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Intracellular domains interactions and gated motions of I(KS) potassium channel subunits.
Haitin Y
,
Wiener R
,
Shaham D
,
Peretz A
,
Cohen EB
,
Shamgar L
,
Pongs O
,
Hirsch JA
,
Attali B
.
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Voltage-gated K(+) channels co-assemble with auxiliary beta subunits to form macromolecular complexes. In heart, assembly of Kv7.1 pore-forming subunits with KCNE1 beta subunits generates the repolarizing K(+) current I(KS). However, the detailed nature of their interface remains unknown. Mutations in either Kv7.1 or KCNE1 produce the life-threatening long or short QT syndromes. Here, we studied the interactions and voltage-dependent motions of I(KS) channel intracellular domains, using fluorescence resonance energy transfer combined with voltage-clamp recording and in vitro binding of purified proteins. The results indicate that the KCNE1distal C-terminus interacts with the coiled-coil helix C of the Kv7.1 tetramerization domain. This association is important for I(KS) channel assembly rules as underscored by Kv7.1 current inhibition produced by a dominant-negative C-terminal domain. On channel opening, the C-termini of Kv7.1 and KCNE1 come close together. Co-expression of Kv7.1 with the KCNE1 long QT mutant D76N abolished the K(+) currents and gated motions. Thus, during channel gating KCNE1 is not static. Instead, the C-termini of both subunits experience molecular motions, which are disrupted by the D76N causing disease mutation.
Abbott,
A superfamily of small potassium channel subunits: form and function of the MinK-related peptides (MiRPs).
1998, Pubmed
Abbott,
A superfamily of small potassium channel subunits: form and function of the MinK-related peptides (MiRPs).
1998,
Pubmed
Abitbol,
Stilbenes and fenamates rescue the loss of I(KS) channel function induced by an LQT5 mutation and other IsK mutants.
1999,
Pubmed
,
Xenbase
Barhanin,
K(V)LQT1 and lsK (minK) proteins associate to form the I(Ks) cardiac potassium current.
1996,
Pubmed
,
Xenbase
Ben-Efraim,
Cytoplasmic and extracellular IsK peptides activate endogenous K+ and Cl- channels in Xenopus oocytes. Evidence for regulatory function.
1996,
Pubmed
,
Xenbase
Bianchi,
Cellular dysfunction of LQT5-minK mutants: abnormalities of IKs, IKr and trafficking in long QT syndrome.
1999,
Pubmed
,
Xenbase
Bykova,
Large movement in the C terminus of CLC-0 chloride channel during slow gating.
2006,
Pubmed
Chandrasekhar,
KCNE1 subunits require co-assembly with K+ channels for efficient trafficking and cell surface expression.
2006,
Pubmed
Chen,
Charybdotoxin binding in the I(Ks) pore demonstrates two MinK subunits in each channel complex.
2003,
Pubmed
,
Xenbase
Cushman,
Voltage dependent activation of potassium channels is coupled to T1 domain structure.
2000,
Pubmed
Etxeberria,
Calmodulin regulates the trafficking of KCNQ2 potassium channels.
2008,
Pubmed
Gamper,
Structural requirements for differential sensitivity of KCNQ K+ channels to modulation by Ca2+/calmodulin.
2005,
Pubmed
Gamper,
Calmodulin mediates Ca2+-dependent modulation of M-type K+ channels.
2003,
Pubmed
Ghosh,
KCNQ1 assembly and function is blocked by long-QT syndrome mutations that disrupt interaction with calmodulin.
2006,
Pubmed
,
Xenbase
Gibor,
External barium affects the gating of KCNQ1 potassium channels and produces a pore block via two discrete sites.
2004,
Pubmed
,
Xenbase
Gulbis,
Structure of the cytoplasmic beta subunit-T1 assembly of voltage-dependent K+ channels.
2000,
Pubmed
,
Xenbase
Haitin,
The C-terminus of Kv7 channels: a multifunctional module.
2008,
Pubmed
Howard,
Structural insight into KCNQ (Kv7) channel assembly and channelopathy.
2007,
Pubmed
Kang,
Structure of KCNE1 and implications for how it modulates the KCNQ1 potassium channel.
2008,
Pubmed
Krumerman,
An LQT mutant minK alters KvLQT1 trafficking.
2004,
Pubmed
Levitan,
Signaling protein complexes associated with neuronal ion channels.
2006,
Pubmed
Li,
Specification of subunit assembly by the hydrophilic amino-terminal domain of the Shaker potassium channel.
1992,
Pubmed
Loussouarn,
Phosphatidylinositol-4,5-bisphosphate, PIP2, controls KCNQ1/KCNE1 voltage-gated potassium channels: a functional homology between voltage-gated and inward rectifier K+ channels.
2003,
Pubmed
Magidovich,
Intrinsic disorder in the C-terminal domain of the Shaker voltage-activated K+ channel modulates its interaction with scaffold proteins.
2007,
Pubmed
Marx,
Requirement of a macromolecular signaling complex for beta adrenergic receptor modulation of the KCNQ1-KCNE1 potassium channel.
2002,
Pubmed
Melman,
Structural determinants of KvLQT1 control by the KCNE family of proteins.
2001,
Pubmed
Melman,
KCNE regulation of KvLQT1 channels: structure-function correlates.
2002,
Pubmed
Minor,
The polar T1 interface is linked to conformational changes that open the voltage-gated potassium channel.
2000,
Pubmed
,
Xenbase
Misonou,
Kv2.1: a voltage-gated k+ channel critical to dynamic control of neuronal excitability.
2005,
Pubmed
Morin,
Counting membrane-embedded KCNE beta-subunits in functioning K+ channel complexes.
2008,
Pubmed
,
Xenbase
Nakajo,
KCNE1 and KCNE3 stabilize and/or slow voltage sensing S4 segment of KCNQ1 channel.
2007,
Pubmed
,
Xenbase
Nerbonne,
Molecular physiology of cardiac repolarization.
2005,
Pubmed
Panaghie,
Interaction of KCNE subunits with the KCNQ1 K+ channel pore.
2006,
Pubmed
,
Xenbase
Panaghie,
The impact of ancillary subunits on small-molecule interactions with voltage-gated potassium channels.
2006,
Pubmed
Park,
Impaired KCNQ1-KCNE1 and phosphatidylinositol-4,5-bisphosphate interaction underlies the long QT syndrome.
2005,
Pubmed
Peroz,
Kv7.1 (KCNQ1) properties and channelopathies.
2008,
Pubmed
Pusch,
Increase of the single-channel conductance of KvLQT1 potassium channels induced by the association with minK.
1998,
Pubmed
,
Xenbase
Robbins,
KCNQ potassium channels: physiology, pathophysiology, and pharmacology.
2001,
Pubmed
Rocheleau,
Secondary structure of a KCNE cytoplasmic domain.
2006,
Pubmed
,
Xenbase
Rocheleau,
KCNE peptides differently affect voltage sensor equilibrium and equilibration rates in KCNQ1 K+ channels.
2008,
Pubmed
,
Xenbase
Sanguinetti,
Coassembly of K(V)LQT1 and minK (IsK) proteins to form cardiac I(Ks) potassium channel.
1996,
Pubmed
,
Xenbase
Schmitt,
A recessive C-terminal Jervell and Lange-Nielsen mutation of the KCNQ1 channel impairs subunit assembly.
2000,
Pubmed
,
Xenbase
Sesti,
Single-channel characteristics of wild-type IKs channels and channels formed with two minK mutants that cause long QT syndrome.
1998,
Pubmed
,
Xenbase
Shamgar,
Calmodulin is essential for cardiac IKS channel gating and assembly: impaired function in long-QT mutations.
2006,
Pubmed
Shamgar,
KCNE1 constrains the voltage sensor of Kv7.1 K+ channels.
2008,
Pubmed
,
Xenbase
Snapp,
Formation of stacked ER cisternae by low affinity protein interactions.
2003,
Pubmed
Splawski,
Mutations in the hminK gene cause long QT syndrome and suppress IKs function.
1997,
Pubmed
,
Xenbase
Takumi,
Alteration of channel activities and gating by mutations of slow ISK potassium channel.
1991,
Pubmed
,
Xenbase
Tapper,
Location and orientation of minK within the I(Ks) potassium channel complex.
2001,
Pubmed
,
Xenbase
Tapper,
MinK subdomains that mediate modulation of and association with KvLQT1.
2000,
Pubmed
,
Xenbase
Tian,
Preparation, functional characterization, and NMR studies of human KCNE1, a voltage-gated potassium channel accessory subunit associated with deafness and long QT syndrome.
2007,
Pubmed
,
Xenbase
Villalba-Galea,
Charge movement of a voltage-sensitive fluorescent protein.
2009,
Pubmed
,
Xenbase
Wen,
Calmodulin is an auxiliary subunit of KCNQ2/3 potassium channels.
2002,
Pubmed
Wiener,
The KCNQ1 (Kv7.1) COOH terminus, a multitiered scaffold for subunit assembly and protein interaction.
2008,
Pubmed
Xu,
KCNQ1 and KCNE1 in the IKs channel complex make state-dependent contacts in their extracellular domains.
2008,
Pubmed
,
Xenbase
Yang,
Single-channel properties of IKs potassium channels.
1998,
Pubmed
,
Xenbase
Yus-Najera,
The identification and characterization of a noncontinuous calmodulin-binding site in noninactivating voltage-dependent KCNQ potassium channels.
2002,
Pubmed
Zacharias,
Partitioning of lipid-modified monomeric GFPs into membrane microdomains of live cells.
2002,
Pubmed
Zareba,
Long QT syndrome and short QT syndrome.
2008,
Pubmed
Zheng,
Stoichiometry and assembly of olfactory cyclic nucleotide-gated channels.
2004,
Pubmed
,
Xenbase
