Emerging Trends in the Field of Inflammation and Proteinopathy in ALS/FTD Spectrum Disorder

Author:

De Marchi Fabiola1ORCID,Franjkic Toni23,Schito Paride4ORCID,Russo Tommaso4,Nimac Jerneja56,Chami Anna A.7ORCID,Mele Angelica1,Vidatic Lea8,Kriz Jasna7ORCID,Julien Jean-Pierre7,Apic Gordana3,Russell Robert B.9ORCID,Rogelj Boris510,Cannon Jason R.1112,Baralle Marco13,Agosta Federica14,Hecimovic Silva8ORCID,Mazzini Letizia1,Buratti Emanuele15ORCID,Munitic Ivana2ORCID

Affiliation:

1. Department of Neurology and ALS Centre, University of Piemonte Orientale, Maggiore Della Carità Hospital, Corso Mazzini 18, 28100 Novara, Italy

2. Laboratory for Molecular Immunology, Department of Biotechnology, University of Rijeka, R. Matejcic 2, 51000 Rijeka, Croatia

3. Metisox, Cambridge CB24 9NL, UK

4. Department of Neurology & Neuropathology Unit, Institute of Experimental Neurology (INSPE), Division of Neuroscience, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy

5. Department of Biotechnology, Jozef Stefan Institute, SI-1000 Ljubljana, Slovenia

6. Graduate School of Biomedicine, Faculty of Medicine, University of Ljubljana, SI-1000 Ljubljana, Slovenia

7. CERVO Research Centre, Laval University, Quebec City, QC G1J 2G3, Canada

8. Laboratory for Neurodegenerative Disease Research, Division of Molecular Medicine, Ruder Boskovic Institute, 10000 Zagreb, Croatia

9. Cell Networks, University of Heidelberg, 69117 Heidelberg, Germany

10. Faculty of Chemistry and Chemical Technology, University of Ljubljana, SI-1000 Ljubljana, Slovenia

11. School of Health Sciences, Purdue University, West Lafayette, IN 47907, USA

12. Purdue Institute for Integrative Neuroscience, Purdue University, West Lafayette, IN 47907, USA

13. RNA Biology, ICGEB, 34149 Trieste, Italy

14. Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy

15. International Centre for Genetic Engineering and Biotechnology (ICGEB), Padriciano 99, 34149 Trieste, Italy

Abstract

Proteinopathy and neuroinflammation are two main hallmarks of neurodegenerative diseases. They also represent rare common events in an exceptionally broad landscape of genetic, environmental, neuropathologic, and clinical heterogeneity present in patients. Here, we aim to recount the emerging trends in amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration (FTD) spectrum disorder. Our review will predominantly focus on neuroinflammation and systemic immune imbalance in ALS and FTD, which have recently been highlighted as novel therapeutic targets. A common mechanism of most ALS and ~50% of FTD patients is dysregulation of TAR DNA-binding protein 43 (TDP-43), an RNA/DNA-binding protein, which becomes depleted from the nucleus and forms cytoplasmic aggregates in neurons and glia. This, in turn, via both gain and loss of function events, alters a variety of TDP-43-mediated cellular events. Experimental attempts to target TDP-43 aggregates or manipulate crosstalk in the context of inflammation will be discussed. Targeting inflammation, and the immune system in general, is of particular interest because of the high plasticity of immune cells compared to neurons.

Funder

Croatian Science Foundation

University of Rijeka

AGING Project for the Department of Excellence at the Department of Translational Medicine (DIMET), Università del Pie-monte Orientale, Novara, Italy

Slovenian Research Agency

Croatian Science Foundation project neuroNiPiC

Hubert Curien “COGITO” programme

Publisher

MDPI AG

Subject

General Biochemistry, Genetics and Molecular Biology,Medicine (miscellaneous)

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