SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.

Huntington's disease (HD) is a neurodegenerative disorder for which there are no disease-modifying treatments. SIRT1 is a NAD+-dependent protein deacetylase that is implicated in maintaining neuronal health during development, differentiation and ageing. Previous studies suggested that the modu...

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Main Authors: Raffaella Tulino, Agnesska C Benjamin, Nelly Jolinon, Donna L Smith, Eduardo N Chini, Alisia Carnemolla, Gillian P Bates
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2016-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0145425
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author Raffaella Tulino
Agnesska C Benjamin
Nelly Jolinon
Donna L Smith
Eduardo N Chini
Alisia Carnemolla
Gillian P Bates
author_facet Raffaella Tulino
Agnesska C Benjamin
Nelly Jolinon
Donna L Smith
Eduardo N Chini
Alisia Carnemolla
Gillian P Bates
author_sort Raffaella Tulino
collection DOAJ
description Huntington's disease (HD) is a neurodegenerative disorder for which there are no disease-modifying treatments. SIRT1 is a NAD+-dependent protein deacetylase that is implicated in maintaining neuronal health during development, differentiation and ageing. Previous studies suggested that the modulation of SIRT1 activity is neuroprotective in HD mouse models, however, the mechanisms controlling SIRT1 activity are unknown. We have identified a striatum-specific phosphorylation-dependent regulatory mechanism of SIRT1 induction under normal physiological conditions, which is impaired in HD. We demonstrate that SIRT1 activity is down-regulated in the brains of two complementary HD mouse models, which correlated with altered SIRT1 phosphorylation levels. This SIRT1 impairment could not be rescued by the ablation of DBC1, a negative regulator of SIRT1, but was linked to changes in the sub-cellular distribution of AMPK-α1, a positive regulator of SIRT1 function. This work provides insights into the regulation of SIRT1 activity with the potential for the development of novel therapeutic strategies.
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spelling doaj-art-fa4ad6c77f364fdf81ee63cfbb192c312025-08-20T02:34:07ZengPublic Library of Science (PLoS)PLoS ONE1932-62032016-01-01111e014542510.1371/journal.pone.0145425SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.Raffaella TulinoAgnesska C BenjaminNelly JolinonDonna L SmithEduardo N ChiniAlisia CarnemollaGillian P BatesHuntington's disease (HD) is a neurodegenerative disorder for which there are no disease-modifying treatments. SIRT1 is a NAD+-dependent protein deacetylase that is implicated in maintaining neuronal health during development, differentiation and ageing. Previous studies suggested that the modulation of SIRT1 activity is neuroprotective in HD mouse models, however, the mechanisms controlling SIRT1 activity are unknown. We have identified a striatum-specific phosphorylation-dependent regulatory mechanism of SIRT1 induction under normal physiological conditions, which is impaired in HD. We demonstrate that SIRT1 activity is down-regulated in the brains of two complementary HD mouse models, which correlated with altered SIRT1 phosphorylation levels. This SIRT1 impairment could not be rescued by the ablation of DBC1, a negative regulator of SIRT1, but was linked to changes in the sub-cellular distribution of AMPK-α1, a positive regulator of SIRT1 function. This work provides insights into the regulation of SIRT1 activity with the potential for the development of novel therapeutic strategies.https://doi.org/10.1371/journal.pone.0145425
spellingShingle Raffaella Tulino
Agnesska C Benjamin
Nelly Jolinon
Donna L Smith
Eduardo N Chini
Alisia Carnemolla
Gillian P Bates
SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.
PLoS ONE
title SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.
title_full SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.
title_fullStr SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.
title_full_unstemmed SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.
title_short SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.
title_sort sirt1 activity is linked to its brain region specific phosphorylation and is impaired in huntington s disease mice
url https://doi.org/10.1371/journal.pone.0145425
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