Kv7.1 (KvLQT1) is a potassium channel protein whose primary subunit in humans is encoded by the KCNQ1 gene. [5] . Its mutation causes Long QT syndrome, K v 7.1 is a voltage and lipid-gated potassium channel present in the cell membranes of cardiac tissue and in inner ear neurons among other tissues.

KVLQT1 has been established as the human chromosome 11-linked gene responsible for more than 50% of inherited long QT syndrome. Here we describe the cloning of a full-length KVLQT1 cDNA and its functional expression. KVLQT1 encodes a 676-amino acid polypeptide with structural characteristics similar to voltage-gated potassium channels.

1996年1月1日 · Here, positional cloning methods establish KVLQT1 as the chromosome 11-linked LQT1 gene responsible for the most common inherited cardiac arrhythmia. KVLQT1 is strongly expressed in the heart...

KvLQT1 is a gene associated with a form of inherited Long QT Syndrome (LQT1) that encodes a potassium channel protein with unique biophysical properties, potentially coassembling with other proteins to form a cardiac potassium channel.

KCNQ1 (also known as Kv7.1 or KvLQT1) is a widely expressed tetrameric voltage-gated potassium (Kv) channel that is involved in heartbeat and is associated with hundreds of mutations linked to deafness and cardiac arrhythmias [1,2].

1997年4月15日 · KVLQT1 has been established as the human chromosome 11-linked gene responsible for more than 50% of inherited long QT syndrome. Here we describe the cloning of a full-length KVLQT1 cDNA and its functional expression. KVLQT1 encodes a 676-amino acid polypeptide with structural characteristics similar to voltage-gated potassium channels.

1997年4月15日 · KVLQT1 has been established as the human chromosome 11-linked gene responsible for more than 50% of inherited long QT syndrome. Here we describe the cloning of a full-length KVLQT1 cDNA and its functional expression. KVLQT1 encodes a 676-amino acid polypeptide with structural characteristics similar to voltage-gated potassium channels.

1996年11月1日 · 在这里，我们展示了 kvlqt1 编码一个 k+ 通道，该通道具有与其他已知心脏电流不同的生物物理特性。 我们认为 KVLQT1 可能与另一个亚基共组装在心肌细胞中形成功能性通道。

KVLQT1 encodes a 676-amino acid polypeptide with struc-tural characteristics similar to voltage-gated potassium chan-nels. Expression of KvLQT1 in Xenopus oocytes and in human embryonic kidney cells elicits a rapidly activating, K+-selective outward current. The lKr-specific blockers, E-4031 and dofetilide, do not inhibit KvLQT1, whereas ...

2000年9月9日 · MinK, a 129 amino acid protein containing one transmembrane-spanning domain modulates KvLQT1, greatly slowing activation, increasing current amplitude, and removing inactivation. Using deletion and chimeric analysis, we have examined the structural determinants of MinK effects on gating modulation and subunit association.

2020年1月23日 · KCNQ1, also known as Kv7.1, is a voltage-dependent K + channel that regulates gastric acid secretion, salt and glucose homeostasis, and heart rhythm. Its functional properties are regulated in a tissue-specific manner through co …

2001年3月2日 · KvLQT1 is a Shaker-like voltage-gated potassium channel that when complexed with minK (KCNE1) produces the slowly activating delayed rectifier I ks. The emerging family of KCNE1-related peptides includes KCNE1 and KCNE3, both of which complex with KvLQT1 to produce functionally distinct currents.

We found that KVLQT1 is expressed in the stria vascularis of mouse inner ear by in situ hybridization. Taken together, our data indicate that KVLQT1 is responsible for both JLN and RW syndromes and has a key role not only in the ventricular repolarization but also in normal hearing, probably via the control of endolymph homeostasis.

LQT1-associated mutations in KVLQT1 caused a spectrum of dysfunction in I(Ks) and KvLQT1 channels. The degree of I(Ks) dysfunction did not correlate with the QTc interval or the presence of symptoms in the respective gene carriers. In contrast to previous reports, we found that loss of function muta …

1999年2月19日 · Abstract —The voltage-gated K + channel KVLQT1 is essential for the repolarization phase of the cardiac action potential and for K + homeostasis in the inner ear. Mutations in the human KCNQ1 gene encoding the α subunit of the KVLQT1 channel cause the long-QT syndrome (LQTS).

In this paper we have analyzed a peculiarity of the gating of homomeric KvLQT1 channels that is absent or strongly attenuated in KvLQT1/minK heteromers. Understanding how the association with minK modifies the gating characteristics of KvLQT1 is important to gaining insights into the functioning of this cardiac delayed rectifier K + channel.

1997年9月1日 · Mutations in the delayed rectifier K + channel subunit KvLQT1 have been identified as responsible for both Romano–Ward (RW) and Jervell and Lange‐Nielsen (JLN) inherited long QT syndromes. We report the molecular cloning of a human KvLQT1 isoform that is expressed in several human tissues including heart.

1997年7月4日 · Mutations in KvLQT1 are associated with a long QT syndrome that causes syncope and sudden death and also with deafness. Here, we show a new mode of association between ion channel forming subunits in that the cytoplasmic C-terminal end of MinK interacts directly with the pore region of KvLQT1.

2004年1月9日 · KvLQT1 was found to associate physically with HERG in both CHO cells and native cardiomyocytes and to increase membrane localization of HERG in CHO cells. KvLQT1-induced increases in HERG membrane expression were paralleled by increased I HERG density.

Co-expression of KvLQT1 with HERG in a mammalian expression system significantly accelerated HERG current deactivation at physiologically relevant potentials by increasing the contribution of the fast component (e.g.