KCNE1

(redirected from JLNS)
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KCNE1

Notation for a voltage-gated potassium-channel gene.

KCNE1

A gene on chromosome 21q22.12 that encodes a member of the KCNE K+ channel family, which are essential to many cellular functions; they are protean in their electrophysiologic and pharmacologic properties. The KCNE1 protein product associates with the KVLQT1 protein product forming the delayed rectifier potassium channel.

Molecular pathology
KVLQT1 mutations are associated with Jervell and Lange-Nielsen and Romano-Ward forms of long-QT syndrome.
References in periodicals archive ?
Hearing loss in JLNS may be treated successfully with cochlear implantation (CI), an intervention that does not interfere with bipolar pacemakers.
In our study, Five of the patients with JLNS had non-stress induced syncope attacks.
It is interesting that, in general, heterozygous mutations in KVLQT1 cause RWS (LQTS only), while homozygous mutations in KVLQT1 cause JLNS (LQTS and deafness).