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ALS Therapy

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https://www.readbyqxmd.com/read/28732762/effect-of-class-ii-hdac-inhibition-on-glutamate-transporter-expression-and-survival-in-sod1-als-mice
#1
Andrea Lapucci, Leonardo Cavone, Daniela Buonvicino, Roberta Felici, Elisabetta Gerace, Clemens Zwergel, Sergio Valente, Antonello Mai, Alberto Chiarugi
Transcriptional deregulation emerges as a key pathogenetic mechanism in ALS pathogenesis, and non-class-specific histone deacetylase (HDACs) inhibitors proved of therapeutic efficacy in preclinical models of ALS. When tested in patients, however, these drugs failed, probably because of a lack of selectivity toward pathogenetic HDACs. Here, we studied the effects of MC1568, an inhibitor of Class-II HDACs which have been reported to contribute to ALS pathogenesis. We focused on transcriptional regulation of glutamate transporter EAAT2, whose reduced expression may contribute to motor neuron degeneration in ALS...
July 18, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28711998/medical-history-of-chemotherapy-or-immunosuppressive-drug-treatment-and-risk-of-amyotrophic-lateral-sclerosis-als
#2
Thomas Kuczmarski, Elijah W Stommel, Kristen Riley, Rup Tandan, Vinay Chaudhry, Lora Clawson, Tracie A Caller, Patricia L Henegan, Dominic N Facciponte, Walter G Bradley, Angeline S Andrew
A recent population-based analysis demonstrated lower risk of the lethal degenerative neuromuscular disease, amyotrophic lateral sclerosis (ALS) associated with history of the use of 'antineoplastic agents' and 'immunosuppressants'. To see if this finding was generalizable to other ALS cohorts, we examined associations between use of these agents and ALS risk in an independent case-control study of n = 414 ALS patients and n = 361 controls in an Eastern US population. Controls were sampled from the general population and among non-neurodegenerative disease patients...
July 15, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28710685/pericytes-extend-survival-of-als-sod1-mice-and-induce-the-expression-of-antioxidant-enzymes-in-the-murine-model-and-in-ipscs-derived-neuronal-cells-from-an-als-patient
#3
Giuliana Castello Coatti, Miriam Frangini, Marcos C Valadares, Juliana Plat Gomes, Natalia O Lima, Natale Cavaçana, Amanda F Assoni, Mayra V Pelatti, Alexander Birbrair, Antonio Carlos Pedroso de Lima, Julio M Singer, Francisco Marcelo M Rocha, Giovani Loiola Da Silva, Mario Sergio Mantovani, Lucia Inês Macedo-Souza, Merari F R Ferrari, Mayana Zatz
Amyotrophic Lateral Sclerosis (ALS) is one of the most common adult-onset motor neuron disease causing a progressive, rapid and irreversible degeneration of motor neurons in the cortex, brain stem and spinal cord. No effective treatment is available and cell therapy clinical trials are currently being tested in ALS affected patients. It is well known that in ALS patients, approximately 50% of pericytes from the spinal cord barrier are lost. In the central nervous system, pericytes act in the formation and maintenance of the blood-brain barrier, a natural defense that slows the progression of symptoms in neurodegenerative diseases...
July 14, 2017: Stem Cell Reviews
https://www.readbyqxmd.com/read/28694091/combined-intranasal-nerve-growth-factor-and-ventricle-neural-stem-cell-grafts-prolong-survival-and-improve-disease-outcome-in-amyotrophic-lateral-sclerosis-transgenic-mice
#4
Shi-Jiang Zhong, Yan-Hua Gong, Yan-Chen Lin
Amyotrophic lateral sclerosis (ALS) is a fatal disease that selectively involves motor neurons. Neurotrophic factor supplementation and neural stem cell (NSC) alternative therapy have been used to treat ALS. The two approaches can affect each other in their pathways of action, and there is a possibility for synergism. However, to date, there have been no studies demonstrating the effects of combined therapy in the treatment of ALS. In this study, for the first time, we adopted a method involving the intranasal administration of nerve growth factor combined with lateral ventricle NSC transplantation using G93A-SOD1 transgenic mice as experimental subjects to explore the treatment effect of this combined therapy in ALS...
July 8, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28674982/motoneuron-disease-clinical
#5
Hristelina Ilieva, Nicholas J Maragakis
ALS is a neurodegenerative disease in which the primary symptoms result in progressive neuromuscular weakness. Recent studies have highlighted that there is significant heterogeneity with regard to anatomical and temporal disease progression. Importantly, more recent advances in genetics have revealed new causative genes to the disease. New efforts have focused on the development of biomarkers that could aid in diagnosis, prognosis, and serve as pharmacodynamics markers. Although traditional pharmaceuticals continue to undergo trials for ALS, new therapeutic strategies including stem cell transplantation studies, gene therapies, and antisense therapies targeting some of the familial forms of ALS are gaining momentum...
2017: Advances in Neurobiology
https://www.readbyqxmd.com/read/28663100/a-new-aav10-u7-mediated-gene-therapy-prolongs-survival-and-restores-function-in-an-als-mouse-model
#6
Maria Grazia Biferi, Mathilde Cohen-Tannoudji, Ambra Cappelletto, Benoit Giroux, Marianne Roda, Stéphanie Astord, Thibaut Marais, Corinne Bos, Thomas Voit, Arnaud Ferry, Martine Barkats
One of the most promising therapeutic approaches for familial amyotrophic lateral sclerosis linked to superoxide dismutase 1 (SOD1) is the suppression of toxic mutant SOD1 in the affected tissues. Here, we report an innovative molecular strategy for inducing substantial, widespread, and sustained reduction of mutant human SOD1 (hSOD1) levels throughout the body of SOD1(G93A) mice, leading to therapeutic effects in animals. Adeno-associated virus serotype rh10 vectors (AAV10) were used to mediate exon skipping of the hSOD1 pre-mRNA by expression of exon-2-targeted antisense sequences embedded in a modified U7 small-nuclear RNA (AAV10-U7-hSOD)...
June 26, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28654923/antibakterielle-in-vitro-wirksamkeit-%C3%A3-therischer-%C3%A3-le-gegen-veterin%C3%A3-rmedizinisch-relevante-keime-klinischer-isolate-von-hunden-katzen-und-pferden
#7
Doris Bismarck, Marianne Schneider, Elisabeth Müller
Einleitung: Ätherische Öle sind die Grundlage der Aromatherapie. Unter anderem wird ihnen eine antibakterielle Wirkung zugeschrieben. In dieser Studie sollte die In-vitro-Wirksamkeit ätherischer Öle gegen ein breites Spektrum veterinärmedizinisch relevanter Erreger getestet werden. Methoden: Die antibakterielle Aktivität von 16 ätherischen Ölen wurde mittels Agardiffusionstest bestimmt. Getestet wurden grampositive und gramnegative Erreger, die aus klinischen Isolaten von Hunden, Katzen und Pferden aus der veterinärmedizinischen Routinediagnostik stammten...
2017: Complementary Medicine Research
https://www.readbyqxmd.com/read/28653554/the-role-of-simulation-to-support-donation-after-circulatory-death-with-extracorporeal-membrane-oxygenation-dcd-ecmo
#8
Mateusz Puślecki, Marcin Ligowski, Marek Dąbrowski, Maciej Sip, Sebastian Stefaniak, Tomasz Kłosiewicz, Łukasz Gąsiorowski, Marek Karczewski, Tomasz Małkiewicz, Małgorzata Ładzińska, Marcin Zieliński, Aleksander Pawlak, Bartłomiej Perek, Michael Czekajlo, Marek Jemielity
Maintaining the viability of organs from donors after circulatory death (DCD) for transplantation is a complicated procedure, from a time perspective in the absence of appropriate organizational capabilities, that makes such transplantation cases difficult and not yet widespread in Poland. We present the procedural preparation for Poland's first case of organ (kidney) transplantation from a DCD donor in which perfusion was supported by extracorporeal membrane oxygenation (ECMO). Because this organizational model is complex and expensive, we used advanced high-fidelity medical simulation to prepare for the real-life implementation...
June 1, 2017: Perfusion
https://www.readbyqxmd.com/read/28642287/genetic-screening-in-sporadic-als-and-ftd
#9
EDITORIAL
Martin R Turner, Ammar Al-Chalabi, Adriano Chio, Orla Hardiman, Matthew C Kiernan, Jonathan D Rohrer, James Rowe, William Seeley, Kevin Talbot
The increasing complexity of the genetic landscape in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) presents a significant resource and physician training challenge. At least 10% of those diagnosed with ALS or FTD are known to carry an autosomal dominant genetic mutation. There is no consensus on what constitutes a positive family history, and ascertainment is unreliable for many reasons. However, symptomatic individuals often wish to understand as much as possible about the cause of their disease, and to share this knowledge with their family...
June 22, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28641540/metallothionein-is-a-potential-therapeutic-strategy-for-amyotrophic-lateral-sclerosis
#10
Shin-Ichi Ono
Lou Gehrig's disease, a synonym of amyotrophic lateral sclerosis, is an adult-onset lethal neurodegenerative disorder. Irrespective of extensive efforts to elucidate the pathogenesis of the disease and searches for therapies, no favorable pharmacotherapeutic strategies have yet to be proposed. In a popular rodent model of ALS, G93A SOD1 mice, intracellular copper conditions were geared toward copper accumulation inside cells, resulting in an acceleration of oxidative stress and apoptotic process. Disruption of intracellular copper homeostasis was common to transgenic mice expressing human mutant SOD1s...
June 22, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28641533/role-and-therapeutic-potential-of-astrocytes-in-amyotrophic-lateral-sclerosis
#11
Mariana Pehar, Benjamin A Harlan, Kelby M Killoy, Marcelo R Vargas
Amyotrophic lateral sclerosis (ALS) is characterized by the progressive degeneration of motor neurons in the spinal cord, brain stem, and motor cortex. The molecular mechanism underlying the progressive degeneration of motor neuron remains uncertain but involves a non-cell autonomous process. In acute injury or degenerative diseases astrocytes adopt a reactive phenotype known as astrogliosis. Astrogliosis is a complex remodeling of astrocyte biology and most likely represents a continuum of potential phenotypes that affect neuronal function and survival in an injury-specific manner...
June 21, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28641283/-healing-of-amyotrophic-lateral-sclerosis-a-case-report
#12
Inge Mangelsdorf, Harald Walach, Joachim Mutter
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a devastating disease leading to death within 3-5 years in most cases. New approaches to treating this disease are needed. Here, we report a successful therapy. CASE REPORT: In a 49-year-old male patient suffering from muscle weakness and fasciculations, progressive muscular atrophy, a variant of ALS, was diagnosed after extensive examinations ruling out other diseases. Due to supposed mercury exposure from residual amalgam, the patient's teeth were restored...
2017: Complementary Medicine Research
https://www.readbyqxmd.com/read/28619002/targeting-astrocytes-for-treatment-in-amyotrophic-lateral-sclerosis
#13
Mina Perić, Dinko Mitrečić, Pavle R Andjus
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder affecting upper and lower motoneurons. The two types, sporadic and familial differ in the aetiopathogenesis but have a similar neuropathology characterized by oxidative stress, excitotoxicity and inflammation. The disease is also characterized by a non-cell autonomous mechanism with astrocytes playing a central role by affecting synaptic glutamate, the blood-brain barrier, and metabolic and trophic support. Two types of therapeutic approaches focusing on astrocytes are presented: a) emerging molecular targets (potassium inward rectifier channels and aquaporins at the astrocyte endfeet, and IP3 receptor signaling pathway), and b) cell therapy with stem cell - generated and transplanted astrocytes...
June 15, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28619000/astrocytes-pathology-in-als-a-potential-therapeutic-target
#14
Sonja Johann
The mechanisms underlying neurodegeneration in amyotrophic lateral sclerosis (ALS) are multifactorial and include genetic and environmental factors. Nowadays, it is well accepted that neuronal loss is driven by non-cell autonomous toxicity. Non-neuronal cells, such as astrocytes, have been described to significantly contribute to motoneuron cell death and disease progression in cell culture experiments and animal models of ALS. Astrocytes are essential for neuronal survival and function by regulating neurotransmitter and ion homeostasis, immune response, blood flow and glucose uptake, antioxidant defence and growth factor release...
June 15, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28612258/downregulated-glia-interplay-and-increased-mirna-155-as-promising-markers-to-track-als-at-an%C3%A2-early-stage
#15
Carolina Cunha, Catarina Santos, Cátia Gomes, Adelaide Fernandes, Alexandra Marçal Correia, Ana Maria Sebastião, Ana Rita Vaz, Dora Brites
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of unknown cause. Absence of specific targets and biomarkers compromise the development of new therapeutic strategies and of innovative tools to stratify patients and assess their responses to treatment. Here, we investigate changes in neuroprotective-neuroinflammatory actions in the spinal cord of SOD1 (G93A) mice, at presymptomatic and symptomatic stages to identify stage-specific biomarkers and potential targets. Results showed that in the presymptomatic stage, there are alterations in both astrocytes and microglia, which comprise decreased expression of GFAP and S100B and upregulation of GLT-1, as well as reduced expression of CD11b, M2-phenotype markers, and a set of inflammatory mediators...
June 13, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28611587/downregulation-of-microrna-193b-3p-promotes-autophagy-and-cell-survival-by-targeting-tsc1-mtor-signaling-in-nsc-34-cells
#16
ChunYu Li, YongPing Chen, XuePing Chen, QianQian Wei, Bei Cao, HuiFang Shang
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the death of upper and lower motor neurons. MicroRNAs (miRNAs) are reported to be closely related to the development of ALS. However, the precise functions of miRNAs in the pathogenesis of ALS remain largely unknown. In previous studies, we determined that miRNA-193b-3p was significantly downregulated in patients with sporadic ALS (sALS). Here, we observed that miRNA-193b-3p was downregulated in the SOD1(G93A) mouse model of ALS and promoted cell death in NSC-34 cells...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28599400/the-use-of-mesenchymal-stem-cells-mscs-for-amyotrophic-lateral-sclerosis-als-therapy-a-perspective-on-cell-biological-mechanisms
#17
Bor Luen Tang
Recent clinical trials of mesenchymal stem cells (MSCs) transplantation have demonstrated procedural safety and clinical proof of principle with a modest indication of benefit in patients with amyotrophic lateral sclerosis (ALS). While replacement therapy remained unrealistic, the clinical efficacy of this therapeutic option could be potentially enhanced if we could better decipher the mechanisms underlying some of the beneficial effects of transplanted cells, and work toward augmenting or combining these in a strategic manner...
May 25, 2017: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/28596290/yeast-genetic-interaction-screen-of-human-genes-associated-with-amyotrophic-lateral-sclerosis-identification-of-map2k5-kinase-as-a-potential-drug-target
#18
Myungjin Jo, Ah Young Chung, Nozomu Yachie, Minchul Seo, Hyejin Jeon, Youngpyo Nam, Yeojin Seo, Eunmi Kim, Quan Zhong, Marc Vidal, Hae Chul Park, Frederick Roth, Kyoungho Suk
To understand disease mechanisms, a large-scale analysis of human-yeast genetic interactions was performed. Of 1,305 human disease genes assayed, 20 genes exhibited strong toxicity in yeast. Human-yeast genetic interactions were identified by en masse transformation of the human disease genes into a pool of 4,653 homozygous diploid yeast deletion mutants with unique barcode sequences, followed by multiplexed barcode sequencing of yeast toxicity modifiers. Subsequent network analyses focusing on amyotrophic lateral sclerosis (ALS)-associated genes, such as optineurin (OPTN) and angiogenin (ANG), showed that the human orthologs of the yeast toxicity modifiers of these ALS genes are enriched for several biological processes, such as cell death, lipid metabolism, and molecular transport...
June 8, 2017: Genome Research
https://www.readbyqxmd.com/read/28573364/-genetic-architecture-of-amyotrophic-lateral-sclerosis-and-frontotemporal-dementia-overlap-and-differences
#19
REVIEW
M Synofzik, M Otto, A Ludolph, J H Weishaupt
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) overlap not only clinically, but also with respect to shared neuropathology and genes. A large number of novel genes has recently been identified which underlie both diseases, e. g., C9orf72, TARDBP, GRN, TBK1, UBQLN2, VCP, CHCHD10, or SQSTM1. In contrast, other genes are still largely associated with only one of the two diseases, e. g., SOD1 with ALS or MAPT with FTD. These genetic findings indicate a large number of shared mechanisms, yet along with still a certain cell-specific vulnerability...
June 1, 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/28566164/cervical-spinal-cord-and-motor-unit-pathology-in-a-canine-model-of-sod1-associated-amyotrophic-lateral-sclerosis
#20
Martin L Katz, Cheryl A Jensen, Jeffrey T Student, Gayle C Johnson, Joan R Coates
Development of effective treatments for amyotrophic lateral sclerosis (ALS) would be facilitated by identification of early events in the pathological cascade of disease progression. Degenerative myelopathy (DM), a naturally occurring disease in dogs, is quite similar to forms of ALS associated with SOD1 mutations and is likely to be a good model for these forms of the human disease. The sequence of histopathological changes that occur in DM was characterized by analyzing tissue samples obtained from affected dogs euthanized at various stages of disease progression...
July 15, 2017: Journal of the Neurological Sciences
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