Low and high-order topological disruption of functional networks in multiple system atrophy with freezing of gait: A resting-state study
CONCLUSION: This study identified a disrupted topology of functional interactions at both low and high levels with extensive alterations in topological properties in MSA patients with FOG, especially those associated with damage to the FPN. These findings offer new insights into the dysfunctional mechanisms of complex networks and suggest potential neuroimaging biomarkers for FOG in patients with MSA.PMID:38615913 | DOI:10.1016/j.nbd.2024.106504 (Source: Neurobiology of Disease)
Source: Neurobiology of Disease - April 14, 2024 Category: Neurology Authors: Guoguang Fan Mengwan Zhao Huize Pang Xiaolu Li Shuting Bu Juzhou Wang Yu Liu Yueluan Jiang Source Type: research

Disrupted autonomic pathways in spinal cord injury: Implications for the immune regulation
Neurobiol Dis. 2024 Apr 11:106500. doi: 10.1016/j.nbd.2024.106500. Online ahead of print.ABSTRACTSpinal Cord Injury (SCI) disrupts critical autonomic pathways responsible for the regulation of the immune function. Consequently, individuals with SCI often exhibit a spectrum of immune dysfunctions ranging from the development of damaging pro-inflammatory responses to severe immunosuppression. Thus, it is imperative to gain a more comprehensive understanding of the extent and mechanisms through which SCI-induced autonomic dysfunction influences the immune response. In this review, we provide an overview of the anatomical orga...
Source: Neurobiology of Disease - April 13, 2024 Category: Neurology Authors: Maria M Moura Andreia Monteiro Ant ónio J Salgado Nuno A Silva Susana Monteiro Source Type: research

Nigrostriatal loop - from basics and beyond
Neurobiol Dis. 2024 Apr 13:106503. doi: 10.1016/j.nbd.2024.106503. Online ahead of print.NO ABSTRACTPMID:38614276 | DOI:10.1016/j.nbd.2024.106503 (Source: Neurobiology of Disease)
Source: Neurobiology of Disease - April 13, 2024 Category: Neurology Authors: Mariana H G Monje J Blesa Source Type: research

Disrupted autonomic pathways in spinal cord injury: Implications for the immune regulation
Neurobiol Dis. 2024 Apr 11:106500. doi: 10.1016/j.nbd.2024.106500. Online ahead of print.ABSTRACTSpinal Cord Injury (SCI) disrupts critical autonomic pathways responsible for the regulation of the immune function. Consequently, individuals with SCI often exhibit a spectrum of immune dysfunctions ranging from the development of damaging pro-inflammatory responses to severe immunosuppression. Thus, it is imperative to gain a more comprehensive understanding of the extent and mechanisms through which SCI-induced autonomic dysfunction influences the immune response. In this review, we provide an overview of the anatomical orga...
Source: Neurobiology of Disease - April 13, 2024 Category: Neurology Authors: Maria M Moura Andreia Monteiro Ant ónio J Salgado Nuno A Silva Susana Monteiro Source Type: research

Nigrostriatal loop - from basics and beyond
Neurobiol Dis. 2024 Apr 11:106503. doi: 10.1016/j.nbd.2024.106503. Online ahead of print.NO ABSTRACTPMID:38614276 | DOI:10.1016/j.nbd.2024.106503 (Source: Neurobiology of Disease)
Source: Neurobiology of Disease - April 13, 2024 Category: Neurology Authors: Mariana H G Monje J Blesa Source Type: research

BDNF and TRiC-inspired reagent rescue cortical synaptic deficits in a mouse model of Huntington's disease
Neurobiol Dis. 2024 Apr 10:106502. doi: 10.1016/j.nbd.2024.106502. Online ahead of print.ABSTRACTSynaptic changes are early manifestations of neuronal dysfunction in Huntington's disease (HD). However, the mechanisms by which mutant HTT protein impacts synaptogenesis and function are not well understood. Herein we explored HD pathogenesis in the BACHD mouse model by examining synaptogenesis and function in long term primary cortical cultures. At DIV14 (days in vitro), BACHD cortical neurons showed no difference from WT neurons in synaptogenesis as revealed by colocalization of a pre-synaptic (Synapsin I) and a post-synapti...
Source: Neurobiology of Disease - April 12, 2024 Category: Neurology Authors: Yingli Gu Alexander Pope Charlene Smith Christopher Carmona Aaron Johnstone Linda Shi Xuqiao Chen Sarai Santos Claire Cecile Bacon-Brenes Thomas Shoff Korbin M Kleczko Judith Frydman Leslie M Thompson William C Mobley Chengbiao Wu Source Type: research

Gut-brain axis in the pathogenesis of sepsis-associated encephalopathy
Neurobiol Dis. 2024 Apr 7;195:106499. doi: 10.1016/j.nbd.2024.106499. Online ahead of print.ABSTRACTThe gut-brain axis is a bidirectional communication network linking the gut and the brain, overseeing digestive functions, emotional responses, body immunity, brain development, and overall health. Substantial research highlights a connection between disruptions of the gut-brain axis and various psychiatric and neurological conditions, including depression and Alzheimer's disease. Given the impact of the gut-brain axis on behavior, cognition, and brain diseases, some studies have started to pay attention to the role of the a...
Source: Neurobiology of Disease - April 8, 2024 Category: Neurology Authors: Xin Wang Xiaoyue Wen Shiying Yuan Jiancheng Zhang Source Type: research

Gut-brain axis in the pathogenesis of sepsis-associated encephalopathy
Neurobiol Dis. 2024 Apr 7;195:106499. doi: 10.1016/j.nbd.2024.106499. Online ahead of print.ABSTRACTThe gut-brain axis is a bidirectional communication network linking the gut and the brain, overseeing digestive functions, emotional responses, body immunity, brain development, and overall health. Substantial research highlights a connection between disruptions of the gut-brain axis and various psychiatric and neurological conditions, including depression and Alzheimer's disease. Given the impact of the gut-brain axis on behavior, cognition, and brain diseases, some studies have started to pay attention to the role of the a...
Source: Neurobiology of Disease - April 8, 2024 Category: Neurology Authors: Xin Wang Xiaoyue Wen Shiying Yuan Jiancheng Zhang Source Type: research

Gut-brain axis in the pathogenesis of sepsis-associated encephalopathy
Neurobiol Dis. 2024 Apr 6:106499. doi: 10.1016/j.nbd.2024.106499. Online ahead of print.ABSTRACTThe gut-brain axis is a bidirectional communication network linking the gut and the brain, overseeing digestive functions, emotional responses, body immunity, brain development, and overall health. Substantial research highlights a connection between disruptions of the gut-brain axis and various psychiatric and neurological conditions, including depression and Alzheimer's disease. Given the impact of the gut-brain axis on behavior, cognition, and brain diseases, some studies have started to pay attention to the role of the axis ...
Source: Neurobiology of Disease - April 8, 2024 Category: Neurology Authors: Xin Wang Xiaoyue Wen Shiying Yuan Jiancheng Zhang Source Type: research

Gut-brain axis in the pathogenesis of sepsis-associated encephalopathy
Neurobiol Dis. 2024 Apr 6:106499. doi: 10.1016/j.nbd.2024.106499. Online ahead of print.ABSTRACTThe gut-brain axis is a bidirectional communication network linking the gut and the brain, overseeing digestive functions, emotional responses, body immunity, brain development, and overall health. Substantial research highlights a connection between disruptions of the gut-brain axis and various psychiatric and neurological conditions, including depression and Alzheimer's disease. Given the impact of the gut-brain axis on behavior, cognition, and brain diseases, some studies have started to pay attention to the role of the axis ...
Source: Neurobiology of Disease - April 8, 2024 Category: Neurology Authors: Xin Wang Xiaoyue Wen Shiying Yuan Jiancheng Zhang Source Type: research

CHCHD10 < sup > S59L/+ < /sup > mouse model: Behavioral and neuropathological features of frontotemporal dementia
Neurobiol Dis. 2024 Apr 5:106498. doi: 10.1016/j.nbd.2024.106498. Online ahead of print.ABSTRACTCHCHD10-related disease causes a spectrum of clinical presentations including mitochondrial myopathy, cardiomyopathy, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). We generated a knock-in mouse model bearing the p.Ser59Leu (S59L) CHCHD10 variant. Chchd10S59L/+ mice have been shown to phenotypically replicate the disorders observed in patients: myopathy with mtDNA instability, cardiomyopathy and typical ALS features (protein aggregation, neuromuscular junction degeneration and spinal motor neuron loss). H...
Source: Neurobiology of Disease - April 7, 2024 Category: Neurology Authors: Emmanuelle C Genin Pauline Pozzo di Borgo Thomas Lorivel Sandrine Hugues M élissa Farinelli Alessandra Mauri-Crouzet Fran çoise Lespinasse Lucas Godin V éronique Paquis-Flucklinger Agn ès Petit-Paitel Source Type: research

Boosting BDNF in muscle rescues impaired axonal transport in a mouse model of DI-CMTC peripheral neuropathy
In this study, we first showed that human DI-CMTC-causing TyrRSE196K mis-interacts with the extracellular domain of the BDNF receptor TrkB, an aberrant association we have previously characterised for several mutant glycyl-tRNA synthetases linked to CMT type 2D (CMT2D). We then performed temporal neuromuscular assessments of YarsE196K mice modelling DI-CMT. We determined that YarsE196K homozygotes display a selective, age-dependent impairment in in vivo axonal transport of neurotrophin-containing signalling endosomes, phenocopying CMT2D mice. This impairment is replicated by injection of recombinant TyrRSE196K, but not Tyr...
Source: Neurobiology of Disease - April 7, 2024 Category: Neurology Authors: Elena R Rhymes Rebecca L Simkin Ji Qu David Villarroel-Campos Sunaina Surana Yao Tong Ryan Shapiro Robert W Burgess Xiang-Lei Yang Giampietro Schiavo James N Sleigh Source Type: research

Genetic and pathological features encipher the phenotypic heterogeneity of Gerstmann-Str äussler-Scheinker disease
CONCLUSION: This study reveals GSS's intricate nature, showing significant variations in clinical presentations, diagnostic findings, and pathological features. Mutation sites and pathological changes play crucial roles in determining the GSS clinical heterogeneity.PMID:38583641 | DOI:10.1016/j.nbd.2024.106497 (Source: Neurobiology of Disease)
Source: Neurobiology of Disease - April 7, 2024 Category: Neurology Authors: Zhongyun Chen Yu Kong Jing Zhang Wen-Quan Zou Liyong Wu Source Type: research

Spatio-temporal brain invasion pattern of Streptococcus pneumoniae and dynamic changes in the cellular environment in bacteremia-derived meningitis
Neurobiol Dis. 2024 Apr 5:106484. doi: 10.1016/j.nbd.2024.106484. Online ahead of print.ABSTRACTStreptococcus pneumoniae (the pneumococcus) is the major cause of bacterial meningitis globally, and pneumococcal meningitis is associated with increased risk of long-term neurological sequelae. These include several sensorimotor functions that are controlled by specific brain regions which, during bacterial meningitis, are damaged by a neuroinflammatory response and the deleterious action of bacterial toxins in the brain. However, little is known about the invasion pattern of the pneumococcus into the brain. Using a bacteremia-...
Source: Neurobiology of Disease - April 7, 2024 Category: Neurology Authors: Kristine Farmen Miguel Tofi ño-Vian Katrin Wellfelt Lars Olsson Federico Iovino Source Type: research

CHCHD10 < sup > S59L/+ < /sup > mouse model: Behavioral and neuropathological features of frontotemporal dementia
Neurobiol Dis. 2024 Apr 5:106498. doi: 10.1016/j.nbd.2024.106498. Online ahead of print.ABSTRACTCHCHD10-related disease causes a spectrum of clinical presentations including mitochondrial myopathy, cardiomyopathy, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). We generated a knock-in mouse model bearing the p.Ser59Leu (S59L) CHCHD10 variant. Chchd10S59L/+ mice have been shown to phenotypically replicate the disorders observed in patients: myopathy with mtDNA instability, cardiomyopathy and typical ALS features (protein aggregation, neuromuscular junction degeneration and spinal motor neuron loss). H...
Source: Neurobiology of Disease - April 7, 2024 Category: Neurology Authors: Emmanuelle C Genin Pauline Pozzo di Borgo Thomas Lorivel Sandrine Hugues M élissa Farinelli Alessandra Mauri-Crouzet Fran çoise Lespinasse Lucas Godin V éronique Paquis-Flucklinger Agn ès Petit-Paitel Source Type: research