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First published online June 23, 2005

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Heartfelt trafficking defects

Human long QT syndrome is a cardiac disorder that is characterised by ventricular arrhythmias and sudden death. One form of this inherited disease, LQT2, is caused by loss-of-function of the human ether-a-go-go-related gene, HERG, which encodes a cyclic-nucleotide-binding domain (CNBD)-containing potassium channel. On p. 2803, Alvin Shrier and co-authors report that, for those LQT2 mutations that lie within the CNBD, HERG loss-of-function is due to abnormal retention of the ion channel in the endoplasmic reticulum – even though it appears to assemble correctly. They also show that removal of the entire CNBD of HERG or of any of its structural motifs prevents its Golgi transit, surface localization and function. Finally, they reveal that complete deletion of the CNBD or mutations at highly conserved CNBD residues prevent Golgi transit in two other CNBD-containing ion channels. Thus, the authors propose, disruption of the CNBD may critically affect the intracellular trafficking of many CNBD-containing ion channels and could underlie several inherited channel disorders.

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Identification of the cyclic-nucleotide-binding domain as a conserved determinant of ion-channel cell-surface localization

Armin Akhavan, Roxana Atanasiu, Tomohiro Noguchi, Wei Han, Natasha Holder, and Alvin Shrier
JCS 2005 118: 2803-2812. [Abstract] [Full Text]  

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in JCS Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Social Bookmarking                         What's this?