Publication date: Feb 11, 2020
Huntington’s disease (HD) is a genetic neurodegenerative disorder caused by a highly polymorphic CAG trinucleotide repeat expansion encoding an extended polyglutamine (polyQ) tract at the N-terminus of huntingtin protein (HTT). The polyQ tract promotes the formation of toxic oligomers and aggregates of HTT, which leads to neuronal dysfunction and death. Therapies to lower mutant HTT (mHTT) and its aggregates appear to be the most promising strategies. Ellagic acid (EA) has been marketed as a dietary supplement with various claimed benefits and neuroprotective effects on several neurodegenerative disorders, while its effect on mHTT pathology is still unknown. Here we reported that EA significantly attenuated motor and cognitive deficits in R6/2 mice. Moreover, EA significantly lowered mHTT levels, reduced neuroinflammation, rescued synapse loss, and decreased oxidative stress in R6/2 mouse brains. These findings indicated that EA has promising therapeutic potential for HD treatment.
Sun, X., Zhu, J., Sun, X.Y., Ji, M., Yu, X.L., and Liu, R.T. Ellagic acid rescues motor and cognitive deficits in the R6/2 mouse model of Huntington’s disease by lowering mutant huntingtin protein. 06925. 2020 Food Funct.
|disease||MESH||trinucleotide repeat expansion|
- Effects of flanking sequences and cellular context on subcellular behavior and pathology of mutant HTT.
- Therapeutic Advances for Huntington’s Disease.