Defining the role of the Bcl-2 family proteins in Huntington's disease

B-cell lymphoma 2 (Bcl-2) family proteins regulate survival, mitochondria morphology dynamics and metabolism in many cell types including neurons. Huntington's disease (HD) is a neurodegenerative disorder caused by an expanded CAG repeat tract in the IT15 gene that encodes for the protein hunti...

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Autores principales: Sassone, J, Maraschi, A, Sassone, F, Silani, V, Ciammola, A
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2013
Materias:
Review
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3763461/
https://www.ncbi.nlm.nih.gov/pubmed/23949221
http://dx.doi.org/10.1038/cddis.2013.300
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author Sassone, J
Maraschi, A
Sassone, F
Silani, V
Ciammola, A
author_facet Sassone, J
Maraschi, A
Sassone, F
Silani, V
Ciammola, A
author_sort Sassone, J
collection PubMed
description B-cell lymphoma 2 (Bcl-2) family proteins regulate survival, mitochondria morphology dynamics and metabolism in many cell types including neurons. Huntington's disease (HD) is a neurodegenerative disorder caused by an expanded CAG repeat tract in the IT15 gene that encodes for the protein huntingtin (htt). In vitro and in vivo models of HD and HD patients' tissues show abnormal mitochondrial function and increased cell death rates associated with alterations in Bcl-2 family protein expression and localization. This review aims to draw together the information related to Bcl-2 family protein alterations in HD to decipher their potential role in mutated htt-related cell death and mitochondrial dysfunction.
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spelling pubmed-37634612013-09-11 Defining the role of the Bcl-2 family proteins in Huntington's disease Sassone, J Maraschi, A Sassone, F Silani, V Ciammola, A Cell Death Dis Review B-cell lymphoma 2 (Bcl-2) family proteins regulate survival, mitochondria morphology dynamics and metabolism in many cell types including neurons. Huntington's disease (HD) is a neurodegenerative disorder caused by an expanded CAG repeat tract in the IT15 gene that encodes for the protein huntingtin (htt). In vitro and in vivo models of HD and HD patients' tissues show abnormal mitochondrial function and increased cell death rates associated with alterations in Bcl-2 family protein expression and localization. This review aims to draw together the information related to Bcl-2 family protein alterations in HD to decipher their potential role in mutated htt-related cell death and mitochondrial dysfunction. Nature Publishing Group 2013-08 2013-08-15 /pmc/articles/PMC3763461/ /pubmed/23949221 http://dx.doi.org/10.1038/cddis.2013.300 Text en Copyright © 2013 Macmillan Publishers Limited http://creativecommons.org/licenses/by-nc-sa/3.0/ This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 3.0 Unported License. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-sa/3.0/
spellingShingle Review
Sassone, J
Maraschi, A
Sassone, F
Silani, V
Ciammola, A
Defining the role of the Bcl-2 family proteins in Huntington's disease
title Defining the role of the Bcl-2 family proteins in Huntington's disease
title_full Defining the role of the Bcl-2 family proteins in Huntington's disease
title_fullStr Defining the role of the Bcl-2 family proteins in Huntington's disease
title_full_unstemmed Defining the role of the Bcl-2 family proteins in Huntington's disease
title_short Defining the role of the Bcl-2 family proteins in Huntington's disease
title_sort defining the role of the bcl-2 family proteins in huntington's disease
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3763461/
https://www.ncbi.nlm.nih.gov/pubmed/23949221
http://dx.doi.org/10.1038/cddis.2013.300
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