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Showing 1-30 of 50 results for "AB5038" within Papers
Aya Murakami et al.
Neuropathology : official journal of the Japanese Society of Neuropathology, 41(5), 357-365 (2021-07-27)
We describe an autopsy case of neuronal intermediate filament inclusion disease (NIFID), a subtype of frontotemporal lobar degeneration (FTLD) with the appearance of fused-in-sarcoma (FUS) inclusions (FTLD-FUS). A 57-year-old man developed dysarthria and dysphagia. One year and five months later, he
Belén Ansoleaga et al.
Journal of neuropathology and experimental neurology, 75(8), 755-769 (2016-06-15)
Neuron loss, synaptic decline, and spongiform change are the hallmarks of sporadic Creutzfeldt-Jakob disease (sCJD), and may be related to deficiencies in mitochondria, energy metabolism, and protein synthesis. To investigate these relationships, we determined the expression levels of genes encoding
Timothy K Cooper et al.
Veterinary pathology, 54(5), 851-854 (2017-06-06)
Intensely eosinophilic and glassy intracytoplasmic inclusions were present in the neurons of the peripheral autonomic ganglia, Meissner's and Auerbach's plexus, and spinal ganglia in 20 aged white-nosed coatis ( Nasua narica, 7-19 years old) and in 4 of 7 brown-nosed
Anders Björklund et al.
Journal of Parkinson's disease, 12(8), 2307-2320 (2022-10-04)
Injections of pre-formed α-synuclein fibrils (PFFs) or overexpression of α-synuclein using AAV vectors are commonly used as models of Parkinson-like synucleinopathy in rats and mice. In the modified method reviewed here, the "SynFib" model, the PFFs and the AAV vector
Mingshu Mo et al.
Oncotarget, 8(1), 15-28 (2016-12-15)
α-synuclein gene mutations can cause α-synuclein protein aggregation in the midbrain of Parkinson's disease (PD) patients. MicroRNAs (miRNAs) play a key role in the metabolism of α-synuclein but the mechanism involved in synucleinopathy remains unclear. In this study, we investigated
Kiren Ubhi et al.
The European journal of neuroscience, 39(6), 1026-1041 (2013-12-07)
MicroRNA (miRNA) are short sequences of RNA that function as post-transcriptional regulators by binding to target mRNA transcripts resulting in translational repression. A number of recent studies have identified miRNA as being involved in neurodegenerative disorders including Alzheimer's disease, Parkinson's
Neuropathology in mice expressing mouse alpha-synuclein.
Rieker, C; Dev, KK; Lehnhoff, K; Barbieri, S; Ksiazek, I; Kauffmann, S; Danner, S; Schell et al.
Testing null
Shinji Higashi et al.
Journal of neuropathology and experimental neurology, 70(4), 264-280 (2011-03-18)
There is emerging evidence implicating a role for the autophagy-lysosome pathway in the pathogenesis of Lewy body disease. We investigated potential neuropathologic and biochemical alterations of autophagy-lysosome pathway-related proteins in the brains of patients with dementia with Lewy bodies (DLB)
Rosa Vaccaro et al.
The Journal of comparative neurology, 523(7), 1095-1124 (2014-12-10)
Alpha synuclein (α-syn) is a 140 amino acid vertebrate-specific protein, highly expressed in the human nervous system and abnormally accumulated in Parkinson's disease and other neurodegenerative disorders, known as synucleinopathies. The common occurrence of α-syn aggregates suggested a role for
Martí Colom-Cadena et al.
Brain : a journal of neurology, 140(12), 3204-3214 (2017-11-28)
Dementia with Lewy bodies is characterized by the accumulation of Lewy bodies and Lewy neurites in the CNS, both of which are composed mainly of aggregated α-synuclein phosphorylated at Ser129. Although phosphorylated α-synuclein is believed to exert toxic effects at
TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis.
Cykowski, MD; Takei, H; Schulz, PE; Appel, SH; Powell, SZ
Acta Neuropathologica Communications null
Martin Regensburger et al.
Brain structure & function, 223(3), 1357-1368 (2017-11-11)
In the adult mammalian hippocampus, new neurons are constantly added to the dentate gyrus. Adult neurogenesis is impaired in several neurodegenerative mouse models including α-synuclein (a-syn) transgenic mice. Among different a-syn species, a-syn oligomers were reported to be the most
Anne-Sophie Van Rompuy et al.
Molecular neurodegeneration, 10, 23-23 (2015-06-24)
Alpha-synuclein is a key protein in the pathogenesis of Parkinson's disease. Mutations in the parkin gene are the most common cause of early-onset autosomal recessive Parkinson's disease, probably through a loss-of-function mechanism. However, the molecular mechanism by which loss of
Keiichi Inoue et al.
Molecular neurodegeneration, 7, 48-48 (2012-09-25)
Macroautophagy is an evolutionarily conserved mechanism for bulk intracellular degradation of proteins and organelles. Pathological studies have implicated macroautophagy defects in human neurodegenerative disorders of aging including Alzheimer's disease and tauopathies. Neuronal deficiency of macroautophagy throughout mouse embryonic development results
Dora Games et al.
The American journal of pathology, 182(3), 940-953 (2013-01-15)
Progressive accumulation of α-synuclein (α-syn) in limbic and striatonigral systems is associated with the neurodegenerative processes in dementia with Lewy bodies (DLB) and Parkinson's disease (PD). The murine Thy-1 (mThy1)-α-syn transgenic (tg) model recapitulates aspects of degenerative processes associated with
Fang Cheng et al.
Glycobiology, 32(4), 333-342 (2021-12-24)
In Parkinson's disease (PD), there is accumulation of α-synuclein (SYN) aggregates in neurons, which is promoted by neuroinflammation. The cytokines TNF-α, IL-1β and IL-6 induce accumulation of degradation products of the amyloid precursor protein (APP) combined with heparan sulfate (HS)
Junghyun Jo et al.
Annals of neurology, 90(3), 490-505 (2021-07-22)
We utilized human midbrain-like organoids (hMLOs) generated from human pluripotent stem cells carrying glucocerebrosidase gene (GBA1) and α-synuclein (α-syn; SNCA) perturbations to investigate genotype-to-phenotype relationships in Parkinson disease, with the particular aim of recapitulating α-syn- and Lewy body-related pathologies and
Wendy Westbroek et al.
Disease models & mechanisms, 9(7), 769-778 (2016-08-03)
Glucocerebrosidase is a lysosomal hydrolase involved in the breakdown of glucosylceramide. Gaucher disease, a recessive lysosomal storage disorder, is caused by mutations in the gene GBA1 Dysfunctional glucocerebrosidase leads to accumulation of glucosylceramide and glycosylsphingosine in various cell types and
Wei Zhang et al.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 19(6), 533-542 (2005-03-26)
A growing body of evidence indicates that an inflammatory process in the substantia nigra, characterized by activation of resident microglia, likely either initiates or aggravates nigral neurodegeneration in Parkinson's disease (PD). To study the mechanisms by which nigral microglia are
Rommani Mondal et al.
Frontiers in neuroscience, 15, 676257-676257 (2021-07-03)
Transgenic mice line M83 that express the A53T mutant α-synuclein protein at six times the level of endogenous mice α-synuclein are a model of α-synucleinopathy found in Parkinson's disease (PD). This Hualpha-Syn (A53T) PD model is useful in assessing non-motor
Connor Bargar et al.
Molecular neurodegeneration, 16(1), 82-82 (2021-12-14)
Detection of the pathological and disease-associated alpha-synuclein (αSynD) in the brain is required to formulate the definitive diagnosis of multiple system atrophy (MSA) and Parkinson's disease (PD). We recently showed that αSynD can be detected in the olfactory mucosa (OM)
Vincenzo Donadio et al.
Annals of neurology, 79(2), 306-316 (2015-11-26)
To characterize the expression in skin nerves of native (n-syn) and misfolded phosphorylated (p-syn) α-synucleins in pure autonomic failure (PAF) and idiopathic Parkinson disease (IPD). The specific aims were to (1) define the importance of n-syn and p-syn as disease
Shino Magaki et al.
Neurobiology of aging, 70, 70-77 (2018-07-15)
Cerebral amyloid angiopathy (CAA), in which amyloid accumulates predominantly in the walls of arterioles and capillaries, is seen in most patients with Alzheimer disease (AD) and may contribute to compromise of blood-brain barrier (BBB) function seen in AD. We investigated
Martin J Dahl et al.
Neurobiology of aging, 112, 39-54 (2022-01-20)
Abnormally phosphorylated tau, an indicator of Alzheimer's disease, accumulates in the first decades of life in the locus coeruleus (LC), the brain's main noradrenaline supply. However, technical challenges in in-vivo assessments have impeded research into the role of the LC
Adrian Flierl et al.
PloS one, 9(11), e112413-e112413 (2014-11-13)
Parkinson disease (PD) is a multi-factorial neurodegenerative disorder with loss of dopaminergic neurons in the substantia nigra and characteristic intracellular inclusions, called Lewy bodies. Genetic predisposition, such as point mutations and copy number variants of the SNCA gene locus can
Renata Areza-Fegyveres et al.
Arquivos de neuro-psiquiatria, 65(3B), 830-833 (2007-10-24)
A 61-year-old ex-boxer presented with a three-year history of progressive memory decline. During a seven-year follow-up period, there was a continuous cognitive decline, very similar to that usually observed in Alzheimer's disease. Parkinsonian, pyramidal or cerebellar signs were conspicuously absent.
Chiara Maria Giulia De Luca et al.
Translational neurodegeneration, 8, 24-24 (2019-08-14)
Parkinson's disease (PD) is a neurodegenerative disorder whose diagnosis is often challenging because symptoms may overlap with neurodegenerative parkinsonisms. PD is characterized by intraneuronal accumulation of abnormal α-synuclein in brainstem while neurodegenerative parkinsonisms might be associated with accumulation of either
Serine 129 phosphorylation of alpha-synuclein induces unfolded protein response-mediated cell death.
Sugeno, N; Takeda, A; Hasegawa, T; Kobayashi, M; Kikuchi, A; Mori, F; Wakabayashi, K; Itoyama, Y
The Journal of Biological Chemistry null
Paula Perez-Pardo et al.
The FEBS journal, 288(14), 4311-4331 (2021-01-21)
Parkinson's disease patients suffer from both motor and nonmotor impairments. There is currently no cure for Parkinson's disease, and the most commonly used treatment, levodopa, only functions as a temporary relief of motor symptoms. Inhibition of the expression of the
Theodore A Sarafian et al.
Journal of neuroscience research, 95(9), 1871-1887 (2017-01-25)
The α-synuclein protein exists in vivo in a variety of covalently modified and aggregated forms associated with Parkinson's disease (PD) pathology. However, the specific proteoform structures involved with neuropathological disease mechanisms are not clearly defined. Since α-synuclein plays a role
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