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TitleStructure of human Na1.5 reveals the fast inactivation-related segments as a mutational hotspot for the long QT syndrome.
Journal, issue, pagesProc Natl Acad Sci U S A, Vol. 118, Issue 11, Year 2021
Publish dateMar 16, 2021
AuthorsZhangqiang Li / Xueqin Jin / Tong Wu / Xin Zhao / Weipeng Wang / Jianlin Lei / Xiaojing Pan / Nieng Yan /
PubMed AbstractNa1.5 is the primary voltage-gated Na (Na) channel in the heart. Mutations of Na1.5 are associated with various cardiac disorders exemplified by the type 3 long QT syndrome (LQT3) and Brugada ...Na1.5 is the primary voltage-gated Na (Na) channel in the heart. Mutations of Na1.5 are associated with various cardiac disorders exemplified by the type 3 long QT syndrome (LQT3) and Brugada syndrome (BrS). E1784K is a common mutation that has been found in both LQT3 and BrS patients. Here we present the cryo-EM structure of the human Na1.5-E1784K variant at an overall resolution of 3.3 Å. The structure is nearly identical to that of the wild-type human Na1.5 bound to quinidine. Structural mapping of 91- and 178-point mutations that are respectively associated with LQT3 and BrS reveals a unique distribution pattern for LQT3 mutations. Whereas the BrS mutations spread evenly on the structure, LQT3 mutations are clustered mainly to the segments in repeats III and IV that are involved in gating, voltage-sensing, and particularly inactivation. A mutational hotspot involving the fast inactivation segments is identified and can be mechanistically interpreted by our "door wedge" model for fast inactivation. The structural analysis presented here, with a focus on the impact of mutations on inactivation and late sodium current, establishes a structure-function relationship for the mechanistic understanding of Na1.5 channelopathies.
External linksProc Natl Acad Sci U S A / PubMed:33712541 / PubMed Central
MethodsEM (single particle)
Resolution3.3 Å
Structure data

30850

7dtc

EMDB-30850, PDB-7dtc:
voltage-gated sodium channel Nav1.5-E1784K
Method: EM (single particle) / Resolution: 3.3 Å

Chemicals

ChemComp-NAG:
2-acetamido-2-deoxy-beta-D-glucopyranose / N-Acetylglucosamine

Source
  • homo sapiens (human)
KeywordsMEMBRANE PROTEIN / voltage-gated sodium channel

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