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Kevin M. Tylock, David S. Auerbach, Zhen Zhi Tang, Charles A. Thornton, Robert T. Dirksen
The expression of expanded CUG-repeat RNAs in myotonic dystrophy type 1 patients is associated with cardiac electrophysiological defects and an elevated risk of sudden cardiac death. Tylock et al. reveal that expression of these RNAs in the murine heart results in prolonged QRS and corrected QT-intervals due to alterations in sodium and potassium channel activity.
Timothy Lynagh, Emelie Flood, Céline Boiteux, Zeshan Pervez Sheikh, Toby W. Allen, Stephan A. Pless
Neuronal ASIC channels mediate excitatory sodium currents in response to extracellular acidification. Lynagh et al. reveal the essential roles of pore-lining glutamates in ion conduction and selectivity for ASIC1a- and ASIC2a-containing channels.
María Queralt-Martín, Lucie Bergdoll, Oscar Teijido, Nabill Munshi, Daniel Jacobs, Adam J. Kuszak, Olga Protchenko, Simona Reina, Andrea Magrì, Vito De Pinto, Sergey M. Bezrukov, Jeff Abramson, Tatiana K. Rostovtseva
Mammalian isoforms of mitochondrial VDAC have unique functional roles in vivo, but they all share similar β-barrel pore structures. Queralt-Martín et al. provide the first mechanistic insights into VDAC’s isoform-specific function by showing that VDAC3 exhibits unique properties when interacting with cytosolic proteins α-synuclein and tubulin.
Briana M. Bohannon, Xiaoan Wu, Xiongyu Wu, Marta E. Perez, Sara I. Liin, H. Peter Larsson
Bohannon et al. show that polyunsaturated fatty acids (PUFAs) vary in their ability to activate cardiac IKs potassium channels depending on the pKa of the PUFA head group, allowing PUFAs to be tuned to treat long QT syndrome mutants with different disease severity.
Aravind R. Gade, Steven O. Marx, Geoffrey S. Pitt
The auxiliary proteins CaM and FHF bind to the C-terminal domain of voltage-gated sodium channels to limit the persistent inward currents associated with cardiac arrhythmias. Gade et al. reveal that CaM and FHF exert their effects by stabilizing an interaction between the channel’s C-terminal domain and III-IV linker region.
Toshiko Yamazawa, Toshiko Yamazawa, Haruo Ogawa, Haruo Ogawa, Takashi Murayama, Maki Yamaguchi, Hideto Oyamada, Junji Suzuki, Nagomi Kurebayashi, Kazunori Kanemaru, Katsuji Oguchi, Takashi Sakurai, Masamitsu Iino
Molecular bases of pathogenic enhancement of Ca2+ release channel activities in RYR1 carrying disease-associated mutations at the N-terminal region were studied. Functional studies and MD simulation revealed that the interactions between domains have a strong correlation with channel activity.
Grant A. Hussey, Nathan E. Thomas, Katherine A. Henzler-Wildman
Secondary active transport mechanisms often depict a limited number of states and transitions, although recent research suggests that alternative “leak” pathways are not always minor. Hussey et al. numerically simulate a fully unrestricted model for the bacterial transporter EmrE and demonstrate that highly efficient transport can be achieved despite the existence of multiple leak pathways.

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Volume 152,
Issue 1,
January 6, 2020
Reviews & Opinions
Research News
Ben Short
JGP study suggests that varying the head group of polyunsaturated fatty acids could enable personalized treatments for long QT syndrome.
Donald W. Hilgemann
Ion transporters can be strongly regulated by autoinhibitory reactions that depend on ion binding within their transport sites.
David A. Eisner
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