Article
Chemistry, Multidisciplinary
Michal Chojnacki, Jakub Anisiewicz, Ilona Lesniowska, Marta Kinga Lemieszek
Summary: Vitamin D3 deficiency is a pandemic with serious health consequences, including chronic respiratory diseases. Vitamin D supplementation has proven ineffective in improving this situation. This study demonstrates the usefulness and safety of delivering 1,25(OH)2-vitamin D3 and its precursor directly into the respiratory tract through nebulization. This method restores physiological calcitriol levels in animals with vitamin D deficiencies.
APPLIED SCIENCES-BASEL
(2023)
Article
Pharmacology & Pharmacy
Nuria Gaja-Capdevila, Neus Hernandez, Xavier Navarro, Mireia Herrando-Grabulosa
Summary: The study demonstrated that Sig-1R ligands, including three different drugs, can partially alleviate muscle function impairment in ALS patients and increase the number of surviving motor neurons. Furthermore, these drugs showed varying effects in improving muscle function and protecting neuromuscular junctions. Although more research is needed to determine their exact mechanisms of action, Sig-1R ligands have the potential to be promising tools for ALS treatment.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Clinical Neurology
C. Toh, A. Keslake, T. Payne, A. Onwuegbuzie, J. Harding, K. Baster, N. Hoggard, P. J. Shaw, I. D. Wilkinson, T. M. Jenkins
Summary: This study analyzed MRI data of the brain and cervical spinal cord to investigate pathophysiological hypotheses in vivo. A cranio-caudal step-change in MND patients was observed, which requires further investigation in larger cohorts.
JOURNAL OF NEUROLOGY
(2023)
Article
Neurosciences
Albert J. B. Lee, Tyler E. E. Kittel, Renaid B. B. Kim, Thao-Nguyen Bach, Tian Zhang, Cassie S. S. Mitchell
Summary: The study aimed to determine the most beneficial pathophysiological treatment targets for ALS. The results showed that treatments targeting inflammation were best at delaying disease onset, oxidative stress treatments significantly prolonged survival, and excitability treatments improved overall health status. The best pathophysiological treatment category varied with disease progression and combination treatments targeting multiple categories performed better than monotherapies at end-stage.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Neurosciences
Jayne McIntosh, Imane Mekrouda, Maryam Dashti, Claudiu V. Giuraniuc, Robert W. Banks, Gareth B. Miles, Guy S. Bewick
Summary: This study found that the ultimate deficit in ALS is the loss of neuromuscular junctions (NMJs), leading to permanent paralysis, especially in respiratory muscles. It is important to understand the functional and structural deficits at NMJs before this loss for therapeutic strategy design. The study revealed that functional deficits in NMJs appear at least 6 weeks before motor symptoms in vivo, while structural deficits occur 4 weeks later, and mainly within NMJs.
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2023)
Article
Nutrition & Dietetics
Christopher Q. Rogers, Melissa Ramirez, Carol S. Landon, Janine M. DeBlasi, Andrew P. Koutnik, Csilla Ari, Dominic P. D'Agostino
Summary: We tested the effects of three different treatments on ALS in a mouse model. The treatments were DP alone, GSP alone, and a combination of the two. Both DP and GSP alone showed beneficial effects on neurological score, strength, endurance, coordination, and lifespan, but the combination treatment did not offer additional benefits.
Review
Clinical Neurology
Riccardo Oggiano, Andrea Pisano, Angela Sabalic, Cristiano Farace, Grazia Fenu, Simone Lintas, Giovanni Forte, Beatrice Bocca, Roberto Madeddu
Summary: Cadmium, a neurotoxic heavy metal, may play a role in the onset of ALS, but the current evidence on its correlation with the disease is unclear and suggests a possible multifactorial and synergistic mechanism. More research efforts are needed to better understand the involvement of Cadmium in this lethal disease through new clinical, biochemical, and epidemiological studies.
NEUROLOGICAL SCIENCES
(2021)
Article
Cell Biology
Rebecca San Gil, Benjamin E. Clarke, Heath Ecroyd, Bernadett Kalmar, Linda Greensmith
Summary: The study examined regional variations in the expression of Hsp25 in glia of the central nervous system, revealing that Hsp25 expression is not upregulated under acute or chronic stress conditions.
Review
Clinical Neurology
Lu Xu, Bingjie He, Yunjing Zhang, Lu Chen, Dongsheng Fan, Siyan Zhan, Shengfeng Wang
Summary: This study reviewed prognostic models for ALS and found methodological pitfalls and lack of external validation by fully independent researchers. Future research should focus on adding novel predictors, external validation, and head-to-head comparisons of existing models.
JOURNAL OF NEUROLOGY
(2021)
Review
Biology
Christoph Schweingruber, Eva Hedlund
Summary: ALS is a fatal disease characterized by the loss of particular neurons called motor neurons, leading to paralysis. The reasons for the vulnerability and resilience of different motor neurons in ALS are not fully understood. Multiple cell types are involved in the disease progression and contribute to the selective vulnerability of motor neurons.
Article
Clinical Neurology
Adriaan D. de Jongh, Nathalie Braun, Markus Weber, Michael A. van Es, Pegah Masrori, Jan H. Veldink, Philip van Damme, Leonard H. van den Berg, Ruben P. A. van Eijk
Summary: This study aimed to characterize disease progression in amyotrophic lateral sclerosis (ALS) according to the Gold Coast criteria (GCC). The results showed that the GCC broadened the definition of ALS, allowing more patients to participate in trials, while minimally impacting population heterogeneity. However, there were variations in survival time and progression rates between different diagnostic categories, suggesting that selecting specific categories for trials may not result in a more homogeneous study population.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2022)
Article
Clinical Neurology
Angela Gento-Caro, Esther Vilches-Herrando, Federico Portillo, David Gonzalez-Forero, Bernardo Moreno-Lopez
Summary: The study suggests that ATX regulates motor neuron excitability and ATX inhibitors can alleviate neuron loss and motor deterioration in ALS mice. The findings indicate that ATX may be a potential therapeutic target in ALS.
Article
Chemistry, Medicinal
Kuniko Kusama-Eguchi, Yuki Tokui, Ai Minoura, Yuta Yanai, Dai Hirose, Megumi Furukawa, Yasuhiro Kosuge, Motofumi Miura, Emika Ohkoshi, Mitsuko Makino, Kimino Minagawa, Keiichi Matsuzaki, Yoshio Ogawa, Kazuko Watanabe, Ayumi Ohsaki
Summary: This study screened a domestic fungal extract library and identified two new compounds and five known compounds that showed protective activity against ALS. One of the compounds exhibited protective effects against SOD1-G93A-induced toxicity and delayed disease progression through its action on the AMPA-type glutamatergic receptor.
JOURNAL OF NATURAL MEDICINES
(2023)
Article
Geriatrics & Gerontology
Sunghee Kim, Subin An, Jinwoo Lee, Yideul Jeong, Chang-Lim You, Hyebeen Kim, Ju-Hyeon Bae, Chae-Eun Yun, Dongryul Ryu, Gyu-Un Bae, Jong-Sun Kang
Summary: The cell adhesion molecule Cdon plays a critical role in motor neuron function and nerve repair. Mice lacking Cdon exhibit middle age onset lethality and aging-related decline in motor function. Additionally, these mice also show impairment in motor function recovery and incomplete neuromuscular junction occupation.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2023)
Review
Biochemistry & Molecular Biology
Alexandra Maximova, Eryn L. Werry, Michael Kassiou
Summary: ALS is a progressive motor neurodegenerative disease with no cure, characterized by dysfunction in glial cells and motor neurons leading to neuroinflammation and degeneration. Targeting senescent glial cells may offer a new therapeutic approach to slow down motor neuron degeneration.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Public, Environmental & Occupational Health
Ban Al-Sahab, Mazen J. Hamadeh, Chris I. Ardern, Hala Tamim
AMERICAN JOURNAL OF EPIDEMIOLOGY
(2011)
Article
Public, Environmental & Occupational Health
Ban Al-Sahab, Linda Adair, Mazen J. Hamadeh, Chris I. Ardern, Hala Tamim
AMERICAN JOURNAL OF EPIDEMIOLOGY
(2011)
Article
Nutrition & Dietetics
Sharona L. Abramovitch, Jacinta I. Reddigan, Mazen J. Hamadeh, Veronica K. Jamnik, Chip P. Rowan, Jennifer L. Kuk
APPLIED PHYSIOLOGY NUTRITION AND METABOLISM
(2012)
Article
Public, Environmental & Occupational Health
Ban Al-Sahab, Chris I. Ardern, Mazen J. Hamadeh, Hala Tamim
Article
Public, Environmental & Occupational Health
Ban Al-Sahab, Chris I. Ardern, Mazen J. Hamadeh, Hala Tamim
Article
Neurosciences
Alexandro Gianforcaro, Mazen J. Hamadeh
CNS NEUROSCIENCE & THERAPEUTICS
(2012)
Article
Endocrinology & Metabolism
Michaela C. Devries, Imtiaz A. Samjoo, Mazen J. Hamadeh, Colin McCready, Sandeep Raha, Matthew J. Watt, Gregory R. Steinberg, Mark A. Tarnopolsky
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2013)
Article
Geriatrics & Gerontology
Justin D. Crane, Michaela C. Devries, Adeel Safdar, Mazen J. Hamadeh, Mark A. Tarnopolsky
JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES
(2010)
Article
Multidisciplinary Sciences
Jesse A. Solomon, Mark A. Tarnopolsky, Mazen J. Hamadeh
Article
Multidisciplinary Sciences
Jesse A. Solomon, Alexandro Gianforcaro, Mazen J. Hamadeh
Article
Multidisciplinary Sciences
Xiaoxing Ma, Mazen J. Hamadeh, Brain R. Christie, Jane A. Foster, Mark A. Tarnopolsky
Article
Multidisciplinary Sciences
Alexandro Gianforcaro, Jesse A. Solomon, Mazen J. Hamadeh
Article
Multidisciplinary Sciences
Imtiaz A. Samjoo, Adeel Safdar, Mazen J. Hamadeh, Alexander W. Glover, Nicholas J. Mocellin, Jose Santana, Jonathan P. Little, Gregory R. Steinberg, Sandeep Raha, Mark A. Tarnopolsky
Article
Endocrinology & Metabolism
I. A. Samjoo, A. Safdar, M. J. Hamadeh, S. Raha, M. A. Tarnopolsky
NUTRITION & DIABETES
(2013)