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Volume 22
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10.3390/ijms22116016
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Review

Protein Aggregation Landscape in Neurodegenerative Diseases: Clinical Relevance and Future Applications

by
Niccolò Candelise
1,2,*,
Silvia Scaricamazza
1,
Illari Salvatori
1,3,
Alberto Ferri
1,2,
Cristiana Valle
1,2,
Valeria Manganelli
3,
Tina Garofalo
3,
Maurizio Sorice
3 and
Roberta Misasi
3
1
Fondazione Santa Lucia IRCCS, c/o CERC, 00143 Rome, Italy
2
Institute of Translational Pharmacology, National Research Council, 00133 Rome, Italy
3
Department of Experimental Medicine, University of Rome “La Sapienza”, 00161 Rome, Italy
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2021, 22(11), 6016; https://doi.org/10.3390/ijms22116016
Submission received: 9 May 2021 / Revised: 28 May 2021 / Accepted: 29 May 2021 / Published: 2 June 2021
(This article belongs to the Special Issue Cell Signaling in Neurodegeneration 2.0)
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Abstract

Intrinsic disorder is a natural feature of polypeptide chains, resulting in the lack of a defined three-dimensional structure. Conformational changes in intrinsically disordered regions of a protein lead to unstable β-sheet enriched intermediates, which are stabilized by intermolecular interactions with other β-sheet enriched molecules, producing stable proteinaceous aggregates. Upon misfolding, several pathways may be undertaken depending on the composition of the amino acidic string and the surrounding environment, leading to different structures. Accumulating evidence is suggesting that the conformational state of a protein may initiate signalling pathways involved both in pathology and physiology. In this review, we will summarize the heterogeneity of structures that are produced from intrinsically disordered protein domains and highlight the routes that lead to the formation of physiological liquid droplets as well as pathogenic aggregates. The most common proteins found in aggregates in neurodegenerative diseases and their structural variability will be addressed. We will further evaluate the clinical relevance and future applications of the study of the structural heterogeneity of protein aggregates, which may aid the understanding of the phenotypic diversity observed in neurodegenerative disorders.
Keywords: intrinsic disorder; phase separation; protein aggregation; neurodegenerative disease; prion protein; alpha synuclein; TDP-43; tau; amyloid beta intrinsic disorder; phase separation; protein aggregation; neurodegenerative disease; prion protein; alpha synuclein; TDP-43; tau; amyloid beta

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MDPI and ACS Style

Candelise, N.; Scaricamazza, S.; Salvatori, I.; Ferri, A.; Valle, C.; Manganelli, V.; Garofalo, T.; Sorice, M.; Misasi, R. Protein Aggregation Landscape in Neurodegenerative Diseases: Clinical Relevance and Future Applications. Int. J. Mol. Sci. 2021, 22, 6016. https://doi.org/10.3390/ijms22116016

AMA Style

Candelise N, Scaricamazza S, Salvatori I, Ferri A, Valle C, Manganelli V, Garofalo T, Sorice M, Misasi R. Protein Aggregation Landscape in Neurodegenerative Diseases: Clinical Relevance and Future Applications. International Journal of Molecular Sciences. 2021; 22(11):6016. https://doi.org/10.3390/ijms22116016

Chicago/Turabian Style

Candelise, Niccolò, Silvia Scaricamazza, Illari Salvatori, Alberto Ferri, Cristiana Valle, Valeria Manganelli, Tina Garofalo, Maurizio Sorice, and Roberta Misasi. 2021. "Protein Aggregation Landscape in Neurodegenerative Diseases: Clinical Relevance and Future Applications" International Journal of Molecular Sciences 22, no. 11: 6016. https://doi.org/10.3390/ijms22116016

APA Style

Candelise, N., Scaricamazza, S., Salvatori, I., Ferri, A., Valle, C., Manganelli, V., Garofalo, T., Sorice, M., & Misasi, R. (2021). Protein Aggregation Landscape in Neurodegenerative Diseases: Clinical Relevance and Future Applications. International Journal of Molecular Sciences, 22(11), 6016. https://doi.org/10.3390/ijms22116016

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