What is the role of rimmed vacuoles in the pathophysiology of sporadic inclusion body myositis (s-IBM)?

Updated: Jun 08, 2018
  • Author: Michael P Collins, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
  • Print
Answer

Most of the rimmed vacuoles in s-IBM are autophagic and composed of lysosomes. [25, 46, 47] Accumulated Aβ and APP are specific targets of macroautophagy in this disease. [47] However, some of the vacuoles lack lysosomal features and instead contain nuclear proteins, suggesting that they result from the breakdown of myonuclei. [48, 49] Nuclear membrane remnants (lamin A/C and emerin), nuclear histones, and the nuclear transcription factor pElk-1 have been found in rimmed vacuoles. [48, 50] Thus, the formation of rimmed vacuoles in s-IBM is probably mediated by more than one mechanism.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!