself, but modulates channel characteristics when associated with isoform 1. Note: This description may include information from UniProtKB.
Protein type: Channel, potassium; Membrane protein, integral; Membrane protein, multi-pass; Motility/polarity/chemotaxis
Chromosomal Location of Human Ortholog: 7q36.1
Cellular Component: cell surface; perinuclear region of cytoplasm; plasma membrane; voltage-gated potassium channel complex
Molecular Function: delayed rectifier potassium channel activity; identical protein binding; inward rectifier potassium channel activity; phosphorelay sensor kinase activity; protein binding; protein homodimerization activity; ubiquitin protein ligase binding; voltage-gated potassium channel activity
Biological Process: cardiac muscle contraction; potassium ion homeostasis; regulation of membrane potential; regulation of the rate of heart contraction by hormone; two-component signal transduction system (phosphorelay)
Disease: Long Qt Syndrome 2; Short Qt Syndrome 1
Reference #:  Q12809 (UniProtKB)
Alt. Names/Synonyms: Eag homolog; Eag-related protein 1; ERG; ERG-1; ERG1; Ether-a-go-go-related gene potassium channel 1; ether-a-go-go-related potassium channel protein; Ether-a-go-go-related protein 1; H-ERG; HERG; hERG-1; hERG1; KCNH2; Kv11.1; LQT2; Potassium voltage-gated channel subfamily H member 2; potassium voltage-gated channel, subfamily H (eag-related), member 2; SQT1; Voltage-gated potassium channel subunit Kv11.1; voltage-gated potassium channel, subfamily H, member 2
Gene Symbols: KCNH2
Molecular weight: 126,655 Da
Basal Isoelectric point: 8.2  Predict pI for various phosphorylation states
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