Article
Neurosciences
Minggang Fang, Sara K. Deibler, Alissa L. Nana, Sarat C. Vatsavayai, Shahid Banday, You Zhou, Sandra Almeida, Alexandra Weiss, Robert H. Brown, William W. Seeley, Fen-Biao Gao, Michael R. Green
Summary: Loss of nuclear TDP-43 function contributes to increased DNA damage in ALS and FTD patients due to defects in DNA repair pathways.
FRONTIERS IN NEUROSCIENCE
(2023)
Review
Biochemistry & Molecular Biology
Katarina Maksimovic, Mohieldin Youssef, Justin You, Hoon-Ki Sung, Jeehye Park
Summary: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects motor neurons, leading to muscle weakness, paralysis, and eventual death. Recent research has shown that ALS is not only limited to motor neurons, but also involves systemic metabolic dysfunction. This review examines the metabolic dysfunction in ALS at various levels, including muscle tissue, adipose tissue, liver, pancreas, and the central nervous system. It also discusses the future prospects of metabolic research in ALS and potential treatment options.
Article
Behavioral Sciences
Agueda Ferrer-Donato, Ana Contreras, Paloma Fernandez, Carmen M. Fernandez-Martos
Summary: The study found that leptin treatment in the ALS mouse model altered expression of adipokines and metabolic proteins, reduced weight loss decline, prolonged disease duration, and improved motor performance. This suggests that leptin could be a potential novel treatment approach for ALS.
BRAIN AND BEHAVIOR
(2022)
Article
Health Care Sciences & Services
Paul Mehta, Jaime Raymond, Moon Kwon Han, Theodore Larson, James D. Berry, Sabrina Paganoni, Hiroshi Mitsumoto, Richard Stanley Bedlack, D. Kevin Horton
Summary: The study describes how the National ALS Registry's Research Notification Mechanism (RNM) connects ALS patients with researchers. From 2013 to 2019, over 600,000 emails were sent to over 2000 patients recruited for clinical trials and studies. The highest participation was among patients aged 60-69, with more male participants (59.32%) than female participants (40.68%).
JOURNAL OF MEDICAL INTERNET RESEARCH
(2021)
Article
Clinical Neurology
Emily M. Lund, Trisha A. Hostetter, Jeri E. Forster, Claire A. Hoffmire, Kelly A. Stearns-Yoder, Lisa A. Brenner, Maryam Tahmasbi Sohi
Summary: The study found that individuals with ALS seeking care in the VHA are at increased risk of suicide, with the hazard being nearly four times higher than those without ALS. To reduce the risk of suicide, additional suicide prevention efforts, including strategies for reducing access to lethal means, are needed.
Article
Biochemistry & Molecular Biology
Agueda Ferrer-Donato, Ana Contreras, Laura M. Frago, Julie A. Chowen, Carmen M. Fernandez-Martos
Summary: The study revealed alterations in leptin signaling in the spinal cord and hypothalamus of TDP-43-induced mice, offering new evidence about the pathways linking leptin signaling to ALS.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Clinical Neurology
Georgiana Soares Leandro, Mario Emilio Teixeira Dourado Junior, Glauciane Costa Santana, Luan Samy Xavier Dantas
Summary: The main coping strategy used by ALS patients is seeking social support, while Confrontive coping and Distancing are less commonly mentioned. The coping strategies of ALS patients do not seem to focus on emotions or stress-triggering problems, and age and gender do not modify the chosen strategy.
JOURNAL OF NEUROLOGY
(2022)
Review
Clinical Neurology
Christina N. Fournier
Summary: Thoughtful clinical trial design and the use of statistical enrichment techniques are crucial for efficient therapeutic development in ALS research. Further advancements in biomarker development are needed to confirm target engagement in treatment trials. Involving patients with ALS as advisors and advocates can strengthen clinical trial design and retention. Respect for patient autonomy and improving access to experimental treatments are important challenges in ALS therapeutic development.
Review
Medicine, General & Internal
Sarah L. Boddy, Ilaria Giovannelli, Matilde Sassani, Johnathan Cooper-Knock, Michael P. Snyder, Eran Segal, Eran Elinav, Lynne A. Barker, Pamela J. Shaw, Christopher J. McDermott
Summary: The gut microbiome may play a significant role in the pathogenesis of ALS, with research indicating that microbial changes can affect disease outcomes. Variability in gut microbiota between individuals may explain the different disease outcomes seen in ALS patients.
Article
Clinical Neurology
Sebastian Michels, Deborah Kurz, Angela Rosenbohm, Raphael S. Peter, Steffen Just, Hansjörg Baezner, Axel Boertlein, Christian Dettmers, Hans-Jürgen Gold, Andreas Kohler, Markus Naumann, Peter Ratzka, Albert C. Ludolph, Dietrich Rothenbacher, Gabriele Nagel, Johannes Dorst
Summary: This large-scale population-based case-control study examined the associations between blood lipid levels and the onset and prognosis of ALS. The results showed a positive correlation between high total cholesterol levels and the risk of ALS. Furthermore, higher levels of HDL and LDL cholesterol were associated with higher mortality in ALS patients, whereas higher triglyceride levels were associated with lower mortality.
JOURNAL OF NEUROLOGY
(2023)
Article
Multidisciplinary Sciences
Radhika Khosla, Manjari Rain, Suresh Sharma, Akshay Anand
Summary: ALS is a degenerative disorder of motor neurons, and early diagnosis is crucial for predicting the disease and its progression. The study presents a predictive statistical model for ALS using plasma and CSF biomarkers, showing good validity, sensitivity, and specificity.
Review
Biochemistry & Molecular Biology
Jannigje Rachel Kok, Nelma M. Palminha, Cleide Dos Santos Souza, Sherif F. El-Khamisy, Laura Ferraiuolo
Summary: Increasing evidence supports the involvement of DNA damage in various neurodegenerative diseases, including ALS. DNA damage in ALS may vary between genetic subgroups, particularly with repeat expansion in the C9ORF72 gene. Several genes associated with ALS, such as TARDBP, FUS, NEK1, SQSTM1 and SETX, may contribute to neuronal death by affecting DNA repair and the DNA damage response.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Soju Seki, Yoshihiro Kitaoka, Sou Kawata, Akira Nishiura, Toshihiro Uchihashi, Shin-ichiro Hiraoka, Yusuke Yokota, Emiko Tanaka Isomura, Mikihiko Kogo, Susumu Tanaka
Summary: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the degeneration of motor neurons, with emerging evidence suggesting involvement of sensory neurons. Clinical research has shown sensory symptoms and abnormal nerve conduction in ALS patients, while preclinical research has identified genetic abnormalities in both sensory and motor neurons. This review highlights the potential of targeting sensory neuron abnormalities in ALS for new therapies.
Article
Environmental Sciences
Angeline Andrew, Jie Zhou, Jiang Gui, Antoinette Harrison, Xun Shi, Meifang Li, Bart Guetti, Ramaa Nathan, Maeve Tischbein, Erik Pioro, Elijah Stommel, Walter Bradley
Summary: This study used geospatial estimation to assess the exposure of ALS patients to airborne contaminants and found that airborne lead and polychlorinated biphenyls (PCBs) may be associated with an increased risk of ALS. The main sources of these contaminants include small aircraft, power plants burning biomass, and industrial boilers. The association with a residential history of lead exposure was confirmed in two additional cohorts.
SCIENCE OF THE TOTAL ENVIRONMENT
(2022)
Article
Environmental Sciences
Fan Wu, Angela M. Malek, Jeanine M. Buchanich, Vincent C. Arena, Judith R. Rager, Ravi K. Sharma, John E. Vena, Todd Bear, Evelyn O. Talbott
Summary: This study aimed to investigate the risk factors for ALS and found that high exposure to vinyl chloride, 2,4-dinitrotoluene, cyanide, cadmium, and carbon disulfide was associated with an increased risk of ALS. Additionally, residential air selenium showed an inverse association with ALS, while exposure to organic/chlorinated solvents was associated with ALS. These findings highlight the importance of ongoing surveillance of potential exposures for at-risk populations.
ENVIRONMENTAL RESEARCH
(2024)
Article
Biochemistry & Molecular Biology
Ryan G. Thys, Christine E. Lehman, Levi C. T. Pierce, Yuh-Hwa Wang
Article
Biotechnology & Applied Microbiology
Ryan G. Thys, Christine E. Lehman, Levi C. T. Pierce, Yuh-Hwa Wang
MUTATION RESEARCH-FUNDAMENTAL AND MOLECULAR MECHANISMS OF MUTAGENESIS
(2015)
Article
Biochemistry & Molecular Biology
Banabihari Giri, Philip J. Smaldino, Ryan G. Thys, Steven D. Creacy, Eric D. Routh, Roy R. Hantgan, Simon Lattmann, Yoshikuni Nagamine, Steven A. Akman, James P. Vaughn
NUCLEIC ACIDS RESEARCH
(2011)
Article
Biotechnology & Applied Microbiology
Karol Szlachta, Ryan G. Thys, Naomi D. Atkin, Levi C. T. Pierce, Stefan Bekiranov, Yuh-Hwa Wang