Clinicopathologic characterization and abnormal autophagy of CSF1R -related leukoencephalopathy
ConclusionsOur findings support the loss-of-function and haploinsufficiency hypothesis in pathogenesis. Autophagy abnormality may play a role in the disease. Repairing or promoting the phosphorylation level of mutant CSF1R may shed light on therapeutic targets in the future. However, whether peripheral polyneuropathy potentially belongs toCSF1R-related spectrum deserves further study with longer follow-up and more patients enrolled.Trial registrationChiCTR,ChiCTR1800015295. Registered 21 March 2018. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - December 1, 2019 Category: Neurology Source Type: research
Oscillation-specific nodal alterations in early to middle stages Parkinson ’s disease
ConclusionsCoupling various oscillations could provide potentially useful information for large-scale network and progressive oscillation-specific nodal alterations were observed in PD patients across early to middle stages. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - November 14, 2019 Category: Neurology Source Type: research
Lower serum expression of miR-181c-5p is associated with increased plasma levels of amyloid-beta 1 –40 and cerebral vulnerability in normal aging
ConclusionsThese findings suggest that deregulation of serum miR-181c-5p may indicate cerebral vulnerability in late life. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - November 3, 2019 Category: Neurology Source Type: research
Transplantation of bone marrow derived macrophages reduces markers of neuropathology in an APP/PS1 mouse model
ConclusionsBM-M systemically transplanted, promote a decrease in neuroinflammation and a limited reversion of amyloid pathology. This exploratory study may support the potential of BM-M or microglia-like cell therapy and further illuminates the mechanisms of action associated with such transplants. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - October 13, 2019 Category: Neurology Source Type: research
Exploratory study on microRNA profiles from plasma-derived extracellular vesicles in Alzheimer ’s disease and dementia with Lewy bodies
ConclusionOur data suggest that plasma-EV associated miRNAs may reflect a differential profile for a given dementia-related disorder which, once validated in larger cohorts of patients, could help to improve the differential diagnosis of DLB versus AD. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - October 2, 2019 Category: Neurology Source Type: research
Bee venom phospholipase A2 ameliorates amyloidogenesis and neuroinflammation through inhibition of signal transducer and activator of transcription-3 pathway in Tg2576 mice
ConclusionThese results suggest that bvPLA2 could restore memory by inhibiting the accumulation of A β and inflammatory responses via blockage of STAT3 activity. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - October 1, 2019 Category: Neurology Source Type: research
A longitudinal assessment of retinal function and structure in the APP/PS1 transgenic mouse model of Alzheimer ’s disease
ConclusionsWe observed an age-related decline in retinal functional and structural parameters in both APP/PS1 and WT controls, however, inter-group analysis revealed that inner retinal functional and structural decline is exacerbated in APP/PS1 mice, and that retinal functional changes precede structural changes in this strain. Further studies are required to confirm whether such phenomenon occurs in humans and if studying retinal functional changes can aid-in early assessment of AD. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - September 30, 2019 Category: Neurology Source Type: research
Xenografting of human umbilical mesenchymal stem cells from Wharton ’s jelly ameliorates mouse spinocerebellar ataxia type 1
ConclusionsOur findings provide hope for a new therapeutic direction for the treatment of SCA1. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - September 4, 2019 Category: Neurology Source Type: research
Beyond the synucleinopathies: alpha synuclein as a driving force in neurodegenerative comorbidities
AbstractThe fundamental role that alpha-synuclein (aSyn) plays in the pathogenesis of neurodegenerative synucleinopathies, including Parkinson ’s disease, dementia with Lewy bodies, and multiple system atrophy, is a well-accepted fact. A wealth of experimental evidence has linked this relatively small but ubiquitously expressed protein to a plethora of cytopathologic mechanisms and suggests that aSyn may be capable of seeding the progres sive spread of synucleinopathy throughout the brain. Beyond the synucleinopathies, the abnormal deposition of aSyn is frequently seen in a variety of other neurodegenerative proteinopath...
Source: Translational Neurodegeneration - September 3, 2019 Category: Neurology Source Type: research
Longitudinal diffusion tensor magnetic resonance imaging analysis at the cohort level reveals disturbed cortical and callosal microstructure with spared corticospinal tract in the TDP-43 G298S ALS mouse model
ConclusionDTI mapping ofTDP-43G298S mice demonstrated progression in motor areas M1/M2. WBSS and TFAS are useful techniques to localizeTDP-43G298S associated alterations over time in this ALS mouse model, as a biological marker. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - August 29, 2019 Category: Neurology Source Type: research
Kallikrein-related peptidases 6 and 10 are elevated in cerebrospinal fluid of patients with Alzheimer ’s disease and associated with CSF-TAU and FDG-PET
ConclusionsKLK6 deserves further investigations as a potential biomarker of Tau pathology in AD. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - August 26, 2019 Category: Neurology Source Type: research
The interplay of aging, genetics and environmental factors in the pathogenesis of Parkinson ’s disease
ConclusionPD is a multifactorial disorder involving interplay of aging, genetics and environmental factors. This has implications on the development of appropriate animal models of PD which take all these factors into account. Common converging pathways likely include mitochondrial dysfunction, impaired autophagy, oxidative stress and neuroinflammation, which are associated with the accumulation and spread of misfolded α-synuclein and neurodegeneration. Understanding the mechanisms involved in the initiation and progression of PD may lead to potential therapeutic targets to prevent PD or modify its course. (Source: Transl...
Source: Translational Neurodegeneration - August 15, 2019 Category: Neurology Source Type: research
Efficient RT-QuIC seeding activity for α-synuclein in olfactory mucosa samples of patients with Parkinson’s disease and multiple system atrophy
ConclusionsOur study provide the proof-of-concept that olfactory mucosa samples collected from patients with PD and MSA possess important seeding activities for α-synuclein. Additional studies are required for (i) estimating sensitivity and specificity of the technique and for (ii) evaluating its application for the diagnosis of PD and neurodegenerative parkinsonisms. RT-QuIC analyses of OM and cerebrospinal fluid (CSF) can be combined with the aim of incr easing the overall diagnostic accuracy of these diseases, especially in the early stages. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - August 7, 2019 Category: Neurology Source Type: research
Parkinson ’s disease in China: a forty-year growing track of bedside work
AbstractThe number and health burden of Parkinson ’s disease increase rapidly in China. It is estimated that China will have nearly half of the Parkinson’s disease population in the world in 2030. In this review, we present an overview of epidemiology and health economics status of Parkinson’s disease across China and discuss the risk factors of Parkinson’s disease and related complications. From the view of clinical research, we also discuss the current status of clinical trials, diagnostic biomarkers, treatment of Parkinson’s disease, tertiary network and post-occupation education in Chinese Parkinson’s disea...
Source: Translational Neurodegeneration - July 30, 2019 Category: Neurology Source Type: research
Alzheimer ’s in a dish – induced pluripotent stem cell-based disease modeling
ConclusionStem cell-based disease models have the potential to induce a paradigm shift in biomedical research. In particular, the combination of the iPSC technology with recent advances in gene editing or 3D cell cultures represents a breakthrough for in vitro disease modeling and provides a platform for a better understanding of disease mechanisms in human cells and the discovery of novel therapeutics. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - July 11, 2019 Category: Neurology Source Type: research
