Andersen-Tawil Syndrome (ATS)
What is Andersen-Tawil Syndrome?
Andersen-Tawil syndrome (ATS) is a
rare disorder which is estimated to affect 1 in one million people.
ATS has three distinct characteristics, episodes of muscle weakness
(periodic paralysis), changes in heart rhythm (arrhythmia), and
developmental abnormalities. The most common changes affecting the
heart are ventricular arrhythmia, which is a disruption in the
rhythm of the heart's lower chambers, and long QT
The condition is caused by a lack
of control over the channel which allows entry and exit of
potassium ions into cells. The specific name for this is a voltage
gated, inward rectifying potassium channel or Kir2.1 channel. These
are found predominantly in skeletal muscle, heart and brain cells.
The incorrectly functioning channels 'leak' or fail to bind with
another protein which regulates its activity this means that their
electrical charge is not controlled properly. These cells lose
their charge and take a long time to get it back. The individual
experiences this as muscle weakness or paralysis. For more
information about Anderson-Tawil Syndrome click here.
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This page was reviewed 19 Feb 2016