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Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies

Background: Huntington’s disease is an inherited autosomal dominant trait neuro-degenerative disorder caused by changes (mutations) of a gene called huntingtin (htt) that is located on the short arm (p) of chromosome 4, CAG expansion mutation. It is characterized by unusual movements, cognitive and...

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Autores principales: Irfan, Zainab, Khanam, Sofia, Karmakar, Varnita, Firdous, Sayeed Mohammed, El Khier, Bothaina Samih Ismail Abou, Khan, Ilyas, Rehman, Muneeb U., Khan, Andleeb
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9599635/
https://www.ncbi.nlm.nih.gov/pubmed/36291322
http://dx.doi.org/10.3390/brainsci12101389
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author Irfan, Zainab
Khanam, Sofia
Karmakar, Varnita
Firdous, Sayeed Mohammed
El Khier, Bothaina Samih Ismail Abou
Khan, Ilyas
Rehman, Muneeb U.
Khan, Andleeb
author_facet Irfan, Zainab
Khanam, Sofia
Karmakar, Varnita
Firdous, Sayeed Mohammed
El Khier, Bothaina Samih Ismail Abou
Khan, Ilyas
Rehman, Muneeb U.
Khan, Andleeb
author_sort Irfan, Zainab
collection PubMed
description Background: Huntington’s disease is an inherited autosomal dominant trait neuro-degenerative disorder caused by changes (mutations) of a gene called huntingtin (htt) that is located on the short arm (p) of chromosome 4, CAG expansion mutation. It is characterized by unusual movements, cognitive and psychiatric disorders. Objective: This review was undertaken to apprehend biological pathways of Huntington’s disease (HD) pathogenesis and its management by nature-derived products. Natural products can be lucrative for the management of HD as it shows protection against HD in pre-clinical trials. Advanced research is still required to assess the therapeutic effectiveness of the known organic products and their isolated compounds in HD experimental models. Summary: Degeneration of neurons in Huntington’s disease is distinguished by progressive loss of motor coordination and muscle function. This is due to the expansion of CAG trinucleotide in the first exon of the htt gene responsible for neuronal death and neuronal network degeneration in the brain. It is believed that the factors such as molecular genetics, oxidative stress, excitotoxicity, mitochondrial dysfunction, neuroglia dysfunction, protein aggregation, and altered UPS leads to HD. The defensive effect of the natural product provides therapeutic efficacy against HD. Recent reports on natural drugs have enlightened the protective role against HD via antioxidant, anti-inflammatory, antiapoptotic, and neurofunctional regulation.
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spelling pubmed-95996352022-10-27 Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies Irfan, Zainab Khanam, Sofia Karmakar, Varnita Firdous, Sayeed Mohammed El Khier, Bothaina Samih Ismail Abou Khan, Ilyas Rehman, Muneeb U. Khan, Andleeb Brain Sci Review Background: Huntington’s disease is an inherited autosomal dominant trait neuro-degenerative disorder caused by changes (mutations) of a gene called huntingtin (htt) that is located on the short arm (p) of chromosome 4, CAG expansion mutation. It is characterized by unusual movements, cognitive and psychiatric disorders. Objective: This review was undertaken to apprehend biological pathways of Huntington’s disease (HD) pathogenesis and its management by nature-derived products. Natural products can be lucrative for the management of HD as it shows protection against HD in pre-clinical trials. Advanced research is still required to assess the therapeutic effectiveness of the known organic products and their isolated compounds in HD experimental models. Summary: Degeneration of neurons in Huntington’s disease is distinguished by progressive loss of motor coordination and muscle function. This is due to the expansion of CAG trinucleotide in the first exon of the htt gene responsible for neuronal death and neuronal network degeneration in the brain. It is believed that the factors such as molecular genetics, oxidative stress, excitotoxicity, mitochondrial dysfunction, neuroglia dysfunction, protein aggregation, and altered UPS leads to HD. The defensive effect of the natural product provides therapeutic efficacy against HD. Recent reports on natural drugs have enlightened the protective role against HD via antioxidant, anti-inflammatory, antiapoptotic, and neurofunctional regulation. MDPI 2022-10-14 /pmc/articles/PMC9599635/ /pubmed/36291322 http://dx.doi.org/10.3390/brainsci12101389 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Irfan, Zainab
Khanam, Sofia
Karmakar, Varnita
Firdous, Sayeed Mohammed
El Khier, Bothaina Samih Ismail Abou
Khan, Ilyas
Rehman, Muneeb U.
Khan, Andleeb
Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies
title Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies
title_full Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies
title_fullStr Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies
title_full_unstemmed Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies
title_short Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies
title_sort pathogenesis of huntington’s disease: an emphasis on molecular pathways and prevention by natural remedies
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9599635/
https://www.ncbi.nlm.nih.gov/pubmed/36291322
http://dx.doi.org/10.3390/brainsci12101389
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