Constitutive, Muscle-Specific Orai1 Knockout Results in the Incomplete Assembly of Ca²⁺ Entry Units and a Reduction in the Age-Dependent Formation of Tubular Aggregates.

ABSTRACT: Store-operated Ca²⁺ entry (SOCE) is a ubiquitous cellular mechanism that cells use to activate extracellular Ca²⁺ entry when intracellular Ca²⁺ stores are depleted. In skeletal muscle, SOCE occurs within Ca²⁺ entry units (CEUs), intracellular junctions between stacks of SR membranes containing STIM1 and transverse tubules (TTs) containing ORAI1. Gain-of-function mutations in STIM1 and ORAI1 are linked to tubular aggregate (TA) myopathy, a disease characterized by the atypical accumulation of tubes of SR origin. Moreover, SOCE and TAs are increased in the muscles of aged male mice. Here, we assessed the longitudinal effects (from 4-6 months to 10-14 months of age) of constitutive, muscle-specific Orai1 knockout (cOrai1 KO) on

SUBMITTER: Di Fonso A 

PROVIDER: S-EPMC11351919 | biostudies-literature | 2024 Jul

REPOSITORIES: biostudies-literature