Elevated Ca²⁺ at the triad junction underlies dysregulation of Ca²⁺ signaling in dysferlin-null skeletal muscle.

ABSTRACT: Dysferlin-null A/J myofibers generate abnormal Ca²⁺ transients that are slightly reduced in amplitude compared to controls. These are further reduced in amplitude by hypoosmotic shock and often appear as Ca²⁺ waves (Lukyanenko et al., J. Physiol., 2017). Ca²⁺ waves are typically associated with Ca²⁺-induced Ca²⁺ release, or CICR, which can be myopathic. We tested the ability of a permeable Ca²⁺ chelator, BAPTA-AM, to inhibit CICR in injured dysferlin-null fibers and found that 10-50 nM BAPTA-AM suppressed all Ca²⁺ waves. The same concentrations of BAPTA-AM increased the amplitude of the Ca²⁺ transient in A/J fibers to wild type levels and protected transients against the loss of amplitude after hypoosmo

SUBMITTER: Lukyanenko V 

PROVIDER: S-EPMC9669649 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature