KBTBD13 and the ever-expanding sarcomeric universe
Stuart G. Campbell1 and Steven A. Niederer2
1Departments of Biomedical Engineering and Cellular and Molecular Physiology, Yale University, New Haven, USA.
2Department of Biomedical Engineering, Kings’ College London, London, United Kingdom.
Address correspondence to: Stuart G. Campbell, 55 Prospect Street, New Haven, Connecticut 06511, USA. Phone: 203.432.4321; Email: stuart.campbell@yale.edu.
Find articles by Campbell, S. in: JCI | PubMed | Google Scholar
1Departments of Biomedical Engineering and Cellular and Molecular Physiology, Yale University, New Haven, USA.
2Department of Biomedical Engineering, Kings’ College London, London, United Kingdom.
Address correspondence to: Stuart G. Campbell, 55 Prospect Street, New Haven, Connecticut 06511, USA. Phone: 203.432.4321; Email: stuart.campbell@yale.edu.
Find articles by
Niederer, S.
in:
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First published January 6, 2020 - More info
J Clin Invest. 2020;130(2):593–594. https://doi.org/10.1172/JCI132954.
© 2020 American Society for Clinical Investigation
KBTBD13 is a protein expressed in striated muscle whose precise function is unknown. Work by de Winter et al. in this issue of the JCI provides evidence that KBTBD13 localizes to the sarcomere and can directly bind actin. A mutation in KBTBD13 that is associated with nemaline myopathy alters the protein’s effects on actin, apparently increasing thin-filament stiffness and ultimately depressing contractile force and relaxation rate. We discuss here the implications of this new sarcomeric protein, some alternate explanations for the effects of KBTBD13R408C, and the advantages of using computational models to interpret functional data from muscle.
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