Erucin, a Natural Isothiocyanate, Prevents Polyglutamine-Induced Toxicity in Caenorhabditis elegans via aak-2/AMPK and daf-16/FOXO Signaling

Several neurodegenerative diseases (NDDs), such as Huntington’s disease, six of the spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and spinobulbar muscular atrophy, are caused by abnormally long polyglutamine (polyQ) tracts. Natural compounds capable of alleviating polyQ-induced toxi...

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Vydáno v:International journal of molecular sciences Ročník 25; číslo 22; s. 12220
Hlavní autoři: Balducci, Martina, Pérez, Julia Tortajada, del Río, Cristina Trujillo, Pérez, Mar Collado, Carranza, Andrea del Valle, Gomez Escribano, Ana Pilar, Vázquez-Manrique, Rafael P., Tarozzi, Andrea
Médium: Journal Article
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Vydáno: Switzerland MDPI AG 14.11.2024
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ISSN:1422-0067, 1661-6596, 1422-0067
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Abstract Several neurodegenerative diseases (NDDs), such as Huntington’s disease, six of the spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and spinobulbar muscular atrophy, are caused by abnormally long polyglutamine (polyQ) tracts. Natural compounds capable of alleviating polyQ-induced toxicity are currently of great interest. In this work, we investigated the modulatory effect against polyQ neurotoxic aggregates exerted by erucin (ERN), an isothiocyanate naturally present in its precursor glucoerucin in rocket salad leaves and in its oxidized form, sulforaphane (SFN), in broccoli. Using C. elegans models expressing polyQ in different tissues, we demonstrated that ERN protects against polyQ-induced toxicity and that its action depends on the catalytic subunit of AMP-activated protein kinase (aak-2/AMPKα2) and, downstream in this pathway, on the daf-16/FOXO transcription factor, since nematodes deficient in aak-2/AMPKα2 and daf-16 did not respond to the treatment, respectively. Although triggered by a different source of neurotoxicity than polyQ diseases, i.e., by α-synuclein (α-syn) aggregates, Parkinson’s disease (PD) was also considered in our study. Our results showed that ERN reduces α-syn aggregates and slightly improves the motility of worms. Therefore, further preclinical studies in mouse models of protein aggregation are justified and could provide insights into testing whether ERN could be a potential neuroprotective compound in humans.
AbstractList Several neurodegenerative diseases (NDDs), such as Huntington's disease, six of the spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and spinobulbar muscular atrophy, are caused by abnormally long polyglutamine (polyQ) tracts. Natural compounds capable of alleviating polyQ-induced toxicity are currently of great interest. In this work, we investigated the modulatory effect against polyQ neurotoxic aggregates exerted by erucin (ERN), an isothiocyanate naturally present in its precursor glucoerucin in rocket salad leaves and in its oxidized form, sulforaphane (SFN), in broccoli. Using C. elegans models expressing polyQ in different tissues, we demonstrated that ERN protects against polyQ-induced toxicity and that its action depends on the catalytic subunit of AMP-activated protein kinase (aak-2/AMPKα2) and, downstream in this pathway, on the daf-16/FOXO transcription factor, since nematodes deficient in aak-2/AMPKα2 and daf-16 did not respond to the treatment, respectively. Although triggered by a different source of neurotoxicity than polyQ diseases, i.e., by α-synuclein (α-syn) aggregates, Parkinson's disease (PD) was also considered in our study. Our results showed that ERN reduces α-syn aggregates and slightly improves the motility of worms. Therefore, further preclinical studies in mouse models of protein aggregation are justified and could provide insights into testing whether ERN could be a potential neuroprotective compound in humans.Several neurodegenerative diseases (NDDs), such as Huntington's disease, six of the spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and spinobulbar muscular atrophy, are caused by abnormally long polyglutamine (polyQ) tracts. Natural compounds capable of alleviating polyQ-induced toxicity are currently of great interest. In this work, we investigated the modulatory effect against polyQ neurotoxic aggregates exerted by erucin (ERN), an isothiocyanate naturally present in its precursor glucoerucin in rocket salad leaves and in its oxidized form, sulforaphane (SFN), in broccoli. Using C. elegans models expressing polyQ in different tissues, we demonstrated that ERN protects against polyQ-induced toxicity and that its action depends on the catalytic subunit of AMP-activated protein kinase (aak-2/AMPKα2) and, downstream in this pathway, on the daf-16/FOXO transcription factor, since nematodes deficient in aak-2/AMPKα2 and daf-16 did not respond to the treatment, respectively. Although triggered by a different source of neurotoxicity than polyQ diseases, i.e., by α-synuclein (α-syn) aggregates, Parkinson's disease (PD) was also considered in our study. Our results showed that ERN reduces α-syn aggregates and slightly improves the motility of worms. Therefore, further preclinical studies in mouse models of protein aggregation are justified and could provide insights into testing whether ERN could be a potential neuroprotective compound in humans.
Several neurodegenerative diseases (NDDs), such as Huntington’s disease, six of the spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and spinobulbar muscular atrophy, are caused by abnormally long polyglutamine (polyQ) tracts. Natural compounds capable of alleviating polyQ-induced toxicity are currently of great interest. In this work, we investigated the modulatory effect against polyQ neurotoxic aggregates exerted by erucin (ERN), an isothiocyanate naturally present in its precursor glucoerucin in rocket salad leaves and in its oxidized form, sulforaphane (SFN), in broccoli. Using C. elegans models expressing polyQ in different tissues, we demonstrated that ERN protects against polyQ-induced toxicity and that its action depends on the catalytic subunit of AMP-activated protein kinase (aak-2 /AMPKα2) and, downstream in this pathway, on the daf-16 /FOXO transcription factor, since nematodes deficient in aak-2 /AMPKα2 and daf-16 did not respond to the treatment, respectively. Although triggered by a different source of neurotoxicity than polyQ diseases, i.e., by α-synuclein (α-syn) aggregates, Parkinson’s disease (PD) was also considered in our study. Our results showed that ERN reduces α-syn aggregates and slightly improves the motility of worms. Therefore, further preclinical studies in mouse models of protein aggregation are justified and could provide insights into testing whether ERN could be a potential neuroprotective compound in humans.
Several neurodegenerative diseases (NDDs), such as Huntington's disease, six of the spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and spinobulbar muscular atrophy, are caused by abnormally long polyglutamine (polyQ) tracts. Natural compounds capable of alleviating polyQ-induced toxicity are currently of great interest. In this work, we investigated the modulatory effect against polyQ neurotoxic aggregates exerted by erucin (ERN), an isothiocyanate naturally present in its precursor glucoerucin in rocket salad leaves and in its oxidized form, sulforaphane (SFN), in broccoli. Using models expressing polyQ in different tissues, we demonstrated that ERN protects against polyQ-induced toxicity and that its action depends on the catalytic subunit of AMP-activated protein kinase ( /AMPKα2) and, downstream in this pathway, on the /FOXO transcription factor, since nematodes deficient in /AMPKα2 and did not respond to the treatment, respectively. Although triggered by a different source of neurotoxicity than polyQ diseases, i.e., by α-synuclein (α-syn) aggregates, Parkinson's disease (PD) was also considered in our study. Our results showed that ERN reduces α-syn aggregates and slightly improves the motility of worms. Therefore, further preclinical studies in mouse models of protein aggregation are justified and could provide insights into testing whether ERN could be a potential neuroprotective compound in humans.
Audience Academic
Author Balducci, Martina
Pérez, Mar Collado
Tarozzi, Andrea
Pérez, Julia Tortajada
del Río, Cristina Trujillo
Carranza, Andrea del Valle
Gomez Escribano, Ana Pilar
Vázquez-Manrique, Rafael P.
AuthorAffiliation 3 Joint Unit for Rare Diseases IIS La Fe-CIPF, 46012 Valencia, Spain
2 Laboratory of Molecular, Cellular and Genomic Biomedicine, Instituto de Investigación Sanitaria La Fe, 46012 Valencia, Spain; julia_tortajada@iislafe.es (J.T.P.); cristina_trujillo@iislafe.es (C.T.d.R.); mar_collado@iislafe.es (M.C.P.); andrea_carranza@iislafe.es (A.d.V.C.); ana_pilar_gomez@iislafe.es (A.P.G.E.); rafael_vazquez@iislafe.es (R.P.V.-M.)
4 Centro de Investigación Biomédica en Red (CIBER), 28029 Madrid, Spain
1 Department for Life Quality Studies, University of Bologna, 47921 Rimini, Italy
5 Biostructures and Biosystems National Institute (INBB), 00136 Rome, Italy
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BackLink https://www.ncbi.nlm.nih.gov/pubmed/39596283$$D View this record in MEDLINE/PubMed
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Keywords Caenorhabditis elegans
AMPK
erucin
neuroprotection
polyQ toxicity
daf-16/FOXO
Language English
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Snippet Several neurodegenerative diseases (NDDs), such as Huntington’s disease, six of the spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and...
Several neurodegenerative diseases (NDDs), such as Huntington's disease, six of the spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, and...
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StartPage 12220
SubjectTerms Aggregates
AMP-Activated Protein Kinases - metabolism
Animals
Caenorhabditis elegans - drug effects
Caenorhabditis elegans - metabolism
Caenorhabditis elegans Proteins - genetics
Caenorhabditis elegans Proteins - metabolism
Cyclic adenylic acid
Degeneration
Disease prevention
Forkhead Transcription Factors - genetics
Forkhead Transcription Factors - metabolism
Genomes
Glutamine
Health aspects
Human subjects
Huntingtons disease
Isothiocyanates - pharmacology
Kinases
Nematodes
Nervous system
Organosulfur compounds
Peptides - metabolism
Peptides - pharmacology
Physiological aspects
Protein kinases
Proteins
Signal transduction
Signal Transduction - drug effects
Sulfides
Sulfoxides - pharmacology
Thiocyanates
Toxicity
Transcription factors
Worms
Young adults
Title Erucin, a Natural Isothiocyanate, Prevents Polyglutamine-Induced Toxicity in Caenorhabditis elegans via aak-2/AMPK and daf-16/FOXO Signaling
URI https://www.ncbi.nlm.nih.gov/pubmed/39596283
https://www.proquest.com/docview/3133091531
https://www.proquest.com/docview/3133456631
https://pubmed.ncbi.nlm.nih.gov/PMC11594550
Volume 25
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