How to spot ocular abnormalities in progressive supranuclear palsy? A practical review
ConclusionThe authors urge all concerned physicians to check for such abnormalities with the naked eye in patients with parkinsonism. This method has advantages, including ease of application, reduced time-consumption, and requirement of minimal resources. It will also help physicians to conduct efficient diagnoses since many patients with PSP could intially present with ocular symptoms in busy outpatient clinics. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - July 9, 2019 Category: Neurology Source Type: research
Clinical features and genetic spectrum in Chinese patients with recessive hereditary spastic paraplegia
ConclusionOur results expanded the genetic spectrum and clinical profiles of AR-HSP patients and further demonstrated the efficiency and reliability of targeted NGS diagnosing suspected HSP patients. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - June 25, 2019 Category: Neurology Source Type: research
Dihydromyricetin and Salvianolic acid B inhibit alpha-synuclein aggregation and enhance chaperone-mediated autophagy
ConclusionsOur data indicate that DHM and Sal B are effective in modulating α-synuclein accumulation and aggregate formation and augmenting activation of CMA, holding potential for the treatment of Parkinson’s disease. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - June 14, 2019 Category: Neurology Source Type: research
Current progress of mitochondrial transplantation that promotes neuronal regeneration
ConclusionKey parameters for the success of mitochondrial transplantation depend on the source and quality of isolated mitochondria, delivery protocol, and cellular uptake of supplemented mitochondria. To expedite clinical application of the mitochondrial transplantation, current isolation protocol needs optimization to obtain high percentage of functional mitochondria, isolated mitochondria may be packaged by biomaterials for successful delivery to brain allowing for efficient neuronal uptake. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - June 13, 2019 Category: Neurology Source Type: research
NF- κB/c-Rel deficiency causes Parkinson’s disease-like prodromal symptoms and progressive pathology in mice
ConclusionsIn c-rel-/- mice, reproducing a parkinsonian progressive pathology with non-motor and motor symptoms, a Braak-like pattern of brain ascending α-synuclein deposition occurs. The peculiar phenotype of c-rel-/- mice envisages a potential contribution of c-Rel dysregulation to the pathogenesis of PD. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - May 20, 2019 Category: Neurology Source Type: research
Erythrocytic α-Synuclein as a potential biomarker for Parkinson’s disease
ConclusionsThese results suggest that total, aggregated and phosphorylated α-Syn levels are altered in PD erythrocytes and peripheral erythrocytic α-Syn is a potential PD biomarker that needs further validation. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - May 14, 2019 Category: Neurology Source Type: research
DNA repair deficiency in neuropathogenesis: when all roads lead to mitochondria
AbstractMutations in DNA repair enzymes can cause two neurological clinical manifestations: a developmental impairment and a degenerative disease. Polynucleotide kinase 3 ′-phosphatase (PNKP) is an enzyme that is actively involved in DNA repair in both single and double strand break repair systems. Mutations in this protein or others in the same pathway are responsible for a complex group of diseases with a broad clinical spectrum. Besides, mitochondrial dysfunctio n also has been consolidated as a hallmark of brain degeneration. Here we provide evidence that supports a shared role between mitochondrial dysfunction and D...
Source: Translational Neurodegeneration - May 8, 2019 Category: Neurology Source Type: research
No association between Parkinson disease and autoantibodies against NMDA-type glutamate receptors
ConclusionIt is unlikely albeit not impossible that NMDA antibodies play a significant role in the pathogenesis or progression of Parkinson disease e.g. to Parkinson disease with dementia, while NMDA IgG antibodies define a separate disease of its own. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - April 2, 2019 Category: Neurology Source Type: research
Cognitive decline is related to high blood glucose levels in older Chinese adults with the ApoE ε3/ε3 genotype
ConclusionWorse cognitive function was correlated withApoE ε3/ε3 genotype carriers with higher BG in community-dwelling older adults. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - April 2, 2019 Category: Neurology Source Type: research
Loss of CREST leads to neuroinflammatory responses and ALS-like motor defects in mice
ConclusionOur findings indicate that elevated inflammatory responses in a subset of ALS may be caused by neuron-derived factors, suggesting potential therapeutic methods through inflammation pathways. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - April 1, 2019 Category: Neurology Source Type: research
Correction to: Better survival in female SOD1 -mutant patients with ALS: a study of SOD1 -related natural history
AbstractIn the original publication of this article [1], a numerical value in the sentence “The mean (SD) AAO was 43.92 years (9.24) for all subjects, with a significant difference between patients carrying mutations in exon 2 (n = 24, 46.83, 8.31) and exon 4 (n = 18, 37.75, 7.67) (p = 0.002).” is wrong. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - March 18, 2019 Category: Neurology Source Type: research
Oral administration of the cannabigerol derivative VCE-003.2 promotes subventricular zone neurogenesis and protects against mutant huntingtin-induced neurodegeneration
ConclusionsThe cannabigerol derivative VCE-003.2 improves subventricular zone-derived neurogenesis in response to mutant huntingtin-induced neurodegeneration, and is neuroprotective by oral administration. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - March 7, 2019 Category: Neurology Source Type: research
Enriched gestation activates the IGF pathway to evoke embryo-adult benefits to prevent Alzheimer ’s disease
ConclusionsThese findings suggest that activation of IGF1R/CaMKIV/HAT/BDNF signaling by gestational environment enrichment may serve as a promising strategy to delay AD progression. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - March 4, 2019 Category: Neurology Source Type: research
Blocking meningeal lymphatic drainage aggravates Parkinson ’s disease-like pathology in mice overexpressing mutated α-synuclein
ConclusionThe results suggest that brain lymphatic clearance dysfunction may be an aggravating factor in PD pathology. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - March 1, 2019 Category: Neurology Source Type: research
Constipation, deficit in colon contractions and alpha-synuclein inclusions within the colon precede motor abnormalities and neurodegeneration in the central nervous system in a mouse model of alpha-synucleinopathy
ConclusionsGastrointestinal dysfunction in A53T αS mice represents an early sign of αS-driven pathology without concomitant CNS involvement. We believe that this model can be very useful to study disease-modifying strategies that could extend the prodromal phase of PD and halt αS pathology from reaching the brain. (Source: Translational Neurodegeneration)
Source: Translational Neurodegeneration - February 6, 2019 Category: Neurology Source Type: research
