PUBLICATION

The distal C terminus of the dihydropyridine receptor β1a subunit is essential for tetrad formation in skeletal muscle

Authors
Dayal, A., Perni, S., Franzini-Armstrong, C., Beam, K.G., Grabner, M.
ID
ZDB-PUB-220506-9
Date
2022
Source
Proceedings of the National Academy of Sciences of the United States of America   119: e2201136119 (Journal)
Registered Authors
Dayal, Anamika, Grabner, Manfred
Keywords
excitation?contraction coupling, skeletal muscle, tetrad formation, voltage-gated Ca2+ channel, ? subunit
MeSH Terms
  • Excitation Contraction Coupling
  • Zebrafish/genetics
  • Zebrafish/metabolism
  • Animals
  • Muscle Fibers, Skeletal*/metabolism
  • Ryanodine Receptor Calcium Release Channel*/metabolism
  • Calcium/metabolism
  • Muscle, Skeletal/metabolism
  • Calcium Channels, L-Type*/metabolism
  • Mice
  • Adaptor Proteins, Signal Transducing
(all 11)
PubMed
35507876 Full text @ Proc. Natl. Acad. Sci. USA
Abstract
SignificanceVertebrate skeletal muscle excitation-contraction coupling (ECC) is based on Ca2+-influx-independent interchannel cross-talk between DHPR and RyR1. The skeletal muscle DHPR complex consists of the main, voltage-sensing, and pore-forming α1S subunit, the auxiliary β1a, α2δ-1, γ1 subunits, and Stac3. The DHPRβ1a subunit plays an essential role in full triad targeting of DHPRα1S, voltage sensing, and tetrad formation (grouping of four DHPRs)-the three prerequisites for skeletal muscle ECC. Hence, a lack of DHPRβ1a results in a lethal phenotype in both β1-null mice and zebrafish. Here, we identified the nonconserved, distal C terminus of DHPRβ1a as playing a pivotal role in the formation of DHPR tetrads, and thus allosteric DHPR-RyR1 coupling, essential for proper skeletal muscle ECC.
Genes / Markers
Figures
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Expression
Phenotype
No data available
Mutations / Transgenics
Allele Construct Type Affected Genomic Region
ts25
    		Point Mutation
    1 - 1 of 1
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    Human Disease / Model
    No data available
    Sequence Targeting Reagents
    Fish
    No data available
    Antibodies
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    Orthology
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    Engineered Foreign Genes
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    Mapping
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    Citation
    Dayal, A., Perni, S., Franzini-Armstrong, C., Beam, K.G., Grabner, M. (2022) The distal C terminus of the dihydropyridine receptor β1a subunit is essential for tetrad formation in skeletal muscle. Proceedings of the National Academy of Sciences of the United States of America. 119:e2201136119.