Rabbit anti-Human KCNH2 Polyclonal Antibody

Uniprot Id

Q12809

Target Species

Human

Target Name

KCNH2

Target Full Name

Voltage-gated inwardly rectifying potassium channel KCNH2

Target Function

Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr).; Has no channel activity by itself, but modulates channel characteristics by forming heterotetramers with other isoforms which are retained intracellularly and undergo ubiquitin-dependent degradation.; Has no channel activity by itself, but modulates channel characteristics by forming heterotetramers with other isoforms which are retained intracellularly and undergo ubiquitin-dependent degradation.

Target Involvement

Long QT syndrome 2 (LQT2); Short QT syndrome 1 (SQT1)

Target Subcellular Location

Cell membrane; Multi-pass membrane protein.

Target Protein Families

Potassium channel family, H (Eag) (TC 1.A.1.20) subfamily, Kv11.1/KCNH2 sub-subfamily

Target Tissue Specificity

Highly expressed in heart and brain. Isoforms USO are frequently overexpressed in cancer cells.

Target Synonyms

ERG1; HERG; LQT2; SQT1; ERG-1; H-ERG; HERG1; Kv11.1; KCNH2

Target Background

This gene encodes a component of a voltage-activated potassium channel found in cardiac muscle, nerve cells, and microglia. Four copies of this protein interact with one copy of the KCNE2 protein to form a functional potassium channel. Mutations in this gene can cause long QT syndrome type 2 (LQT2). Transcript variants encoding distinct isoforms have been identified.