scholarly journals Immature-like molecular expression patterns in the hippocampus of a mouse model of dementia with Lewy body-linked mutant β-synuclein

2018 ◽  
Vol 11 (1) ◽  
Author(s):  
Hideo Hagihara ◽  
Masayo Fujita ◽  
Juzoh Umemori ◽  
Makoto Hashimoto ◽  
Tsuyoshi Miyakawa
Viruses ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 759
Author(s):  
Jung-Youn Han ◽  
Chaewon Shin ◽  
Young Pyo Choi

In synucleinopathies such as Parkinson’s disease (PD) and dementia with Lewy body (DLB), pathological alpha-synuclein (α-syn) aggregates are found in the gastrointestinal (GI) tract as well as in the brain. In this study, using real-time quaking-induced conversion (RT-QuIC), we investigated the presence of α-syn seeding activity in the brain and colon tissue of G2-3 transgenic mice expressing human A53T α-syn. Here we show that pathological α-syn aggregates with seeding activity were present in the colon of G2-3 mice as early as 3 months old, which is in the presymptomatic stage prior to the observation of any neurological abnormalities. In contrast, α-syn seeding activity was not detectable in 3 month-old mouse brains and only identified at 6 months of age in one of three mice. In the symptomatic stage of 12 months of age, RT-QuIC seeding activity was consistently detectable in both the brain and colon of G2-3 mice. Our results indicate that the RT-QuIC assay can presymptomatically detect pathological α-syn aggregates in the colon of G2-3 mice several months prior to their detection in brain tissue.


2005 ◽  
Vol 230 (6) ◽  
pp. 366-375 ◽  
Author(s):  
Kathleen K. Sulik

The initial diagnosis of fetal alcohol syndrome (FAS) in the United States was made because of the facial features common to the first cohort of patients. This article reviews the development of an FAS mouse model whose craniofacial features are remarkably similar to those of affected humans. The model is based on short-term maternal treatment with a high dosage of ethanol at stages of pregnancy that are equivalent to Weeks 3 and 4 of human gestation. At these early stages of development, alcohol’s insult to the developing face is concurrent with that to the brain, eyes, and inner ear. That facial and central nervous system defects consistent with FAS can be induced by more “realistic” alcohol dosages as illustrated with data from an oral alcohol intake mouse model in which maternal blood alcohol levels do not exceed 200 mg/dl. The ethanol-induced pathogenesis involves apoptosis that occurs within 12 hrs of alcohol exposure in selected cell populations of Day 7, 8, and 9 mouse embryos. Experimental evidence from other species also shows that apoptosis underlies ethanol-induced malformations. With knowledge of sensitive and resistant cell populations at specific developmental stages, studies designed to identify the basis for these differing cellular responses and, therefore, to determine the primary mechanisms of ethanol’s teratogenesis are possible. For example, microarray comparisons of sensitive and resistant embryonic cell populations have been made, as have in situ studies of gene expression patterns in the populations of interest. Studies that illustrate agents that are effective in diminishing or exacerbating ethanol’s teratogenesis have also been helpful in determining mechanisms. Among these agents are antioxidants, sonic hedgehog protein, retinoids, and the peptides SAL and NAP.


2017 ◽  
Vol 13 (7S_Part_8) ◽  
pp. P414-P414
Author(s):  
Seung Ha Lee ◽  
Hanna Cho ◽  
Jae Yong Choi ◽  
Young Sook Choi ◽  
Jae Hoon Lee ◽  
...  

2020 ◽  
Vol 35 (6) ◽  
pp. 820-820
Author(s):  
Duarte A ◽  
Alcina J ◽  
Rodriguez M

Abstract The present review aimed to examine sensitivity and specificity across neuropsychological tests for Dementia with Lewy Body (DLB), to enhance diagnostic utility. A systematic search of the literature was conducted. Databases used: PsycInfo, Discovery Service for Carlos Albizu University-Miami, PsycARTICLES. Data selection criteria entailed articles from 2010–2020. Search terms included: Dementia with Lewy body, sensitivity, specificity, neuropsychological assessment, neuropsychological testing. Original search yielded 27 results, from those, only 8 articles contained sensitivity and specificity regarding DLB. The target population of review were older adults 65 and older. The assessment measures analyzed were the BSID, HVLT, MMSE QSPT, SAI, MMSE, and ICS, and others. Findings of eight studies were evaluated regarding sensitivity and specificity across neuropsychological assessments for DLB. Among the most specific and sensitive measures were the Alba Screening Instrument (ASI; sensitivity 90.7%, specificity 93.6%), and a combination of the Auditory Verbal Learning Test (AVLT) percent retention, Block Design, Trail Making Test--Part A, and Benton Visual Form Discrimination (specificity 96.1% and sensitivity 88.6%). Lower specificity and sensitivity were found in Illusory contour (ICs-4; specificity and sensitivity of 37.1% and 88.6%) and the Minimental State Examination-Pentagon Test (MMSE-QSPT; specificity 78.67% and sensitivity 70.29%). Other tests and combinations were explored. Specificity ranged from 76% to 96.1%, while sensitivity ranged from 37.1% to 90.7% throughout the neuropsychological tests examined. One major limitation across the studies constituted lack of pathological, post-mortem, confirmation. Additionally, while the use of cutoff scores across assessments appeared to enhance the sensitivity and specificity, it seemed to compromise the diagnostic accuracy of AD.


2019 ◽  
Vol 7 (2) ◽  
pp. 83-98 ◽  
Author(s):  
Stelvio Sestini ◽  
Pierpaolo Alongi ◽  
Valentina Berti ◽  
Maria Lucia Calcagni ◽  
Diego Cecchin ◽  
...  

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Diana L Castillo-Carranza

This scientific commentary refers to ‘Analysis of α-synuclein species enriched from cerebral cortex of humans with sporadic dementia with Lewy bodies’, by Sanderson et al. (https://doi.org/10.1093/braincomms/fcaa010).


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