Title | Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases. |
Publication Type | Journal Article |
Year of Publication | 2017 |
Authors | Kawamata H, Manfredi G |
Journal | J Cell Biol |
Volume | 216 |
Issue | 12 |
Pagination | 3917-3929 |
Date Published | 2017 Dec 04 |
ISSN | 1540-8140 |
Keywords | alpha-Synuclein, Amyloid beta-Peptides, Animals, Gene Expression Regulation, Humans, Mitochondria, Mitochondrial Proteins, Neurodegenerative Diseases, Oxidative Phosphorylation, Protein Interaction Mapping, Proteostasis Deficiencies |
Abstract | Mitochondria participate in essential processes in the nervous system such as energy and intermediate metabolism, calcium homeostasis, and apoptosis. Major neurodegenerative diseases are characterized pathologically by accumulation of misfolded proteins as a result of gene mutations or abnormal protein homeostasis. Misfolded proteins associate with mitochondria, forming oligomeric and fibrillary aggregates. As mitochondrial dysfunction, particularly of the oxidative phosphorylation system (OXPHOS), occurs in neurodegeneration, it is postulated that such defects are caused by the accumulation of misfolded proteins. However, this hypothesis and the pathological role of proteinopathies in mitochondria remain elusive. In this study, we critically review the proposed mechanisms whereby exemplary misfolded proteins associate with mitochondria and their consequences on OXPHOS. |
DOI | 10.1083/jcb.201709172 |
Alternate Journal | J. Cell Biol. |
PubMed ID | 29167179 |
PubMed Central ID | PMC5716291 |
Grant List | R01 NS062055 / NS / NINDS NIH HHS / United States R01 NS093872 / NS / NINDS NIH HHS / United States |
Pfizer COVID-19 vaccine appointments are available to our patients. Sign up for Connect today to schedule your vaccination. Continue your routine care with us by scheduling an in-person appointment or Video Visit.