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Determination of neuroprotective effects of medium chain fatty acids and their derivatives on mutant huntingtin aggregates, oxidative stress and ATP levels in HD150Q cell line model of Huntington’s disease

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Title Determination of neuroprotective effects of medium chain fatty acids and their derivatives on mutant huntingtin aggregates, oxidative stress and ATP levels in HD150Q cell line model of Huntington’s disease
 
Creator Dabhi, Rajubhai
Mehta, Ragi
Paneri, Jayshree
Kakadiya, Dhruvi
Shah, Anjali
Singh, Sushmita
Vijayvargia, Ravi
 
Subject Decanoic acid
2-butyloctanoic acid
Glyceryl triacetate
Huntington’s disease
Mitochondrial dysfunction
Mutant huntingtin aggregates
Oxidative stress
 
Description 741-749
Huntington's disease (HD) is a rare autosomal dominant genetic disorder resulting from expansion of polymorphic CAG repeats in the exon 1 of huntingtin gene that translates into elongated polyglutamine (ployQ) tract in huntingtin protein (HTT).PolyQ expansion alters HTT structure resulting in abnormal protein-protein interactions, aggregation, mitochondrial dysfunction, oxidative and endoplasmic reticulum stress, inflammation and altered gene expression leading to neuronal cell death.HD symptoms involves chorea, dementia, behavioural and psychological problems and currently there is no cure highlighting the need for novel therapeutic interventions. Several fatty acids have been reported to have protective effects in neurological disorders including Alzheimer’s disease, Parkinson’s disease and epilepsy. However, their effects in HD is largely unexplored. Neurodegenerative diseases share several common pathways and thus it is likely that a combination of selected fatty acids show neuroprotective effects in HD. This study utilized a cell line model of HD expressing inducible mutant huntingtin fragment with 150 polyQ repeats (HD150Q) to investigate neuroprotective effects of two medium chain fatty acids and one triglyceride. Significant reduction in mutant HTT aggregates and mitochondrial oxidative stress and restoration of ATP levels was observed upon treatment with Decanoic acid, 2-butyloctanoic acid, and Glyceryl triacetate. Encouraging results in the cell line model opens avenues for investigating the underlying molecular mechanisms and validation in the animal models.
 
Date 2023-09-19T10:25:38Z
2023-09-19T10:25:38Z
2023-09
 
Type Article
 
Identifier 0975-0959 (Online); 0301-1208 (Print)
http://nopr.niscpr.res.in/handle/123456789/62525
https://doi.org/10.56042/ijbb.v60i9.4047
 
Language en
 
Publisher NIScPR-CSIR,India
 
Source IJBB Vol.60(09) [September 2023]