Review
Biochemistry & Molecular Biology
Francesca Provenzano, Carola Torazza, Tiziana Bonifacino, Giambattista Bonanno, Marco Milanese
Summary: In the last two decades, increasing evidence has shown the active contribution of non-neuronal cells to neurodegenerative disorders. Among glial cells, astrocytes play a crucial role in driving the progression of amyotrophic lateral sclerosis (ALS) through various pathological mechanisms, such as neuroinflammation, mitochondrial dysfunction, oxidative stress, energy metabolism impairment, and altered release of miRNAs, extracellular vesicles, and neurotrophic factors. This review focuses on the specific role of ALS astrocytes in glutamate excitotoxicity, highlighting the molecular mechanisms by which astrocytes contribute to the increase in extracellular glutamate levels and also experience the toxic effects of excessive glutamate. The review also identifies knowledge gaps that warrant further investigation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Oncology
Qinming Zhou, Lu He, Jin Hu, Yining Gao, Dingding Shen, You Ni, Yuening Qin, Huafeng Liang, Jun Liu, Weidong Le, Sheng Chen
Summary: The role of CORO1A in ALS pathogenesis was discovered, potentially affecting the disease onset and progression by blocking autophagic flux. Therefore, CORO1A might be a potential biomarker and therapeutic target for ALS.
FRONTIERS OF MEDICINE
(2022)
Article
Neurosciences
Koh Tadokoro, Toru Yamashita, Jingwei Shang, Yasuyuki Ohta, Emi Nomura, Ryuta Morihara, Yoshio Omote, Mami Takemoto, Koji Abe
Summary: The molecular switch from the ubiquitin-proteasome system (UPS) to autophagy in ALS not only occurred in motor neurons but also in astroglia, exacerbating the production of protein aggregates.
Review
Biochemistry & Molecular Biology
Davide Bassani, Matteo Pavan, Stephanie Federico, Giampiero Spalluto, Mattia Sturlese, Stefano Moro
Summary: This article provides an overview of the role of G protein-coupled receptors (GPCRs) in the onset and progression of amyotrophic lateral sclerosis (ALS), offering useful information and insights. This is of great importance for scientists worldwide in the fight against this disease.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Chemistry, Multidisciplinary
Wei Liu, Shi-ou Zhu, Yu-lin Guo, Long-fang Tu, Yong-qi Zhen, Rong-yan Zhao, Liang Ou-Yang, Hiroshi Kurihara, Rong-Rong He, Bo Liu
Summary: Amyotrophic lateral sclerosis (ALS) is a common fatal neurodegenerative disease. The ULK1 activator BL-918 has shown therapeutic potential in treating ALS by inducing cytoprotective autophagy. This study provides further evidence of autophagic dysfunction in ALS pathogenesis.
ACTA PHARMACOLOGICA SINICA
(2023)
Article
Biochemistry & Molecular Biology
Teng Guan, Ting Zhou, Xiaosha Zhang, Ying Guo, Chaoxian Yang, Justin Lin, Jiasi Vicky Zhang, Yongquan Cheng, Hassan Marzban, Yu Tian Wang, Jiming Kong
Summary: Selective removal of misfolded SOD1 delays the onset of ALS, indicating that it is a promising treatment for the disease.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2023)
Review
Neurosciences
Xiaojiao Xu, Dingding Shen, Yining Gao, Qinming Zhou, You Ni, Huanyu Meng, Hongqin Shi, Weidong Le, Shengdi Chen, Sheng Chen
Summary: ALS is a progressive neurodegenerative disease with limited treatment options currently available. While riluzole has shown modest efficacy, there is still debate on the effectiveness of edaravone in slowing disease progression. Impaired autophagy plays a critical role in ALS pathogenesis, highlighting the need for novel therapeutic strategies.
TRANSLATIONAL NEURODEGENERATION
(2021)
Article
Clinical Neurology
Philippe Corcia, Pascal Lejeune, Patrick Vourc'h, Stephane Beltran, Anne-Sophie Piegay, Helene Blasco, Vincent Meininger
Summary: This study characterized the prototypical phenotype of patients with amyotrophic lateral sclerosis (ALS) associated with PFN1 mutations and identified clinical indications for testing mutations in this gene. The main clinical findings for familial ALS linked to PFN1 were identified as pedigrees with over five cases, an onset age around 50 years, onset in the lower limbs, and the absence of cognitive impairment. The similarities with other ALS mutations prompt a review of ALS classifications based on both phenotype and genotype.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Review
Clinical Neurology
Thomas H. Julian, Sarah Boddy, Mahjabin Islam, Julian Kurz, Katherine J. Whittaker, Tobias Moll, Calum Harvey, Sai Zhang, Michael P. Snyder, Christopher McDermott, Johnathan Cooper-Knock, Pamela J. Shaw
Summary: Mendelian randomization studies on amyotrophic lateral sclerosis show a causal link between blood lipids and the disease risk, while factors like smoking and immune function require further investigation for confirmation. The use of high methodological standards and replication across different datasets are essential for reliable results in Mendelian randomization studies.
Review
Biochemistry & Molecular Biology
Andre D. J. Mckenzie, Taylor R. Garrett, Eryn L. Werry, Michael Kassiou
Summary: This review summarizes the current understanding of amyotrophic lateral sclerosis (ALS), the role of purinergic P2X(7) receptor (P2X(7)R) in ALS, the current landscape of P2X(7)R antagonists, and their potential in preclinical ALS models.
ACS CHEMICAL NEUROSCIENCE
(2022)
Article
Biochemistry & Molecular Biology
Yevgeniya I. I. Shurubor, Alexander E. E. Rogozhin, Elena P. P. Isakova, Yulia I. I. Deryabina, Boris F. F. Krasnikov
Summary: This study examines the delayed consequences of hepatic encephalopathy (HE) on animal metabolism. The development of acute HE under the influence of thioacetamide (TAA) toxin was found to cause liver pathology, imbalance in CoA and acetyl CoA, and changes in TCA cycle metabolites. Six days after exposure to TAA, alterations in amino acids, related metabolites, glutamine transaminase (GTK) and omega-amidase enzymes were observed in vital organs. Despite apparent physiological recovery, an imbalance in amino acids and enzymes persisted, providing insight into metabolic trends for prognostic and therapeutic purposes.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Medicine, General & Internal
Can Cui, Jiangwei Sun, Kyla A. McKay, Caroline Ingre, Fang Fang
Summary: This systematic review investigated the association between medication use and ALS risk, and found no strong evidence linking any medication use with the risk of ALS.
Article
Neurosciences
Shuangwu Liu, Yuying Zhao, Qingguo Ren, Dong Zhang, Kai Shao, Pengfei Lin, Ying Yuan, Tingjun Dai, Yongqing Zhang, Ling Li, Wei Li, Peiyan Shan, Xiangshui Meng, Qian Wang, Chuanzhu Yan
Summary: This study investigated amygdala abnormalities in ALS patients, revealing distinct patterns at different clinical disease stages and highlighting their impact on anxiety and cognitive dysfunction.
HUMAN BRAIN MAPPING
(2022)
Article
Clinical Neurology
Philippe Corcia, Christian Lunetta, Philippe Couratier, Patrick Vourc'h, Marta Gromicho, Claude Desnuelle, Marie-Helene Soriani, Susana Pinto, Mamede de Carvalho
Summary: The study found that PLS and ALS cases occurred in nine families, generally among first-degree relatives. Patients with both diseases exhibited typical disease characteristics, and genetic studies revealed mutations in specific genes in some patients. These results strongly support a phenotypic continuum between PLS and ALS.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Review
Cell Biology
O. H. Houghton, S. Mizielinska, P. Gomez-Suaga
Summary: Amyotrophic lateral sclerosis and frontotemporal dementia are neurodegenerative disorders with shared genetic causes and pathology. Autophagy and RNA homeostasis play significant roles in disease pathogenesis, and there is evidence of a critical interplay between these pathways.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)