The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.
KCNQ1 Assembly and Function Is Blocked by Long-QT Syndrome Mutations That Disrupt Interaction With Calmodulin
Abstract
Calmodulin (CaM) has been recognized as an obligate subunit for many ion channels in which its function has not been clearly established. Because channel subunits associate early during channel biosynthesis, CaM may provide a mechanism for Ca 2+ -dependent regulation of channel formation. Here we show that CaM is a constitutive component of KCNQ1 K + channels, the most commonly mutated long-QT syndrome (LQTS) locus. CaM not only acts as a regulator of channel gating, relieving inactivation in a Ca 2+ -dependent manner, but it also contributes to control of channel assembly. Formation of functional tetramers requires CaM interaction with the KCNQ1 C-terminus. This CaM-regulated process is essential: LQTS mutants that disrupt CaM interaction prevent functional assembly of channels in a dominant-negative manner. These findings offer a new mechanism for LQTS defects and provide a basis for understanding novel ways that intracellular Ca 2+ and CaM regulate ion channels.
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Cites
CaV1.2 Calcium Channel Dysfunction Causes a Multisystem Disorder Including Arrhythmia and Autism
Splawski, I., Timothy, K. W., Sharpe, L. M., Decher, N., Kumar, P., Bloise, R., Napolitano, C., Schwartz, P. J., Joseph, R. M., Condouris, K., et al. (2004) · Cell · doi:10.1016/j.cell.2004.09.011 · Cited by 1,586
A Potassium Channel Mutation in Neonatal Human Epilepsy
Biervert, C., Schroeder, B. C., Kubisch, C., Berkovic, S. F., Propping, P., Jentsch, T. J., & Steinlein, O. K. (1998) · Science · doi:10.1126/science.279.5349.403 · Cited by 1,073
Ankyrin-B mutation causes type 4 long-QT cardiac arrhythmia and sudden cardiac death
Mohler, P. J., Schott, J.-J., Gramolini, A. O., Dilly, K. W., Guatimosim, S., duBell, W. H., Song, L.-S., Haurogné, K., Kyndt, F., Ali, M. E., et al. (2003) · Nature · doi:10.1038/nature01335 · Cited by 957
Cited by
Calcium Cycling and Signaling in Cardiac Myocytes
Bers, D. M. (2008) · Annual Review of Physiology · doi:10.1146/annurev.physiol.70.113006.100455 · Cited by 1,302
ter Keurs, H. E. D. J., & Boyden, P. A. (2007) · Physiological Reviews · doi:10.1152/physrev.00011.2006 · Cited by 356
Maier, L. S., & Bers, D. M. (2007) · Cardiovascular Research · doi:10.1016/j.cardiores.2006.11.005 · Cited by 330
Cryo-EM Structure of a KCNQ1/CaM Complex Reveals Insights into Congenital Long QT Syndrome
Sun, J., & MacKinnon, R. (2017) · Cell · doi:10.1016/j.cell.2017.05.019 · Cited by 305
Structural Basis of Human KCNQ1 Modulation and Gating
Sun, J., & MacKinnon, R. (2020) · Cell · doi:10.1016/j.cell.2019.12.003 · Cited by 293
Metadata
- Authors
- Ghosh, S., Nunziato, D. A., & Pitt, G. S.
- Venue
- Circulation Research
- Published
- 2006-04-28
- Volume
- 98
- Issue
- 8
- Pages
- 1048-1054
- ISSN
- 0009-7330 (ISSN-L)
- 1524-4571
- OpenAlex
- W2135227689
- Cited by
- 175
- References
- 3
- FWCI
- 3.81
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: KCNQ1 Assembly and Function Is Blocked by Long-QT Syndrome Mutations That Disrupt Interaction With Calmodulin. https://app.cochat.ai/papers/10.1161/01.res.0000218863.44140.f2
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- Ghosh, S., Nunziato, D. A., & Pitt, G. S. (2006). KCNQ1 Assembly and Function Is Blocked by Long-QT Syndrome Mutations That Disrupt Interaction With Calmodulin. Circulation Research. https://doi.org/10.1161/01.res.0000218863.44140.f2
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