Alteration of Motor Protein Expression Involved in Bidirectional Transport in Peripheral Blood Mononuclear Cells of Patients with Amyotrophic Lateral Sclerosis

2016 ◽  
Vol 16 (3-4) ◽  
pp. 235-244 ◽  
Author(s):  
Magdalena Kuźma-Kozakiewicz ◽  
Beata Kaźmierczak ◽  
Agnieszka Chudy ◽  
Beata Gajewska ◽  
Anna Barańczyk-Kuźma
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Protein Expression ◽  
Peripheral Blood Mononuclear Cells ◽  
Peripheral Blood ◽  
Mononuclear Cells ◽  
Motor Protein ◽  
Peripheral Blood Mononuclear ◽  
Blood Mononuclear Cells ◽  
Bidirectional Transport ◽  
Lateral Sclerosis

scholarly journals Amyotrophic Lateral Sclerosis Multiprotein Biomarkers in Peripheral Blood Mononuclear Cells

PLoS ONE ◽  
2011 ◽  
Vol 6 (10) ◽  
pp. e25545 ◽  
Author(s):  
Giovanni Nardo ◽  
Silvia Pozzi ◽  
Mauro Pignataro ◽  
Eliana Lauranzano ◽  
Giorgia Spano ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Peripheral Blood Mononuclear Cells ◽  
Peripheral Blood ◽  
Mononuclear Cells ◽  
Peripheral Blood Mononuclear ◽  
Blood Mononuclear Cells ◽  
Lateral Sclerosis

scholarly journals Decreased Mitochondrial Function, Biogenesis, and Degradation in Peripheral Blood Mononuclear Cells from Amyotrophic Lateral Sclerosis Patients as a Potential Tool for Biomarker Research

2020 ◽  
Vol 57 (12) ◽  
pp. 5084-5102
Author(s):  
Beatriz Grisolia Araujo ◽  
Luiz Felipe Souza e Silva ◽  
Jorge Luiz de Barros Torresi ◽  
Amanda Siena ◽  
Berenice Cataldo Oliveira Valerio ◽  
...  
Keyword(s):  
Gene Expression ◽  
Amyotrophic Lateral Sclerosis ◽  
Peripheral Blood Mononuclear Cells ◽  
Mitochondrial Function ◽  
Peripheral Blood ◽  
Mononuclear Cells ◽  
Peripheral Blood Mononuclear ◽  
Blood Mononuclear Cells ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Amyotrophic lateral sclerosis (ALS) is a multifactorial and progressive neurodegenerative disease of unknown etiology. Due to ALS’s unpredictable onset and progression rate, the search for biomarkers that allow the detection and tracking of its development and therapeutic efficacy would be of significant medical value. Considering that alterations of energy supply are one of ALS’s main hallmarks and that a correlation has been established between gene expression in human brain tissue and peripheral blood mononuclear cells (PBMCs), the present work investigates whether changes in mitochondrial function could be used to monitor ALS. To achieve this goal, PBMCs from ALS patients and control subjects were used; blood sampling is a quite non-invasive method and is cost-effective. Different parameters were evaluated, namely cytosolic calcium levels, mitochondrial membrane potential, oxidative stress, and metabolic compounds levels, as well as mitochondrial dynamics and degradation. Altogether, we observed lower mitochondrial calcium uptake/retention, mitochondria depolarization, and redox homeostasis deregulation, in addition to a decrease in critical metabolic genes, a diminishment in mitochondrial biogenesis, and an augmentation in mitochondrial fission and autophagy-related gene expression. All of these changes can contribute to the decreased ATP and pyruvate levels observed in ALS PBMCs. Our data indicate that PBMCs from ALS patients show a significant mitochondrial dysfunction, resembling several findings from ALS’ neural cells/models, which could be exploited as a powerful tool in ALS research. Our findings can also guide future studies on new pharmacological interventions for ALS since assessments of brain samples are challenging and represent a relevant limited strategy.

Sign in / Sign up

Export Citation Format

Share Document