Interplay between immunity and amyotrophic lateral sclerosis: Clinical impact

NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS

卷:127

Review

De Marchi, Fabiola^1,2  Munitic, Ivana³  Amedei, Amedeo⁴  Berry, James D.⁵  Feldman, Eva L.⁶  Aronica, Eleonora⁷  Nardo, Giovanni⁸  Van Weehaeghe, Donatienne⁹  Niccolai, Elena⁴  Prtenjaca, Nikolina³  Sakowski, Stacey A.⁶  Bendotti, Caterina⁸  Mazzini, Letizia^1,2 

[1] Univ Piemonte Orientale, Maggiore Carita Hosp, Dept Neurol, Corso Mazzini 18, I-28100 Novara, Italy

[2] Univ Piemonte Orientale, Maggiore Carita Hosp, ALS Ctr, Corso Mazzini 18, I-28100 Novara, Italy

[3] Univ Rijeka, Dept Biotechnol, Lab Mol Immunol, R Matejcic 2, Rijeka 51000, Croatia

[4] Univ Florence, Dept Clin & Expt Med, Florence, Italy

[5] Massachusetts Gen Hosp, Sean M Healey & AMG Ctr ALS, Dept Neurol, 165 Cambridge St,Suite 600, Boston, MA 02114 USA

[6] Univ Michigan, Dept Neurol, Ann Arbor, MI 48109 USA

[7] Univ Amsterdam, Dept Neuro Pathol, Amsterdam Neurosci, Amsterdam UMC, Meibergdreef 9, NL-1105 AZ Amsterdam, Netherlands

[8] Ist Ric Farmacol Mario Negri IRCCS, Lab Mol Neurobiol, Dept Neurosci, Via Mario Negri 2, I-20156 Milanm, Italy

[9] Katholieke Univ Leuven, Univ Hosp Leuven, Dept Imaging & Pathol, Div Nucl Med, Leuven, Belgium

关键词: Netherlands;    Immunity;    Inflammation;    Amyotrophic lateral sclerosis;    Biomarkers;    Targeted therapies;    Target;   

DOI  :  10.1016/j.neubiorev.2021.06.027

来源: Elsevier

【 摘 要 】

Amyotrophic lateral sclerosis (ALS) is a debilitating and rapidly fatal neurodegenerative disease. Despite decades of research and many new insights into disease biology over the 150 years since the disease was first described, causative pathogenic mechanisms in ALS remain poorly understood, especially in sporadic cases. Our understanding of the role of the immune system in ALS pathophysiology, however, is rapidly expanding. The aim of this manuscript is to summarize the recent advances regarding the immune system involvement in ALS, with particular attention to clinical translation. We focus on the potential pathophysiologic mechanism of the immune system in ALS, discussing local and systemic factors (blood, cerebrospinal fluid, and microbiota) that influence ALS onset and progression in animal models and people. We also explore the potential of Positron Emission Tomography to detect neuroinflammation in vivo, and discuss ongoing clinical trials of therapies targeting the immune system. With validation in human patients, new evidence in this emerging field will serve to identify novel therapeutic targets and provide realistic hope for personalized treatment strategies.

【 授权许可】

Free