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Specialty: Neurology
Source: Neurobiology of Disease
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Total 1847 results found since Jan 2013.
Protein kinase D2 confers neuroprotection by promoting AKT and CREB activation in ischemic stroke
In this study, we found that PKD2 expression and activity were significantly upregulated in the ipsilateral side of the brain after transient focal cerebral ischemia, which coincides with the upregulation of PKD2 in primary neurons in response to in vitro ischemia, implying a potential role of PKD2 in neuronal survival in ischemic stroke. Using kinase-dead PKD2 knock-in (PKD2-KI) mice, we examined whether loss of PKD2 activity affected stroke outcomes in mice subjected to 1 h of transient middle cerebral artery occlusion (tMCAO) and 24 h of reperfusion. Our data demonstrated that PKD2-KI mice exhibited larger infarction vo...
Source: Neurobiology of Disease - September 20, 2023 Category: Neurology Authors: Jaclyn A Connelly Xuejing Zhang Yuzhou Chen Yapeng Chao Yejie Shi Tija C Jacob Q Jane Wang Source Type: research
Corrigendum to "Understanding the role of the hematopoietic niche in Huntington's disease's phenotypic expression: In vivo evidence using a parabiosis model" Neurobiol Dis. 2023 May;180:106091. doi:10.1016/j.nbd.2023.106091. Epub 2023 Mar 24
Neurobiol Dis. 2023 Sep 16:106289. doi: 10.1016/j.nbd.2023.106289. Online ahead of print.NO ABSTRACTPMID:37722998 | DOI:10.1016/j.nbd.2023.106289
Source: Neurobiology of Disease - September 18, 2023 Category: Neurology Authors: Marie Rieux Melanie Alpaugh Shireen Salem Alberto Siddu Martine Saint-Pierre H élèna L Denis Heike Rohweder Frank Herrmann Chantal Bazenet Steve Lacroix Francesca Cicchetti Source Type: research
Transcending the amyloid-beta dominance paradigm in Alzheimer's disease: An exploration of behavioural, metabolic, and gut microbiota phenotypes in 5xFAD mice
Neurobiol Dis. 2023 Sep 15:106295. doi: 10.1016/j.nbd.2023.106295. Online ahead of print.ABSTRACTThe amyloid cascade hypothesis is widely accepted as an explanation for the neuropathological changes in Alzheimer's disease (AD). However, the role of amyloid-beta (Aβ) as the sole cause of these changes is being questioned. Using the 5xFAD mouse model of AD, we investigated various factors contributing to neuropathology, including genetic load (heterozygous (HTZ) versus homozygous (HZ) condition), behavioural phenotype, neuropathology markers, metabolic physiology, and gut microbiota composition at early (5 months of age) an...
Source: Neurobiology of Disease - September 17, 2023 Category: Neurology Authors: Dina Medina-Vera Emma N Zambrana-Infantes Antonio J L ópez-Gambero Julia Verheul-Campos Luis J Sant ín Elena Baixeras Juan Suarez Francisco J Pavon Cristina Rosell-Valle Fernando Rodr íguez de Fonseca Source Type: research
A murine model of BSCL2-associated Celia's encephalopathy
Neurobiol Dis. 2023 Sep 15:106300. doi: 10.1016/j.nbd.2023.106300. Online ahead of print.ABSTRACTCelia's encephalopathy or progressive encephalopathy with/without lipodystrophy is a neurodegenerative disease with a fatal prognosis in childhood. It is generally caused by the c.985C > T variant in the BSCL2 gene, leading to the skipping of exon 7 and resulting in an aberrant seipin protein (Celia-seipin). To precisely define the temporal evolution and the mechanisms involved in neurodegeneration, lipodystrophy and fatty liver in Celia's encephalopathy, our group has generated the first global knock-in murine model for the...
Source: Neurobiology of Disease - September 17, 2023 Category: Neurology Authors: Silvia Cobelo-G ómez Sof ía Sánchez-Iglesias Alberto R ábano Ana Senra Pablo Aguiar Noem í Gómez-Lado Lara Garc ía-Varela Iv án Burgueño-García Laura Lamp ón-Fernández Ant ía Fernández-Pombo Everardo Josu é Díaz-López Teresa Prado-Mora ñ Source Type: research
PIEZO1 expression at the glio-vascular unit adjusts to neuroinflammation in seizure conditions
Neurobiol Dis. 2023 Sep 15:106297. doi: 10.1016/j.nbd.2023.106297. Online ahead of print.ABSTRACTMechanosensors are emerging players responding to hemodynamic and physical inputs. Their significance in the central nervous system remains relatively uncharted. Using human-derived brain specimens or cells and a pre-clinical model of mesio-temporal lobe epilepsy (MTLE), we examined how the mRNA levels of the mechanosensitive channel PIEZO1 adjust to disease-associated pro-inflammatory trajectories. In brain tissue micro-punches obtained from 18 drug-resistant MTLE patients, PIEZO1 expression positively correlated with that of ...
Source: Neurobiology of Disease - September 17, 2023 Category: Neurology Authors: Valentin Garcia Marine Blaquiere Alicia Janvier Noemie Cresto Carla Lana Athenais Genin Helene Hirbec Etienne Audinat Adele Faucherre Emmanuel L Barbier Sophie Hamelin Philippe Kahane Chris Jopling Nicola Marchi Source Type: research
Transcending the amyloid-beta dominance paradigm in Alzheimer's disease: An exploration of behavioural, metabolic, and gut microbiota phenotypes in 5xFAD mice
Neurobiol Dis. 2023 Sep 15:106295. doi: 10.1016/j.nbd.2023.106295. Online ahead of print.ABSTRACTThe amyloid cascade hypothesis is widely accepted as an explanation for the neuropathological changes in Alzheimer's disease (AD). However, the role of amyloid-beta (Aβ) as the sole cause of these changes is being questioned. Using the 5xFAD mouse model of AD, we investigated various factors contributing to neuropathology, including genetic load (heterozygous (HTZ) versus homozygous (HZ) condition), behavioural phenotype, neuropathology markers, metabolic physiology, and gut microbiota composition at early (5 months of age) an...
Source: Neurobiology of Disease - September 17, 2023 Category: Neurology Authors: Dina Medina-Vera Emma N Zambrana-Infantes Antonio J L ópez-Gambero Julia Verheul-Campos Luis J Sant ín Elena Baixeras Juan Suarez Francisco J Pavon Cristina Rosell-Valle Fernando Rodr íguez de Fonseca Source Type: research
A murine model of BSCL2-associated Celia's encephalopathy
Neurobiol Dis. 2023 Sep 15:106300. doi: 10.1016/j.nbd.2023.106300. Online ahead of print.ABSTRACTCelia's encephalopathy or progressive encephalopathy with/without lipodystrophy is a neurodegenerative disease with a fatal prognosis in childhood. It is generally caused by the c.985C > T variant in the BSCL2 gene, leading to the skipping of exon 7 and resulting in an aberrant seipin protein (Celia-seipin). To precisely define the temporal evolution and the mechanisms involved in neurodegeneration, lipodystrophy and fatty liver in Celia's encephalopathy, our group has generated the first global knock-in murine model for the...
Source: Neurobiology of Disease - September 17, 2023 Category: Neurology Authors: Silvia Cobelo-G ómez Sof ía Sánchez-Iglesias Alberto R ábano Ana Senra Pablo Aguiar Noem í Gómez-Lado Lara Garc ía-Varela Iv án Burgueño-García Laura Lamp ón-Fernández Ant ía Fernández-Pombo Everardo Josu é Díaz-López Teresa Prado-Mora ñ Source Type: research
PIEZO1 expression at the glio-vascular unit adjusts to neuroinflammation in seizure conditions
Neurobiol Dis. 2023 Sep 15:106297. doi: 10.1016/j.nbd.2023.106297. Online ahead of print.ABSTRACTMechanosensors are emerging players responding to hemodynamic and physical inputs. Their significance in the central nervous system remains relatively uncharted. Using human-derived brain specimens or cells and a pre-clinical model of mesio-temporal lobe epilepsy (MTLE), we examined how the mRNA levels of the mechanosensitive channel PIEZO1 adjust to disease-associated pro-inflammatory trajectories. In brain tissue micro-punches obtained from 18 drug-resistant MTLE patients, PIEZO1 expression positively correlated with that of ...
Source: Neurobiology of Disease - September 17, 2023 Category: Neurology Authors: Valentin Garcia Marine Blaquiere Alicia Janvier Noemie Cresto Carla Lana Athenais Genin Helene Hirbec Etienne Audinat Adele Faucherre Emmanuel L Barbier Sophie Hamelin Philippe Kahane Chris Jopling Nicola Marchi Source Type: research
Microvascular blood-brain barrier alterations in isolated brain capillaries of mice over-expressing alpha-synuclein (Thy1-aSyn line 61)
Neurobiol Dis. 2023 Sep 14:106298. doi: 10.1016/j.nbd.2023.106298. Online ahead of print.ABSTRACTDysfunction of the blood-brain barrier (BBB) is suggested to play a critical role in the pathological mechanisms of Parkinson's disease (PD). PD-related pathology such as alpha-synuclein accumulation and inflammatory processes potentially affect the integrity of the BBB early in disease progression, which in turn may alter the crosstalk of the central and peripheral immune response. Importantly, BBB dysfunction could also affect drug response in PD. Here we analyzed microvascular changes in isolated brain capillaries and brain ...
Source: Neurobiology of Disease - September 16, 2023 Category: Neurology Authors: Kristina Lau Lisa T Porschen Franziska Richter Birthe Gericke Source Type: research
Aberrant splicing of mutant huntingtin in Huntington's disease knock-in pigs
In this study, HTT exon1 production was examined in the HD knock-in (KI) pig model, which more closely recapitulates neuropathology seen in HD patient brains than HD mouse models. The study revealed that aberrant spliced HTT exon1 is also present in the brains of HD pigs, but it is expressed at a much lower level than the normally spliced HTT exon products. These findings suggest that careful consideration is needed when assessing the contribution of aberrantly spliced mHTT exon1 to HD pathogenesis, and further rigorous investigation is required.PMID:37716514 | DOI:10.1016/j.nbd.2023.106291
Source: Neurobiology of Disease - September 16, 2023 Category: Neurology Authors: Huichun Tong Tianqi Yang Li Liu Caijuan Li Yize Sun Qingqing Jia Yiyang Qin Laiqiang Chen Xianxian Zhao Gongke Zhou Sen Yan Xiao-Jiang Li Shihua Li Source Type: research
Microvascular blood-brain barrier alterations in isolated brain capillaries of mice over-expressing alpha-synuclein (Thy1-aSyn line 61)
Neurobiol Dis. 2023 Sep 14:106298. doi: 10.1016/j.nbd.2023.106298. Online ahead of print.ABSTRACTDysfunction of the blood-brain barrier (BBB) is suggested to play a critical role in the pathological mechanisms of Parkinson's disease (PD). PD-related pathology such as alpha-synuclein accumulation and inflammatory processes potentially affect the integrity of the BBB early in disease progression, which in turn may alter the crosstalk of the central and peripheral immune response. Importantly, BBB dysfunction could also affect drug response in PD. Here we analyzed microvascular changes in isolated brain capillaries and brain ...
Source: Neurobiology of Disease - September 16, 2023 Category: Neurology Authors: Kristina Lau Lisa T Porschen Franziska Richter Birthe Gericke Source Type: research
Aberrant splicing of mutant huntingtin in Huntington's disease knock-in pigs
In this study, HTT exon1 production was examined in the HD knock-in (KI) pig model, which more closely recapitulates neuropathology seen in HD patient brains than HD mouse models. The study revealed that aberrant spliced HTT exon1 is also present in the brains of HD pigs, but it is expressed at a much lower level than the normally spliced HTT exon products. These findings suggest that careful consideration is needed when assessing the contribution of aberrantly spliced mHTT exon1 to HD pathogenesis, and further rigorous investigation is required.PMID:37716514 | DOI:10.1016/j.nbd.2023.106291
Source: Neurobiology of Disease - September 16, 2023 Category: Neurology Authors: Huichun Tong Tianqi Yang Li Liu Caijuan Li Yize Sun Qingqing Jia Yiyang Qin Laiqiang Chen Xianxian Zhao Gongke Zhou Sen Yan Xiao-Jiang Li Shihua Li Source Type: research
VPS13A knockdown impairs corticostriatal synaptic plasticity and locomotor behavior in a new mouse model of chorea-acanthocytosis
Neurobiol Dis. 2023 Sep 13:106292. doi: 10.1016/j.nbd.2023.106292. Online ahead of print.ABSTRACTChorea-acanthocytosis (ChAc) is an inherited neurodegenerative movement disorder caused by VPS13A gene mutations leading to the absence of protein expression. The striatum is the most affected brain region in ChAc patients. However, the study of the VPS13A function in the brain has been poorly addressed. Here we generated a VPS13A knockdown (KD) model and aimed to elucidate the contribution of VPS13A to synaptic plasticity and neuronal communication in the corticostriatal circuit. First, we infected primary cortical neurons wit...
Source: Neurobiology of Disease - September 15, 2023 Category: Neurology Authors: Esther Garc ía-García Alba Ram ón-Lainez Sara Conde-Berriozabal Daniel Del Toro Georgia Escaramis Albert Giralt Merc è Masana Jordi Alberch Manuel J Rodr íguez Source Type: research
Understanding the potential causes of gastrointestinal dysfunctions in multiple system atrophy
Neurobiol Dis. 2023 Sep 13:106296. doi: 10.1016/j.nbd.2023.106296. Online ahead of print.ABSTRACTMultiple system atrophy (MSA) is a rare, progressive neurodegenerative disorder characterised by autonomic, pyramidal, parkinsonian and/or cerebellar dysfunction. Autonomic symptoms of MSA include deficits associated with the gastrointestinal (GI) system, such as difficulty swallowing, abdominal pain and bloating, nausea, delayed gastric emptying, and constipation. To date, studies assessing GI dysfunctions in MSA have primarily focused on alterations of the gut microbiome, however growing evidence indicates other structural co...
Source: Neurobiology of Disease - September 15, 2023 Category: Neurology Authors: Colin F Craig David I Finkelstein Rachel M McQuade Shanti Diwakarla Source Type: research
