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Amyotrophic Lateral Sclerosis - Astrophysics Data System

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Last Updated: 27 April 2022

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Body fat compartment determination by encoder-decoder convolutional neural network: application to amyotrophic lateral sclerosis

In the ALS group, the dice coefficients were 0. 87 0. 04 for SAT and 0. 64 0. 17 for VAT, respectively, and 0. 84 0. 15 for VAT. In summary, the CNN approach using CNN of U-Net architecture for automated segmentation of abdominal adipose tissue significantly simplifies data processing and gives the ability to clearly distinguish abdominal SAT and VAT compartments. The objective assessment of body fat components could lead to the approval of these parameters as a potential biological marker or a secondary read-out for clinical trials within the neuroscience field of neurodegenerative disorders with body composition changes such as ALS.

Source link: https://ui.adsabs.harvard.edu/abs/2022NatSR..12.5513V/abstract


Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis is an incurable neurodegenerative disorder that causes paralysis and death. The findings show that a partial lack of TDP-43 function is sufficient to cause neurodegeneration and ALS symptoms. In addition, we have discovered TDP-43 imbalance in human ALS. TDP-43 deficiency in the human disease leads to neurodegeneration, and future therapy should aim to restore TDP-43's normal function.

Source link: https://ui.adsabs.harvard.edu/abs/2014PNAS..111E1121Y/abstract


Metabolic signatures of amyotrophic lateral sclerosis reveal insights into disease pathogenesis

Metabolic dysfunction is a key influencer of disease course in amyotrophic lateral sclerosis. We present here that a familial mouse model of ALS contributes to a progressive state of acidosis that is associated with many metabolic shifts that favor lipolysis. ALS mice have an elevated accumulation of glycogen in CNS and visceral tissues, and they are the product of a compensatory response to avert pathological acidosis. In spinal cord tissue samples of autopsied patients with ALS, disease-related changes in glycogen, glucose, and -glucosidase enzyme activity are also present in disease-related changes in glycogen, glucose, and -glucosidase enzyme activity.

Source link: https://ui.adsabs.harvard.edu/abs/2013PNAS..11010812D/abstract


Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase

Both cell autonomous and noncell autonomous processes are involved in the motor neuron cell death caused by this mutation, as shown by this result when motor neurons were cocultured on transduced astrocytes expressing R199W. In cases of sporadic ALS and a mouse model of ALS, DAO monitors the level of D-serine, which accumulates in the spinal cord, indicating that this anomaly may be a fundamental component of ALS pathogenesis.

Source link: https://ui.adsabs.harvard.edu/abs/2010PNAS..107.7556M/abstract


Strategies for stabilizing superoxide dismutase (SOD1), the protein destabilized in the most common form of familial amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis is a disorder characterized by the death of both upper and lower motor neurons as well as by 3- to 5-yr median survival postdiagnosis. Riluzole, the only U. S. Food and Drug Administration-approved drug for the treatment of ALS, has a best, moderate, and life-long illness; therefore, new approaches are required to combat neurodegenerative disease. mutant SOD1's common ancestry and absence of signs in knockout mice, as opposed to a lack of function, suggest a "more toxic function" as opposed to a loss of function. Dimmer destabilization and dissociation as an early step in SOD1 aggregation is a common explanation for the toxicity of fALS-SOD1 variants, or the increase of toxic function. We present a program in which we chemically cross-link the SOD1 dimer with two adjacent cysteine residues on each monomer.

Source link: https://ui.adsabs.harvard.edu/abs/2010PNAS..10721394A/abstract


From the Cover: Phenotypically aberrant astrocytes that promote motoneuron damage in a model of inherited amyotrophic lateral sclerosis

However, it is also unknown if ALS progression is related to the emergence of a specific astrocytic phenotype with neurotoxic characteristics. Here, we discuss the isolation of astrocytes with distinct phenotype from primary spinal cord cultures of symptomatic rats carrying the SOD1 G93A mutation. AbA cells were based on AbA cells' demonstrated proliferative ability and lack of replicative senescence, allowing for oligoclonal cell expansion for 1 y. but, AbA cells did not have glutamate transporter 1 and the glial progenitor marker NG2 glycoprotein. Notably, AbA cells secretly produced motoneuron death with a 10-fold higher potency than neonatal SOD1 G93A astrocytes. AbA cells seem to be an as-yet unknown astrocyte population arising during ALS disease progression with unprecedented proliferative and neurotoxic capacity, and may be potential cellular targets for slowing ALS progression.

Source link: https://ui.adsabs.harvard.edu/abs/2011PNAS..10818126D/abstract


Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration

Here we created both Drosophila and mammalian models of this extended hexanucleotide repeat, and it was shown that the increased GGCC repeat RNA expression was sufficient to cause neurodegeneration. Pur as the RNA-binding protein of rGGGGCC repeats has been identified by We have found that Pur and rGGGGCC repeats interact in vitro and in vivo in a sequence-specific manner that is conserved between mammals and Drosophila.

Source link: https://ui.adsabs.harvard.edu/abs/2013PNAS..110.7778X/abstract


The Folding process of Human Profilin-1, a novel protein associated with familial amyotrophic lateral sclerosis

Human profilin-1 is a novel protein associated with a recently identified form of familial amyotrophic lateral sclerosis. The results obtained with intrinsic fluorescence and circular dichroism indicate a single phase with no changes in the corresponding signals prior to the major refolding transition. Profilin-1 is the first example of an amyloid-related protein where folding occurs in the absence of thermodynamically stable partially folded states, according to our knowledge.

Source link: https://ui.adsabs.harvard.edu/abs/2015NatSR...512332D/abstract


Clinical characteristics and prognosis of amyotrophic lateral sclerosis with autoimmune diseases

Introduction The prevalence of autoimmune disorders in amyotrophic lateral sclerosis patients has been widely reported, but no details are given about the patient's clinical phenotype. Clinical features and inflammatory biomarkers at registration were compared between ALS patients with coexisting AIDs and those without AIDs. Patients with ALS had longer onset and poorer respiratory function than controls. Before and after matching, we found no differences in overall survival of ALS patients with and without AIDs before and after matching. 8 out of ALS patients with AIDs had a history of immunotherapy and showed modestly improved longevity in comparison to those without immunotherapy, but the findings had no scientific significance. Patients with coexisting ALS and AIDs had an older onset age and reduced respiratory function, but overall survival was similar to those with pure ALS.

Source link: https://ui.adsabs.harvard.edu/abs/2022PLoSO..1766529L/abstract


Axonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis

We now find that organelle transport and axon degeneration in several animal models of amyotrophic lateral sclerosis deficits can change independently, following changes in axonal morphology and organelle transport over time. Despite long-running transport shortages, Axons can survive in the SOD G93A model of ALS: Transport inefficiencies are discovered shortly after birth, months before the onset of axon degeneration, which is based on the following results: This conclusion is based on the following findings: In the SOD G93A model of ALS: Transport shortages are evident shortly after birth. In the SOD G85R model of ALS, motor axons degenerate, but transport is unaffected, although transport is not.

Source link: https://ui.adsabs.harvard.edu/abs/2012PNAS..109.4296M/abstract

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions