Ellagic acid rescues motor and cognitive deficits in the R6/2 mouse model of Huntington’s disease by lowering mutant huntingtin protein.

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.

Concepts Keywords
Cognitive Neuroprotective neurodegenerative disorders
Dietary Supplement Neuronal dysfunction
Ellagic Acid Dietary supplement
Genetic Branches of biology
Huntingtin Neurology
Huntingtin Protein Huntingtin
Huntington Neurodegeneration
Mice Polyglutamine tract
Mutant PolyQ
N Terminus Trinucleotide repeat disorder
Neurodegenerative Disorder Neuroprotection
Neurodegenerative Disorders Neuroinflammation
Neuroprotective MTOR
Oligomers Huntington’s disease
Oxidative Stress
Pathology
Polymorphic
PolyQ
Synapse
Trinucleotide Repeat Expansion

Semantics

Type Source Name
disease MESH oxidative stress
disease MESH pathology
disease MESH death
disease MESH trinucleotide repeat expansion
disease MESH neurodegenerative disorder
drug DRUGBANK Ellagic Acid

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