Frontiers in Amyotrophic Lateral Sclerosis

A special issue of Brain Sciences (ISSN 2076-3425). This special issue belongs to the section "Neurodegenerative Diseases".

Deadline for manuscript submissions: closed (28 February 2022) | Viewed by 3166

Special Issue Editors


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Guest Editor
Faculty of Medicine-IMM, University of Lisbon, 1649-028 Lisbon, Portugal
Interests: ALS; bigdata; biomarkers; electromyography; neurophysiology

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Guest Editor
Department of Neurology, Hannover Medical School, Hannover, Germany
Interests: neurodegeneration; neurological diseases; PCR; electrophysiology; neurology; neuroprotection; neurodegenerative diseases; mouse models; neurobiology; neuron

Special Issue Information

Dear Colleagues,

Amyotrophic lateral sclerosis is a progressive neurodegenerative disorder with a rapid functional decline and a short survival, but its progression rate has a large interindividual variability. The identification of biomarkers of disease progression would make it possible to improve clinical trials design and facilitate an easier identification of effective compounds. There is an extensive body of literature on neurophysiological, imaging, and neurochemical markers for investigating CSF and blood samples, cells, and tissues. Phosphorylated neurofilament heavy chain (pNfH) and the neurofilament light chain (NfL) are now considered as the most promising biomarkers. However, investigators persist in a continuous effort to find other biomarkers, as demonstrated by the emerging literature on chitinases in ALS. The impact of these different markers on clinical trials depends on their predictive value for disease outcome.

In this Special Issue we intended to collect original contributions on novel markers of disease progression and related outcomes, in the perspective of their potential interest for clinical trials. We aim to create a space for new findings, some of which will find a role in finding effective drugs for ALS.

Prof. Dr. Mamede de Carvalho
Prof. Dr. Susanne Petri
Guest Editors

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Keywords

  • amyotrophic lateral sclerosis
  • progression rate
  • biomarkers
  • neurofilaments
  • clinical trials

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Published Papers (1 paper)

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Research

15 pages, 1288 KiB  
Article
BDNF and Pro-BDNF in Amyotrophic Lateral Sclerosis: A New Perspective for Biomarkers of Neurodegeneration
by Giulia Riolo, Claudia Ricci, Nicoletta De Angelis, Carlotta Marzocchi, Gisella Guerrera, Giovanna Borsellino, Fabio Giannini and Stefania Battistini
Brain Sci. 2022, 12(5), 617; https://doi.org/10.3390/brainsci12050617 - 9 May 2022
Cited by 13 | Viewed by 2675
Abstract
Amyotrophic Lateral Sclerosis (ALS) is characterized by the progressive degeneration of upper or lower motor neurons, leading to muscle wasting and paralysis, resulting in respiratory failure and death. The precise ALS aetiology is poorly understood, mainly due to clinical and genetic heterogeneity. Thus, [...] Read more.
Amyotrophic Lateral Sclerosis (ALS) is characterized by the progressive degeneration of upper or lower motor neurons, leading to muscle wasting and paralysis, resulting in respiratory failure and death. The precise ALS aetiology is poorly understood, mainly due to clinical and genetic heterogeneity. Thus, the identification of reliable biomarkers of disease could be helpful in clinical practice. In this study, we investigated whether the levels of brain-derived neurotrophic factor (BDNF) and its precursor Pro-BDNF in serum and cerebrospinal fluid (CSF) may reflect the pathological changes related to ALS. We found higher BDNF and lower Pro-BDNF levels in ALS sera compared to healthy controls. BDNF/Pro-BDNF ratio turned out to be accurate in distinguishing ALS patients from controls. Then, the correlations of these markers with several ALS clinical variables were evaluated. This analysis revealed three statistically significant associations: (1) Patients carrying the C9orf72 expansion significantly differed from non-carrier patients and showed serum BDNF levels comparable to control subjects; (2) BDNF levels in CSF were significantly higher in ALS patients with faster disease progression; (3) lower serum levels of Pro-BDNF were associated with a shorter survival. Therefore, we suggest that BDNF and Pro-BDNF, alone or in combination, might be used as ALS prognostic biomarkers. Full article
(This article belongs to the Special Issue Frontiers in Amyotrophic Lateral Sclerosis)
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