Review
Biochemistry & Molecular Biology
Takashi Hosaka, Hiroshi Tsuji, Shin Kwak
Summary: A-to-I RNA editing is a critical post-transcriptional modification that can alter protein function and is implicated in the pathogenesis of neurological diseases, particularly ALS. The dysregulation of RNA editing at the Q/R site of GluA2 and the downregulation of ADAR2 in motor neurons suggest potential therapeutic targets for ALS, including Ca2+-permeable AMPA receptors. Gene therapy targeting RNA editing dysregulation has emerged as a promising therapeutic approach for ALS and other neurological diseases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Pharmacology & Pharmacy
Mohamed Z. Habib, Esraa M. Elnahas, Yasmin M. Aboul-Ela, Mai A. Ebeid, Marwa Tarek, Doaa R. Sadek, Eman A. Negm, Dina A. Abdelhakam, Sawsan Aboul-Fotouh
Summary: Several reports suggest that calcium permeable AMPA glutamate receptors with RNA hypo-editing at the GluA2 Q/R site and excitotoxicity-mediated neuronal death play a role in the pathogenesis of neurological disorders including autism spectrum disorder (ASD). This study examined the effects of risperidone treatment on Adar2 expression, GluA2 editing, oxidative/nitrosative stress, and neuronal death in a rat model of ASD. The results showed that risperidone improved behavioral deficits and increased Adar2 expression and GluA2 editing, while reducing oxidative/nitrosative stress and neurodegeneration.
EUROPEAN JOURNAL OF PHARMACOLOGY
(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.
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)
Article
Clinical Neurology
Hitoshi Aizawa, Haruhisa Kato, Koji Oba, Takuya Kawahara, Yoshihiko Okubo, Tomoko Saito, Makiko Naito, Makoto Urushitani, Akira Tamaoka, Kiyotaka Nakamagoe, Kazuhiro Ishii, Takashi Kanda, Masahisa Katsuno, Naoki Atsuta, Yasushi Maeda, Makiko Nagai, Kazutoshi Nishiyama, Hiroyuki Ishiura, Tatsushi Toda, Akihiro Kawata, Koji Abe, Ichiro Yabe, Ikuko Takahashi-Iwata, Hidenao Sasaki, Hitoshi Warita, Masashi Aoki, Gen Sobue, Hidehiro Mizusawa, Yutaka Matsuyama, Tomohiro Haga, Shin Kwak
Summary: The study evaluated the efficacy and safety of Perampanel in patients with sporadic amyotrophic lateral sclerosis (SALS). It found that Perampanel was associated with a significant decline in ALSFRS-R score, especially in the 8 mg group. Serious adverse events were more frequent in the Perampanel 8 mg group compared to the placebo group.
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.
Review
Biochemistry & Molecular Biology
Laura Lopez-Pingarron, Henrique Almeida, Marisol Soria-Aznar, Marcos C. C. Reyes-Gonzales, Maria Pilar Terron, Joaquin J. Garcia
Summary: In this review, the etiology and pathophysiological mechanisms involved in ALS were discussed. ALS can be divided into hereditary and sporadic types, and its pathophysiology is characterized by oxidative stress, glutamate excitotoxicity, and neuroinflammation. Further research is needed to improve our understanding of the etiopathogenesis of ALS and enhance patients' prognosis and survival.
CURRENT ISSUES IN MOLECULAR BIOLOGY
(2023)
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
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
Nutrition & Dietetics
Juan Jose Lopez-Gomez, Maria D. Ballesteros-Pomar, Beatriz Torres-Torres, Begona Pintor De la Maza, M. Angeles Penacho-Lazaro, Jose M. Palacio-Mures, Cristina Abreu-Padin, Antonio Lopez-Guzman, Daniel A. De Luis-Roman
Summary: The study evaluated the nutritional status of ALS patients at the beginning of specialized nutritional treatment and its influence on disease progression and survival. The results showed that patients with worse nutritional status had a lower survival median, highlighting the importance of nutritional status in ALS management.
CLINICAL NUTRITION
(2021)
Review
Clinical Neurology
Diane B. Re, Beizhan Yan, Lilian Calderon-Garciduenas, Angeline S. Andrew, Maeve Tischbein, Elijah W. Stommel
Summary: Multiple studies show that US veterans are at higher risk of developing ALS compared to civilians, possibly due to neurotoxic exposures experienced during their service. However, limited research has been done on the specific causes of ALS in veterans and biomarker-based assessment of their exposures.
JOURNAL OF NEUROLOGY
(2022)
Article
Geriatrics & Gerontology
Seonil Kim, Caroline J. Violette, Edward B. Ziff
NEUROBIOLOGY OF AGING
(2015)
Article
Neurosciences
X. -X. Peng, A. Lister, A. Rabinowitsch, R. Kolaric, S. Cabeza De Vaca, E. B. Ziff, K. D. Carr
Article
Multidisciplinary Sciences
Seonil Kim, Roseann F. Titcombe, Hong Zhang, Latika Khatri, Hiwot K. Girma, Franz Hofmann, Ottavio Arancio, Edward B. Ziff
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2015)
Article
Neurosciences
Chen Su, James D'amour, Michelle Lee, Hau-Yeuh Lin, Toby Manders, Duo Xu, Sarah E. Eberle, Yossef Goffer, Anthony H. Zou, Maisha Rahman, Edward Ziff, Robert C. Froemke, Dong Huang, Jing Wang
Article
Biochemistry & Molecular Biology
Joseph E. Pick, Latika Khatri, Matheus F. Sathler, Edward B. Ziff
Article
Medicine, Research & Experimental
Ana Claudia Piccinelli, Priscila Neder Morato, Marcelo dos Santos Barbosa, Julio Croda, Jared Sampson, Xiangpeng Kong, Elisabete Castelon Konkiewitz, Edward B. Ziff, Jaime Amaya-Farfan, Cndida Aparecida Leite Kassuya
Article
Neurosciences
Mobeen Farooq, Seonil Kim, Sunny Patel, Latika Khatri, Takuya Hikima, Margaret E. Rice, Edward B. Ziff
Article
Neurosciences
Seonil Kim, Jiayi Shou, Sinedu Abera, Edward B. Ziff
Article
Biochemistry & Molecular Biology
Wei Lu, Latika Khatri, Edward B. Ziff
JOURNAL OF BIOLOGICAL CHEMISTRY
(2014)
Review
Neurosciences
Isabella Cristina Gomes Barreto, Patricia Viegas, Edward B. Ziff, Elisabete Castelon Konkiewitz
JOURNAL OF NEUROIMMUNE PHARMACOLOGY
(2014)
Article
Behavioral Sciences
Charlotte M. Wincott, Sinedu Abera, Sarah A. Vunck, Natasha Tirko, Yoon Choi, Roseann F. Titcombe, Shannon O. Antoine, David S. Tukey, Loren M. DeVito, Franz Hofmann, Charles A. Hoeffer, Edward B. Ziff
NEUROBIOLOGY OF LEARNING AND MEMORY
(2014)
Article
Neurosciences
Ana Claudia Piccinelli, Joyce Alencar Santos, Elisabete Castelon Konkiewitz, Silvia Aparecida Oesterreich, Anelise Samara Nazari Formagio, Julio Croda, Edward Benjamim Ziff, Candida Aparecida Leite Kassuya
NUTRITIONAL NEUROSCIENCE
(2015)
Article
Neurosciences
Xing-Xiang Peng, Soledad Cabeza de Vaca, Edward B. Ziff, Kenneth D. Carr
PSYCHOPHARMACOLOGY
(2014)
Article
Neurosciences
Seonil Kim, Joseph E. Pick, Sinedu Abera, Latika Khatri, Danielle D. P. Ferreira, Matheus F. Sathler, Sage L. Morison, Franz Hofmann, Edward B. Ziff
Article
Health Policy & Services
Silmara Harumi Nomoto, Renata Marrona Praca Longhi, Bruna Paes de Barros, Julio Croda, Edward Benjamin Ziff, Elisabete Castelon Konkiewitz
AIDS CARE-PSYCHOLOGICAL AND SOCIO-MEDICAL ASPECTS OF AIDS/HIV
(2015)
Article
Neurosciences
Yang He, Jun Tang, Meng Zhang, Junjie Ying, Dezhi Mu
Summary: This study investigated the protective effects and mechanisms of human placenta derived mesenchymal stem cells (hPMSCs) transplantation in a rat model of hypoxic-ischemic encephalopathy (HIE). The results showed that hPMSCs transplantation reduced apoptosis and improved long-term neurological prognosis. Furthermore, the downregulation of Sema 3A/NRP-1 expression and activation of the PI3K/Akt/mTOR signaling pathway played a key role in the protective effects of hPMSCs.
Article
Neurosciences
Emily L. Isenstein, Edward G. Freedman, Jiayi Xu, Ian A. DeAndrea-Lazarus, John J. Foxe
Summary: This study evaluated electrophysiological discrimination of parametric somatosensory stimuli in healthy young adults to understand how the brain processes the duration of tactile information. The results showed that participants did not electrophysiologically discriminate between 100 and 115 ms, but they exhibited distinct electrophysiological responses when the deviant stimuli were 130, 145, and 160 ms. These findings contribute to a better understanding of tactile sensitivity in different clinical conditions.
Article
Neurosciences
Juliana R. Souza, Ludmila Lima-Silveira, Daniela Accorsi-Mendonca, Benedito H. Machado
Summary: This study demonstrates that A2A receptors play a crucial role in modulating synaptic transmission in the NTS neurons and are required for the enhancement of glutamatergic transmission observed under short-term sustained hypoxia conditions.
Article
Neurosciences
Miki Hashizume, Rina Ito, Rie Suge, Yasushi Hojo, Gen Murakami, Takayuki Murakoshi
Summary: The basolateral amygdaloid complex (BLA) is closely involved in the formation of emotional memories, including both aversive memory and contextual fear memory. Acute sleep deprivation (SD) disrupts the acquisition of tone-associated fear memory in juvenile rats, but has no significant effect on contextual fear memory. Slow network oscillation in the amygdala contributes to the formation of amygdala-dependent fear memory in relation to sleep.
Article
Neurosciences
Qunxian Wang, Shipeng Guo, Dongjie Hu, Xiangjun Dong, Zijun Meng, Yanshuang Jiang, Zijuan Feng, Weihui Zhou, Weihong Song
Summary: GSDME plays a crucial role in the pathogenesis of Alzheimer's disease by regulating the switch from apoptosis to pyroptosis and participating in neuroinflammatory response. Knockdown of GSDME has been shown to improve cognitive impairments, indicating that GSDME could be a therapeutic target for Alzheimer's disease.