The importance of a proper SOCE in skeletal muscle is evidenced by the observation that mutations in STIM1 and/or Orai1 genes or defects in STIM1/Orai1-mediated SOCE cause or contribute both directly and indirectly to the pathogenesis of various skeletal muscle disorders, including myopathies, dystrophies, cachexia, and age-related sarcopenia (Table 1). Here, STIM1 is linked to myopathy.