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Neurobiology of Disease
IF
5.16
Papers
4663
Papers 4745
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Published in Neurobiology of Disease5.16
Suning Ping1
Estimated H-index: 1
(State University of New York Upstate Medical University),
Xuecheng Qiu1
Estimated H-index: 1
(State University of New York Upstate Medical University)
+ -3 AuthorsLi-Ru Zhao2
Estimated H-index: 2
(State University of New York Upstate Medical University)
Abstract Cerebral autosomal dominant arteriopathy with subcortical infarct and leukoencephalopathy (CADASIL) is a cerebral small vascular disease caused by NOTCH3 gene mutation in vascular smooth muscle cells (VSMCs), leading to ischemic stroke and vascular dementia. To date, the pathogenesis of CADASIL remains poorly understood, and there is no treatment that can slow the progression of CADASIL. Using a transgenic mouse model of CADASIL (TgNotch3R90C), this study reveals novel findings for unde...
Published on Jul 1, 2019in Neurobiology of Disease5.16
geun Hwa Park1
Estimated H-index: 1
(Ajou University),
Hee Sun Shin (Ajou University)+ 6 AuthorsJi Man Hong14
Estimated H-index: 14
(Ajou University)
Abstract It is challenging to revitalize ischemic penumbra after an acute stroke with intracranial perfusion insufficiency. To evaluate whether cranial burr hole and erythropoietin (EPO) generate effective revascularization, we investigated the efficacy of the augmentation method for reverse arteriogenesis from the healthy extracranial milieu. An intracranial perfusion insufficiency was created through bilateral internal carotid artery ligation (bICAL) in Sprague-Dawley rats. We administered rec...
Published on Jul 1, 2019in Neurobiology of Disease5.16
Lena F. Burbulla13
Estimated H-index: 13
,
Dimitri Krainc47
Estimated H-index: 47
Published in Neurobiology of Disease5.16
Nesli-Ece Sen1
Estimated H-index: 1
(Goethe University Frankfurt),
Júlia Canet-Pons1
Estimated H-index: 1
(Goethe University Frankfurt)
+ -3 AuthorsMichel Mittelbronn42
Estimated H-index: 42
Abstract Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disorder caused by CAG-expansion mutations in the ATXN2 gene, mainly affecting motor neurons in the spinal cord and Purkinje neurons in the cerebellum. While the large expansions were shown to cause SCA2, the intermediate length expansions lead to increased risk for several atrophic processes including amyotrophic lateral sclerosis and Parkinson variants, e.g. progressive supranuclear palsy. Intense efforts ...
Published on Jul 1, 2019in Neurobiology of Disease5.16
Susanna B. Mierau2
Estimated H-index: 2
(University of Cambridge),
Ann M. Neumeyer11
Estimated H-index: 11
(Harvard University)
Abstract Metabolic interventions including special diets and supplements are commonly used in Autism Spectrum Disorder (ASD). Yet little is known about how these interventions, typically initiated by caregivers, may affect metabolic function or the core symptoms of ASD. This review examines possible direct and indirect roles for metabolism in the core symptoms of ASD as well as evidence for metabolic dysfunction and nutritional deficiencies. We also discuss some of the most popular diets and sup...
Published in Neurobiology of Disease5.16
Marie Sjögren2
Estimated H-index: 2
(Lund University),
Rana Soylu-Kucharz4
Estimated H-index: 4
(Lund University)
+ -3 AuthorsMaria Björkqvist30
Estimated H-index: 30
(Lund University)
Abstract Body weight has been shown to be a predictor of clinical progression in Huntington's disease (HD). Alongside widespread neuronal pathology, both HD patients and the R6/2 mouse model of HD exhibit weight loss and increased energy expenditure, providing a rationale for targeting whole-body energy metabolism in HD. Leptin- deficient mice display low energy expenditure and increased body weight. We therefore hypothesized that normalizing energy metabolism in R6/2 mice, utilizing leptin- def...
Published on Jul 1, 2019in Neurobiology of Disease5.16
Sean L. Johnson (WSU: Wayne State University), Jessica R. Blount8
Estimated H-index: 8
(WSU: Wayne State University)
+ 4 AuthorsSokol V. Todi19
Estimated H-index: 19
(WSU: Wayne State University)
Abstract The most commonly inherited dominant ataxia, Spinocerebellar Ataxia Type 3 (SCA3), is caused by a CAG repeat expansion that encodes an abnormally long polyglutamine (polyQ) repeat in the disease protein ataxin-3, a deubiquitinase. Two major full-length isoforms of ataxin-3 exist, both of which contain the same N-terminal portion and polyQ repeat, but differ in their C-termini; one (denoted here as isoform 1) contains a motif that binds ataxin-3's substrate, ubiquitin, whereas the other ...
Published in Neurobiology of Disease5.16
Matteo Pedrazzoli (University of Verona), Morris Losurdo (University of Milano-Bicocca)+ -3 AuthorsMario Buffelli14
Estimated H-index: 14
(University of Verona)
Abstract Chronic exposure to high circulating levels of glucocorticoids (GCs) may be a key risk factor for Alzheimer's Disease (AD) development and progression. In addition, hyper-activation of glucocorticoid receptors (GRs) induces brain alterations comparable to those produced by AD. In transgenic mouse models of AD, GCs increase the production of the most important and typical hallmarks of this dementia such as: Aβ40, Aβ42 and tau protein (both the total tau and its hyperphosphorylated isofor...
Published in Neurobiology of Disease5.16
Elena Arystarkhova21
Estimated H-index: 21
(Harvard University),
Ihtsham Haq19
Estimated H-index: 19
(Wake Forest University)
+ -3 AuthorsScott Demarest4
Estimated H-index: 4
(University of Colorado Denver)
Abstract Dominant mutations of ATP1A3, a neuronal Na,K-ATPase α subunit isoform, cause neurological disorders with an exceptionally wide range of severity. Several new mutations and their phenotypes are reported here (p.Asp366His, p.Asp742Tyr, p.Asp743His, p.Leu924Pro, and a VUS, p.Arg463Cys). Mutations associated with mild or severe phenotypes [rapid-onset dystonia-parkinsonism (RDP), alternating hemiplegia of childhood (AHC), or early infantile epileptic encephalopathy (EIEE)] were expressed i...
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