SIRT1 activation rescues the mislocalization of RNA-binding proteins and cognitive defects induced by inherited cobalamin disorders

Published: 10-28-2019 In Publication


主题:

Highlights

 

• Defects of cobalamin metabolism lead to mislocalization of RNA binding proteins.
• SRT1720 treatment rescued abnormal localization of RNA binding proteins.
• SRT1720 treatment improved the cognitive defects observed in a transgenic mouse model.

 

Abstract

Background

The molecular consequences of inborn errors of vitamin B12 or cobalamin metabolism are far from being understood. Moreover, innovative therapeutic strategies are needed for the treatment of neurological outcomes that are usually resistant to conventional treatments. Our previous findings suggest a link between SIRT1, cellular stress and RNA binding proteins (RBP) mislocalization in the pathological mechanisms triggered by impaired vitamin B12 metabolism.