Relationship between Liver Pathology and Disease Progression in a Murine Model of Amyotrophic Lateral Sclerosis.

CONCLUSIONS: Taken together, these findings suggest that liver dysfunction in hSOD1G93A transgenic mice is mediated by increased inflammation and oxidative stress as well as the upregulation of fibrosis-related proteins. PMID: 30130789 [PubMed - as supplied by publisher]
Source: Neuro-Degenerative Diseases - Category: Neurology Authors: Tags: Neurodegener Dis Source Type: research

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AbstractIn amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), spinal and lower brainstem motor neurons degenerate, but some motor neuron subtypes are spared, including oculomotor neurons (OMNs). The mechanisms responsible for this selective degeneration are largely unknown, but the molecular signatures of resistant and vulnerable motor neurons are distinct and offer clues to neuronal resilience and susceptibility. Here, we demonstrate that healthy OMNs preferentially expressSynaptotagmin 13 (SYT13) compared to spinal motor neurons. In end-stage ALS patients,SYT13 is enriched in both OMNs and the remaini...
Source: Acta Neuropathologica - Category: Neurology Source Type: research
Abstract Amyotrophic lateral sclerosis (ALS) is an upper motor neuron disease with an unknown pathogenesis and no effective treatment. A recent study found that treatment of a mouse model of ALS (TgSOD1 mice) intraperitoneally with the mast-cell blocker disodium chromoglycate (cromolyn) had a small but significant effect on disease onset, improvement of neurologic symptoms, and decrease in the expression of proinflammatory cytokines and chemokines in the spinal cord and plasma of the TgSOD1 mice. Treatment with cromolyn also reduced degranulation of mast cells in the tibialis anterior muscle. There was no effect o...
Source: Clinical Therapeutics - Category: Drugs & Pharmacology Authors: Tags: Clin Ther Source Type: research
CONCLUSION: In proposed Auto Regressive (AR) model has been used for Amyotrophic Lateral Sclerosis (ALS) patient data analysis. The 4th order of Yule Walker auto-regressive model is giving best fitting on this problem. PMID: 32008542 [PubMed - in process]
Source: Current Medical Imaging Reviews - Category: Radiology Tags: Curr Med Imaging Rev Source Type: research
Conclusion: This Phase 2 multi-center, randomized, placebo controlled, double blind clinical trial will provide evidence of efficacy and safety of Tecfidera in sporadic ALS.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Study Protocol Clinical Trial Source Type: research
CONCLUSION: The different expression of miRNAs and HDAC4 in genetic ALS vs. SALS and UMN cases is likely to be correlated to different pathogenic mechanisms.
. PMID: 32000889 [PubMed - as supplied by publisher]
Source: Clinical Neuropathology - Category: Pathology Authors: Tags: Clin Neuropathol Source Type: research
Abstract Amyotrophic lateral sclerosis (ALS) is characterized by progressive degeneration of motor neurons. Astrocytes from diverse ALS models induce motor neuron death in co-culture. Enhancing NAD+ availability, or increasing the expression of the NAD+-dependent deacylases SIRT3 and SIRT6, abrogates their neurotoxicity in cell culture models. To determine the effect of increasing NAD+ availability in ALS mouse models we used two strategies, ablation of a NAD+-consuming enzyme (CD38) and supplementation with a bioavailable NAD+ precursor (nicotinamide riboside, NR). Deletion of CD38 had no effect in the survival o...
Source: Experimental Neurology - Category: Neurology Authors: Tags: Exp Neurol Source Type: research
nco M Abstract The pleiotropic peptide insulin-like growth factor 1 (IGF-I) regulates human body homeostasis and cell growth. IGF-I activates two major signaling pathways, namely phosphoinositide-3-kinase (PI3K)/protein kinase B (PKB/Akt) and Ras/extracellular signal-regulated kinase (ERK), which contribute to brain development, metabolism and function as well as to neuronal maintenance and survival. In this review, we discuss the general and tissue-specific effects of the IGF-I pathways. In addition, we present a comprehensive overview examining the role of IGF-I in neurodegenerative diseases, such as spinal and ...
Source: Frontiers in Neuroendocrinology - Category: Endocrinology Authors: Tags: Front Neuroendocrinol Source Type: research
The original version of this article unfortunately contained a mistake. Oliver Hanemann name was incorrect in the in the acknowledgements section of this paper.
Source: Journal of Neurology - Category: Neurology Source Type: research
PMID: 31987064 [PubMed - as supplied by publisher]
Source: The Canadian Journal of Neurological Sciences - Category: Neurology Authors: Tags: Can J Neurol Sci Source Type: research
ConclusionsBulbar onset was a significant risk predictor for survival. Slower disease progression correlated with better outcomes. Age of onset may differ among ethnic groups. Male patients are more likely to develop Amyotrophic Lateral Sclerosis (ALS) and have shorter survival duration.
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
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