Analysis of mitochondrial protein aggregation and disaggregation.

Autor:	Voos W; Institute of Biochemistry and Molecular Biology (IBMB), Faculty of Medicine, University of Bonn, Bonn, Germany. Electronic address: wolfgang.voos@uni-bonn.de., Wilkening A; Institute of Biochemistry and Molecular Biology (IBMB), Faculty of Medicine, University of Bonn, Bonn, Germany., Ostermann R; Institute of Biochemistry and Molecular Biology (IBMB), Faculty of Medicine, University of Bonn, Bonn, Germany., Bruderek M; Institute of Biochemistry and Molecular Biology (IBMB), Faculty of Medicine, University of Bonn, Bonn, Germany., Jaworek W; Institute of Biochemistry and Molecular Biology (IBMB), Faculty of Medicine, University of Bonn, Bonn, Germany., Ruland L; Institute of Biochemistry and Molecular Biology (IBMB), Faculty of Medicine, University of Bonn, Bonn, Germany.
Jazyk:	angličtina
Zdroj:	Methods in enzymology [Methods Enzymol] 2024; Vol. 707, pp. 475-498. Date of Electronic Publication: 2024 Aug 20.
DOI:	10.1016/bs.mie.2024.07.048
Abstrakt:	Deficits of mitochondrial functions have been identified in many human pathologies, in particular in age-related human neurodegenerative diseases. Hence, the molecular causes for mitochondrial dysfunction and potential protection mechanisms have become a major topic in modern cell biology. Apart from defects in their structural integrity, problems in mitochondrial protein biogenesis, including polypeptide transport, folding and assembly to active enzymes, all may result in some degree of functional defects of the organelle. An accumulation of misfolded polypeptides inside mitochondria, confounded by the dual source of mitochondrial polypeptides, will result in the formation of protein aggregates. Such aggregate accumulation bears a cell-toxic potential, resulting in mitochondrial and correlated cellular damages, summarized in the term "aggregate proteotoxicity". Here, we discuss methods to analyze protein aggregation in the mitochondrial matrix compartment. We also address techniques to characterize the biochemical mechanisms that reduce aggregate proteotoxicity, the disaggregation or resolubilization of aggregated polypeptides and the sequestration and neutralization of mitochondrial aggregates at specific sites inside a cell. (Copyright © 2024. Published by Elsevier Inc.)
Databáze:	MEDLINE
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