A natural history comparison of SOD1-mutant patients with amyotrophic lateral sclerosis between Chinese and German populations
(Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - October 28, 2021 Category: Neurology Source Type: research
Correction to: Neurodegenerative diseases: a hotbed for splicing defects and the potential therapies
(Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - October 25, 2021 Category: Neurology Source Type: research
Astrocyte dysfunction in Parkinson's disease: from the perspectives of transmitted α-synuclein and genetic modulation
AbstractParkinson ’s disease (PD) is a common neurodegenerative disorder that primarily affects the elderly. While the etiology of PD is likely multifactorial with the involvement of genetic, environmental, aging and other factors, α-synuclein (α-syn) pathology is a pivotal mechanism underlying the development of PD. In recent years, astrocytes have attracted considerable attention in the field. Although astrocytes perform a variety of physiological functions in the brain, they are pivotal mediators of α-syn toxicity since they internalize α-syn released from damaged neurons, and this triggers an inflamma tory respon...
Source: Translational Neurodegeneration - October 18, 2021 Category: Neurology Source Type: research
Genetic spectrum and clinical features in a cohort of Chinese patients with autosomal recessive cerebellar ataxias
ConclusionsOur results expand the genetic spectrum and clinical profiles of ARCA patients, demonstrate the high efficiency and reliability of WES combined with CNV analysis in the diagnosis of suspected ARCA, and emphasize the importance of complete bioinformatics analysis of WES data for accurate diagnosis. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - October 18, 2021 Category: Neurology Source Type: research
Novel multivalent design of a monoclonal antibody improves binding strength to soluble aggregates of amyloid beta
ConclusionWe provide a new antibody design with increased valency to promote binding avidity to an enhanced range of sizes of A β aggregates. This approach should be general and work for any aggregated protein or repetitive target. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - September 28, 2021 Category: Neurology Source Type: research
Dense core vesicle markers in CSF and cortical tissues of patients with Alzheimer ’s disease
ConclusionsThese results demonstrate marked alterations of neuronal-specific prohormone convertases in CSF and cortical tissues of AD patients. The neuronal DCV cargos are biomarker candidates for synaptic dysfunction and neurodegeneration in AD. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - September 26, 2021 Category: Neurology Source Type: research
Multi-mechanical waves against Alzheimer ’s disease pathology: a systematic review
AbstractAlzheimer ’s disease (AD) is the most common cause of dementia, affecting approximately 40 million people worldwide. The ineffectiveness of the available pharmacological treatments against AD has fostered researchers to focus on alternative strategies to overcome this challenge. Mechanical vibrations delive red in different stimulation modes have been associated with marked improvements in cognitive and physical performance in both demented and non-demented elderly. Some of the mechanical-based stimulation modalities in efforts are earlier whole-body vibration, transcranial ultrasound stimulation with microbubble...
Source: Translational Neurodegeneration - September 24, 2021 Category: Neurology Source Type: research
Disruption of the white matter structural network and its correlation with baseline progression rate in patients with sporadic amyotrophic lateral sclerosis
ConclusionDisruption of the WM structural networks in ALS is indicated by weaker small-worldness and disturbances in regions outside of the motor systems, extending the classical pathophysiological understanding of ALS as a motor disorder. The individual WM structural network matrices of ALS patients are potential neuroimaging biomarkers for the baseline disease progression in clinical practice. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - September 13, 2021 Category: Neurology Source Type: research
NMDAR-dependent somatic potentiation of synaptic inputs is correlated with β amyloid-mediated neuronal hyperactivity
ConclusionsThe somatic potentiation of synaptic inputs may represent a novel amplification mechanism that elevates cell excitability and thus contributes to neuronal hyperactivity initiated by impaired glutamate reuptake in AD. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - September 8, 2021 Category: Neurology Source Type: research
Mitochondrial links between brain aging and Alzheimer ’s disease
AbstractAdvancing age is a major risk factor for Alzheimer ’s disease (AD). This raises the question of whether AD biology mechanistically diverges from aging biology or alternatively represents exaggerated aging. Correlative and modeling studies can inform this question, but without a firm grasp of what drives aging and AD it is difficult to definitively resolve this quandary. This review speculates over the relevance of a particular hallmark of aging, mitochondrial function, to AD, and further provides background information that is pertinent to and provides perspective on this speculation. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - September 1, 2021 Category: Neurology Source Type: research
Predictability of polygenic risk score for progression to dementia and its interaction with APOE ε4 in mild cognitive impairment
ConclusionsPRS could predict the conversion of MCI to dementia with a stronger association inAPOEε4 non-carriers thanAPOEε4 carriers. This indicates PRS as a potential genetic predictor particularly for MCI with noAPOEε4 alleles. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - August 31, 2021 Category: Neurology Source Type: research
Neurofilament light and heterogeneity of disease progression in amyotrophic lateral sclerosis: development and validation of a prediction model to improve interventional trials
ConclusionThe use of the NfL-based prediction model to compensate for clinical heterogeneity in ALS could significantly increase the trial power.NCT00868166, registered March
23, 2009; NCT02306590, registered December 2, 2014. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - August 26, 2021 Category: Neurology Source Type: research
Roles of physical exercise in neurodegeneration: reversal of epigenetic clock
AbstractThe epigenetic clock is defined by the DNA methylation (DNAm) level and has been extensively applied to distinguish biological age from chronological age. Aging-related neurodegeneration is associated with epigenetic alteration, which determines the status of diseases. In recent years, extensive research has shown that physical exercise (PE) can affect the DNAm level, implying a reversal of the epigenetic clock in neurodegeneration. PE also regulates brain plasticity, neuroinflammation, and molecular signaling cascades associated with epigenetics. This review summarizes the effects of PE on neurodegenerative diseas...
Source: Translational Neurodegeneration - August 13, 2021 Category: Neurology Source Type: research
A perspective on therapies for amyotrophic lateral sclerosis: can disease progression be curbed?
AbstractAmyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper and lower motor neurons, leading to paralysis and eventually death. Symptomatic treatments such as inhibition of salivation, alleviation of muscle cramps, and relief of spasticity and pain still play an important role in enhancing the quality of life. To date, riluzole and edaravone are the only two drugs approved by the Food and Drug Administration for the treatment of ALS in a few countries. While there is adequate consensus on the modest efficacy of riluzole, there are still open questions concerning the efficacy ...
Source: Translational Neurodegeneration - August 10, 2021 Category: Neurology Source Type: research
Optimization of cerebral organoids: a more qualified model for Alzheimer ’s disease research
AbstractAlzheimer ’s disease (AD) is a neurodegenerative disease that currently cannot be cured by any drug or intervention, due to its complicated pathogenesis. Current animal and cellular models of AD are unable to meet research needs for AD. However, recent three-dimensional (3D) cerebral organoid models derived from human stem cells have provided a new tool to study molecular mechanisms and pharmaceutical developments of AD. In this review, we discuss the advantages and key limitations of the AD cerebral organoid system in comparison to the commonly used AD models, and propose possible solutions, in order to improve ...
Source: Translational Neurodegeneration - August 9, 2021 Category: Neurology Source Type: research
