BACHD rats are a model of HD expressing the full length mutant human HTT under the control of the hu... Background: Nieman-Pick type C (NPC) disease is a lysosomal storage disorder caused by mutations in NPC1 or NPC2 genes. Huntington’s disease (HD) is an inherited, autosomal, neurodegenerative disease. Transgenic animal models of synucleinopathies are frequently based on over-expressi... Alzheimer’s disease (AD) is a progressive neurodegenerative disorder that is associated with the aggregation of the amyloid β protein (Aβ). The NPC1 cerebella show a partial loss of Purkinje neurons at 7-weeks of age, while at 10-weeks the majority of Purkinje cell immunoreactivity is lost. Join Facebook to connect with Birgit Hutter and others you may know. For this reason, dependable in vitro and in vivo models... Sequential cleavage of amyloid precursor protein (APP) by β- and γ-secretases and the formation of Aβ peptides are pivotal for Alzheimer's disease. The aim of this study was to evaluate whether diffusion kurtosis imaging (DKI) will provide a sensitive tool for differentiating between α-synuclein-overexpressing transgenic mouse model of PD (TNWT-61) and wild-type (WT) l... β-Synuclein (β-Syn) is a member of the highly homologous synuclein protein family. NPC1 mouse brains show a strong immunoreactivity against GFAP indicating profound neuroinflammation, a characteristic feature of NPC disease. The relationship between β-amyloid (Aβ) and tau is not fully understood, though it is proposed that in the pathogenesis of Alzheimer's disease (AD) Aβ accumulation precedes and promotes tau hyperphosphorylation via activation of glycogen synthase kinase-3beta (GSK-3β). Ageing Res. Reducing tau phosphorylation by boosting the activity of the key phosphatase/s that mediate dephosphorylation... Cerebral accumulation of amyloid-beta (Abeta) is characteristic of Alzheimer disease and of amyloid precursor protein (APP) transgenic mice. Januar 2020 um 02:27 Uhr bearbeitet. Cell nuclei are stained by DAPI. bodyweight) and the emotional status of the animals. Beside many other variables, lipids, especially cholesterol and its derivatives, are discussed to contribute to AD pathogenesis. Only verified researchers can join ResearchGate and send messages to other members. (PDF). We found BACE1 (green) specific staining only in the mossy fibers in the hippocampus of BACE1+/+ mice. University students and faculty, institute members, and independent researchers, Technology or product developers, R&D specialists, and government or NGO employees in scientific roles, Health care professionals, including clinical researchers, Journalists, citizen scientists, or anyone interested in reading and discovering research. Bis(monoacylglycerol)phosphate (BMP) is a phospholipid that is crucial for lipid degradation and sorting in acidic organelles. In this work, we characterized reg... Astrogliosis in 10-weeks old NPC1 vs. wt mouse brains. Several studies show that cholesterol affects APP metabolism. It is intriguing that a rare, inherited lysosomal storage disorder Niemann-Pick type C (NPC) shares similarities with Alzheimer’s disease (AD). Evaluation of motor deficits in rodents is mostly restricted to limb motor tests that are often high stressors for the animals. Biochemical characterization of these T... Background: Alzheimer's disease is one of the most devastating neurodegenerative diseases of the 21 st century. Consequently, development of new compounds capable of preventing tau hyperphosphorylation is an increasingly hot topic. She is known for her work on Das weite Land (1987), The Devil's Violinist (2013) and Tabu - Es ist die Seele ein Fremdes auf Erden (2011). Representative images of glial fibrillary acidic protein (GFAP) staining of cerebellum, cortex and hippocampus.
However, many pharmacological compounds have been identified in vitro in immortalized APP overexpressing cell lines rather... Elucidating the age-dependent alterations in transgenic (Tg) mice overexpressing amyloid-β protein precursor (AβPP) is important for understanding the pathogenesis of Alzheimer's disease (AD) and designing experimental therapies. Li and colleagues summarized the most frequently used Alzheimer's disease (AD) mouse models available for drug testing and the mediating effects of the different compounds. Sorry, you need to be a researcher to join ResearchGate. Sie arbeitete als Assistentin bei Vivienne Westwood und Marc Bohan und lehrte an der Hochschule für Angewandte Kunst Wien bei Paolo Piva. Diese Seite wurde zuletzt am 7. Evidence suggests that accumulation and aggregation of α-synuclein contribute to the pathogenesis of Parkinson's disease (PD). Genetic and drug-induced lysosomal storage disorders (LSDs) are associated with increased BMP concentrations in tissues and in the circulation. Thus, their elimination is highly desirable for therapy development. Also the conve... Diffusion kurtosis imaging (DKI) by measuring non-Gaussian diffusion allows an accurate estimation of the distribution of water molecule displacement and may correctly characterize microstructural brain changes caused by neurodegeneration. Therefore, a large number of drugs has been developed targeting APP metabolism. (TIF), Microglial activation in 10-weeks old NPC1 vs. wt mouse brains. All rights reserved. en Aunt Berit's looking after the girls, to everyone's mutual delight. Both events contribute to learning and memory impairments. See full bio » With almost 300 cited publications, authors present the research community's effort of the last 10 years in finding a new drug for the treatment of AD. Deficiency in GCase activity leads to a multisystemic accumulation of substrate in lysosomes. Birgit Hutter was born on April 26, 1941 in Vienna, Austria. Symptoms of HD patients often include motor, emotional and cognitive deficits. Synucleinopathies such as Parkinson’s disease or multiple system atrophy are characterized by Lewy bodies in distinct brain areas. Birgit Hutter was born on April 26, 1941 in Vienna, Austria. View the profiles of people named Birgit Hutter. One strategy to slow disease progression is to prevent the misfolding and aggregation of its native monomeric form. The role of hyperphosphorylation of the microtubule-associated protein tau in the pathological processes of several neurodegenerative diseases is becoming better understood. (B) Latency to fall in the wire suspension test in seconds of 1.5, 9 and 12 months old TAR6 mice. Check out some of the IMDb editors' favorites movies and shows to round out your Watchlist. Beyond cognitive decline, Alzheimer’s disease (AD) is characterized by numerous neuropathological changes in the brain. A visceral and neuronal phenotype mimicking human NPC1 disease has been described in NPC1 mutant mice. (TIF), NPC1 mice show progressive neurodegeneration of cerebellar Purkinje neurons. Previously, in one of the models of NPC disease (CHO NPC1-null cells), we have shown a clear co-localizat... Background The pathology of Parkinson's disease and other synucleinopathies is characterized by the formation of intracellular inclusions comprised primarily of misfolded, fibrillar α-synuclein (α-syn). Representative images of calbindin staining in 4-, 7- and 10-weeks old NPC1 mouse cerebellum. (A) Cortical, (B) medulla oblongata, (C) spinal cord GFAP expression levels labelling reactive astrocytes and (D) CD11b expression levels labeling reactive microglia.
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