Mitochondrial dysfunction in protein conformational disorders
Mitochondrial dysfunction in protein conformational disorders
Protein aggregation is a hallmark of many neurodegenerative diseases. In Parkinson's disease protein misfolding of α-synuclein involves conformational changes in the protein structure that often results in self-association and aggregation leading to accumulation of α-synuclein in neuronal cells. The underlying mechanisms by which aggregations can lead to impaired cellular functions are often not understood. Meanwhile, there is growing evidence that links mitochondrial dysfunction to Parkinson's disease. As both mitochondria and protein aggregation of α-synuclein have been shown to play a major role in Parkinson's disease, it seems likely that a converging mechanism exists that links the two pathways.
Animals, Humans, Mitochondria/pathology, Neurodegenerative Diseases/pathology, Parkinson Disease/pathology, Protein Conformation, alpha-Synuclein/chemistry
703-713
Brielle, Shlomi
580d39ee-c7f1-4cf0-84be-2967ee240e13
Kaganovich, Daniel
ebb13f4e-e925-4aef-88e7-ddc25ef52d8f
20 July 2018
Brielle, Shlomi
580d39ee-c7f1-4cf0-84be-2967ee240e13
Kaganovich, Daniel
ebb13f4e-e925-4aef-88e7-ddc25ef52d8f
Brielle, Shlomi and Kaganovich, Daniel
(2018)
Mitochondrial dysfunction in protein conformational disorders.
Journal of Genetics, 97 (3), .
(doi:10.1007/s12041-018-0958-0).
Abstract
Protein aggregation is a hallmark of many neurodegenerative diseases. In Parkinson's disease protein misfolding of α-synuclein involves conformational changes in the protein structure that often results in self-association and aggregation leading to accumulation of α-synuclein in neuronal cells. The underlying mechanisms by which aggregations can lead to impaired cellular functions are often not understood. Meanwhile, there is growing evidence that links mitochondrial dysfunction to Parkinson's disease. As both mitochondria and protein aggregation of α-synuclein have been shown to play a major role in Parkinson's disease, it seems likely that a converging mechanism exists that links the two pathways.
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Accepted/In Press date: 3 April 2018
Published date: 20 July 2018
Keywords:
Animals, Humans, Mitochondria/pathology, Neurodegenerative Diseases/pathology, Parkinson Disease/pathology, Protein Conformation, alpha-Synuclein/chemistry
Identifiers
Local EPrints ID: 474236
URI: http://eprints.soton.ac.uk/id/eprint/474236
ISSN: 0022-1333
PURE UUID: fb4d0b93-ee57-4067-9ab7-23d11cf8e13c
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Date deposited: 16 Feb 2023 17:53
Last modified: 17 Mar 2024 04:17
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Contributors
Author:
Shlomi Brielle
Author:
Daniel Kaganovich
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