Immunotherapy Promising for Slowing Progression of ALS

MONDAY, June 4, 2018 -- A new immunotherapy appears to be well tolerated and possibly of benefit in patients with amyotrophic lateral sclerosis (ALS), according to a phase 1, first-in-human study published online May 18 in Neurology: Neuroimmunology...
Source: Drugs.com - Pharma News - Category: Pharmaceuticals Source Type: news

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Authors: Pinto WBVR, Nunes P, Lima E Teixeira I, Assis ACD, Naylor FGM, Chieia MAT, Souza PVS, A S B Oliveira Abstract Atypical motor neuron disease represents a rare heterogeneous group of neurodegenerative disorders with clinical, genetic and neuroimaging features distinct from those of the classic spinal or bulbar-onset amyotrophic lateral sclerosis (ALS). O'Sullivan-McLeod syndrome represents an extremely rare lower motor neuronopathy with early adult-onset distal amyotrophy and weakness in the upper limbs with asymmetrical involvement. To add to the few case series and epidemiological and genetic studies descr...
Source: Revue Neurologique - Category: Neurology Tags: Rev Neurol (Paris) Source Type: research
AbstractAmyotrophic lateral sclerosis (ALS) is a serious neurodegenerative disease that affects motor neurons and leads to death within 2 to 3  years after the first symptoms manifest. MicroRNAs (miRNAs) are small non-coding RNA molecules that regulate gene expression in fundamental cellular processes and, post-transcriptionally, the translation levels of target mRNA transcripts. We searched PubMed for studies that examined miRNAs in ALS patients and attempted to group the results in order to find the strongest miRNA candidate for servings as an ALS biomarker. The studies on humans so far have been diverse, yielding c...
Source: Journal of Molecular Neuroscience - Category: Neuroscience Source Type: research
In this report, we analyzed 19 different cytokines and growth factors in the cerebrospinal fluid of 77 ALS patients and 13 controls by decision tree and PanelomiX program. Results showed an increase of Adipsin, MIP-1b, and IL-6, associated with a decrease of IL-8 thresholds, related with ALS patients. This biomarker panel analysis could represent an important aid for diagnosis of ALS alongside the clinical and neurophysiological criteria. PMID: 30405855 [PubMed - in process]
Source: Disease Markers - Category: Laboratory Medicine Tags: Dis Markers Source Type: research
In this study, scAAV9-hIGF-1 was intramuscularly injected into transgenic SOD1G93A mice and administered to cell lines expressing the ~25-kDa C-terminal fragment of transactive response DNA-binding protein (TDP-25). The mitochondrial electrical transmembrane potential was hyperpolarized, and electron microscopy findings revealed that the abnormal mitochondria were transformed. Moreover, the intrinsic mitochondrial apoptotic process was modified through the upregulation of anti-apoptotic proteins (B-cell lymphoma-extra large (Bcl-xl) and B-cell lymphoma-2 (Bcl-2)), the downregulation of pro-apoptotic proteins (Bcl-2-associa...
Source: Brain Research Bulletin - Category: Neurology Authors: Tags: Brain Res Bull Source Type: research
Conclusions The findings suggest that declines greater than 12% sentence intelligibility and 37 WPM are required to be outside measurement error and that these estimates vary widely across dysarthria severities. The MDC and MCID metrics used in this study to detect real and clinically relevant change should be estimated for other measures of speech outcomes in intervention research.
Source: Journal of Speech, Language, and Hearing Research - Category: Speech-Language Pathology Source Type: research
It is unclear to what extent pre-clinical studies in genetically homogeneous animal models of amyotrophic lateral sclerosis (ALS), an invariably fatal neurodegenerative disorder, can be informative of human pa...
Source: Molecular Neurodegeneration - Category: Neurology Authors: Tags: Research article Source Type: research
Publication date: Available online 5 November 2018Source: The Lancet NeurologyAuthor(s): Giancarlo Logroscino, Marco Piccininni, Benoît Marin, Emma Nichols, Foad Abd-Allah, Ahmed Abdelalim, Fares Alahdab, Solomon Weldegebreal Asgedom, Ashish Awasthi, Yazan Chaiah, Ahmad Daryani, Huyen Phuc Do, Manisha Dubey, Alexis Elbaz, Sharareh Eskandarieh, Farzaneh Farhadi, Farshad Farzadfar, Seyed-Mohammad Fereshtehnejad, Eduarda Fernandes, Irina FilipSummaryBackgroundUnderstanding how prevalence, incidence, and mortality of motor neuron diseases change over time and by location is crucial for understanding the causes of these d...
Source: The Lancet Neurology - Category: Neurology Source Type: research
A new way to deliver DNA to spinal nerve cells brings us a step closer to a gene therapy for amyotrophic lateral sclerosis
Source: New Scientist - Health - Category: Consumer Health News Source Type: research
Publication date: Available online 5 November 2018Source: MitochondrionAuthor(s): Komal Panchal, Anand Krishna TiwariAbstractNeurodegenerative diseases (NDs) are the group of disorder that includes brain, peripheral nerves, spinal cord and results in sensory and motor neuron dysfunction. Several studies have shown that mitochondrial dynamics and their axonal transport play a central role in most common NDs such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD) and Amyotrophic Lateral Sclerosis (ALS) etc. In normal physiological condition, there is a balance between mitochondrial fission and f...
Source: Mitochondrion - Category: Biochemistry Source Type: research
(Institute for Basic Science) IBS Scientists have explained how liquid-like droplets made of proteins and DNA form in vitro. Currently, there is a huge interest in understanding the molecular mechanisms behind the creation of such droplets, as it is linked to some human diseases, such as amyotrophic lateral sclerosis (ALS). The results showed how much the sequence of DNA matters in the formation of such droplets.
Source: EurekAlert! - Biology - Category: Biology Source Type: news
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