HERG

HERG (Human Ether-a-go-go-Related Gene) is a gene that encodes a protein known as Kv11.1, the alpha subunit of a potassium ion channel. This ion channel (sometimes referred to as 'HERG') is best known for its contribution to the electrical activity of the heart that coordinates the heart's beating (cardiac rhythm).

Function

The HERG gene encodes a protein that is a member of the potassium channel, voltage-gated, subfamily H. This protein is an integral membrane protein and a voltage-gated potassium channel subunit. Mutations in this gene have been associated with hereditary long QT syndrome, also known as an abnormal heart rhythm disorder (arrhythmia).

Clinical significance

Mutations in HERG can lead to defects in the protein's structure and function, which in turn can cause a decrease in the flow of potassium ions. This can lead to a type of disorder known as Long QT syndrome (LQTS). LQTS from HERG mutations is specifically classified as LQT2. LQT2 is one of the most common forms of inherited LQTS.

Drug-induced Long QT syndrome

Many drugs, for example some for arrhythmia, antidepressants, antibiotics, and antihistamines, can interfere with the HERG potassium channel, leading to a reduction in the repolarizing current leading to a prolongation of the QT interval and a risk for torsade de pointes.

See also

• Long QT syndrome
• Potassium channel
• Cardiac rhythm
• Arrhythmia

External links

• WikiMD

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