Structural interhemispheric connectivity defects in mouse models of BBSOAS: Insights from high spatial resolution 3D white matter tractography
In this study, we used 3D DTI to unveil brain-specific tract defects in two mouse models lacking the Nr2f1 gene, which mutations in patients have been proven to cause an emerging NDD, called Bosch-Boonstra-Schaaf Optic Atrophy (BBSOAS). We aimed to investigate the impact of the lack of cortical Nr2f1 function on WM morphometry and tract microstructure quantifications. We found in both mutant mice partial loss of fibers and severe misrouting of the two major cortical commissural tracts, the corpus callosum, and the anterior commissure, as well as the two major hippocampal efferent tracts, the post-commissural fornix, and th...
Source: Neurobiology of Disease - February 26, 2024 Category: Neurology Authors: Jean Christophe Deloulme Maxime Leclercq Olivier Deschaux Gemma Flore Laetitia Capellano Chiara Tocco Barbara Yael Braz Mich èle Studer Hana Lahrech Source Type: research
hnRNP R regulates mitochondrial movement and membrane potential in axons of motoneurons
Neurobiol Dis. 2024 Feb 24:106454. doi: 10.1016/j.nbd.2024.106454. Online ahead of print.ABSTRACTAxonal mitochondria defects are early events in the pathogenesis of motoneuron disorders such as spinal muscular atrophy and amyotrophic lateral sclerosis. The RNA-binding protein hnRNP R interacts with different motoneuron disease-related proteins such as SMN and TDP-43 and has important roles in axons of motoneurons, including axonal mRNA transport. However, whether hnRNP R also modulates axonal mitochondria is currently unknown. Here, we show that axonal mitochondria exhibit altered function and motility in hnRNP R-deficient...
Source: Neurobiology of Disease - February 26, 2024 Category: Neurology Authors: Sophia Dithmar Abdolhossein Zare Saeede Salehi Michael Briese Michael Sendtner Source Type: research
Structural interhemispheric connectivity defects in mouse models of BBSOAS: Insights from high spatial resolution 3D white matter tractography
In this study, we used 3D DTI to unveil brain-specific tract defects in two mouse models lacking the Nr2f1 gene, which mutations in patients have been proven to cause an emerging NDD, called Bosch-Boonstra-Schaaf Optic Atrophy (BBSOAS). We aimed to investigate the impact of the lack of cortical Nr2f1 function on WM morphometry and tract microstructure quantifications. We found in both mutant mice partial loss of fibers and severe misrouting of the two major cortical commissural tracts, the corpus callosum, and the anterior commissure, as well as the two major hippocampal efferent tracts, the post-commissural fornix, and th...
Source: Neurobiology of Disease - February 26, 2024 Category: Neurology Authors: Jean Christophe Deloulme Maxime Leclercq Olivier Deschaux Gemma Flore Laetitia Capellano Chiara Tocco Barbara Yael Braz Mich èle Studer Hana Lahrech Source Type: research
hnRNP R regulates mitochondrial movement and membrane potential in axons of motoneurons
Neurobiol Dis. 2024 Feb 24:106454. doi: 10.1016/j.nbd.2024.106454. Online ahead of print.ABSTRACTAxonal mitochondria defects are early events in the pathogenesis of motoneuron disorders such as spinal muscular atrophy and amyotrophic lateral sclerosis. The RNA-binding protein hnRNP R interacts with different motoneuron disease-related proteins such as SMN and TDP-43 and has important roles in axons of motoneurons, including axonal mRNA transport. However, whether hnRNP R also modulates axonal mitochondria is currently unknown. Here, we show that axonal mitochondria exhibit altered function and motility in hnRNP R-deficient...
Source: Neurobiology of Disease - February 26, 2024 Category: Neurology Authors: Sophia Dithmar Abdolhossein Zare Saeede Salehi Michael Briese Michael Sendtner Source Type: research
Structural interhemispheric connectivity defects in mouse models of BBSOAS: Insights from high spatial resolution 3D white matter tractography
In this study, we used 3D DTI to unveil brain-specific tract defects in two mouse models lacking the Nr2f1 gene, which mutations in patients have been proven to cause an emerging NDD, called Bosch-Boonstra-Schaaf Optic Atrophy (BBSOAS). We aimed to investigate the impact of the lack of cortical Nr2f1 function on WM morphometry and tract microstructure quantifications. We found in both mutant mice partial loss of fibers and severe misrouting of the two major cortical commissural tracts, the corpus callosum, and the anterior commissure, as well as the two major hippocampal efferent tracts, the post-commissural fornix, and th...
Source: Neurobiology of Disease - February 26, 2024 Category: Neurology Authors: Jean Christophe Deloulme Maxime Leclercq Olivier Deschaux Gemma Flore Laetitia Capellano Chiara Tocco Barbara Yael Braz Mich èle Studer Hana Lahrech Source Type: research
hnRNP R regulates mitochondrial movement and membrane potential in axons of motoneurons
Neurobiol Dis. 2024 Feb 24:106454. doi: 10.1016/j.nbd.2024.106454. Online ahead of print.ABSTRACTAxonal mitochondria defects are early events in the pathogenesis of motoneuron disorders such as spinal muscular atrophy and amyotrophic lateral sclerosis. The RNA-binding protein hnRNP R interacts with different motoneuron disease-related proteins such as SMN and TDP-43 and has important roles in axons of motoneurons, including axonal mRNA transport. However, whether hnRNP R also modulates axonal mitochondria is currently unknown. Here, we show that axonal mitochondria exhibit altered function and motility in hnRNP R-deficient...
Source: Neurobiology of Disease - February 26, 2024 Category: Neurology Authors: Sophia Dithmar Abdolhossein Zare Saeede Salehi Michael Briese Michael Sendtner Source Type: research
Structural interhemispheric connectivity defects in mouse models of BBSOAS: Insights from high spatial resolution 3D white matter tractography
In this study, we used 3D DTI to unveil brain-specific tract defects in two mouse models lacking the Nr2f1 gene, which mutations in patients have been proven to cause an emerging NDD, called Bosch-Boonstra-Schaaf Optic Atrophy (BBSOAS). We aimed to investigate the impact of the lack of cortical Nr2f1 function on WM morphometry and tract microstructure quantifications. We found in both mutant mice partial loss of fibers and severe misrouting of the two major cortical commissural tracts, the corpus callosum, and the anterior commissure, as well as the two major hippocampal efferent tracts, the post-commissural fornix, and th...
Source: Neurobiology of Disease - February 26, 2024 Category: Neurology Authors: Jean Christophe Deloulme Maxime Leclercq Olivier Deschaux Gemma Flore Laetitia Capellano Chiara Tocco Barbara Yael Braz Mich èle Studer Hana Lahrech Source Type: research
Gene-environment interaction elicits dystonia-like features and impaired translational regulation in a DYT-TOR1A mouse model
Neurobiol Dis. 2024 Feb 23:106453. doi: 10.1016/j.nbd.2024.106453. Online ahead of print.ABSTRACTDYT-TOR1A dystonia is the most common monogenic dystonia characterized by involuntary muscle contractions and lack of therapeutic options. Despite some insights into its etiology, the disease's pathophysiology remains unclear. The reduced penetrance of about 30% suggests that extragenetic factors are needed to develop a dystonic phenotype. In order to systematically investigate this hypothesis, we induced a sciatic nerve crush injury in a genetically predisposed DYT-TOR1A mouse model (DYT1KI) to evoke a dystonic phenotype. Subs...
Source: Neurobiology of Disease - February 25, 2024 Category: Neurology Authors: Colette Reinhold Susanne Knorr Rhonda L McFleder Lisa Rauschenberger Muthuraman Muthuraman Panagiota Arampatzi Tom Gr äfenhan Andreas Schlosser Michael Sendtner Jens Volkmann Chi Wang Ip Source Type: research
Suppression of presynaptic corticostriatal glutamate activity attenuates L-dopa-induced dyskinesia in 6-OHDA-lesioned Parkinson's disease mice
In this study, we explored whether suppression of presynaptic corticostriatal glutamate inputs might affect the behavioral and biochemical outcomes associated with LID. We first established an animal model in which 6-hydroxydopamine (6-OHDA)-lesioned mice were treated daily with L-dopa (10 mg/kg, i.p.) for 2 weeks; these mice developed stereotypical abnormal involuntary movements (AIMs). When the mice were pretreated with the NMDA antagonist, amantadine, we observed suppression of AIMs and reductions of phosphorylated ERK1/2 and NR2B in the striatum. We then took an optogenetic approach to manipulate glutamatergic activity...
Source: Neurobiology of Disease - February 24, 2024 Category: Neurology Authors: Yu-Ting Huang Ya-Wen Chen Tze-Yen Lin Jin-Chung Chen Source Type: research
Corrigendum to "Reducing huntingtin by immunotherapy delays disease progression in a mouse model of Huntington disease" [Neurobiology of Disease, 2024 Jan:190:106376]
Neurobiol Dis. 2024 Feb 23:106444. doi: 10.1016/j.nbd.2024.106444. Online ahead of print.NO ABSTRACTPMID:38402018 | DOI:10.1016/j.nbd.2024.106444 (Source: Neurobiology of Disease)
Source: Neurobiology of Disease - February 24, 2024 Category: Neurology Authors: Stefan Bartl Yuanyun Xie Nalini Potluri Ratnesh Kesineni Katlin Hencak Louisa Dal Cengio Katja Balazs Abid Oueslati Michela Parth Nina Salhat Alberto Siddu Oskar Smrzka Francesca Cicchetti G ünther Staffler Michael R Hayden Amber L Southwell Source Type: research
Suppression of presynaptic corticostriatal glutamate activity attenuates L-dopa-induced dyskinesia in 6-OHDA-lesioned Parkinson's disease mice
In this study, we explored whether suppression of presynaptic corticostriatal glutamate inputs might affect the behavioral and biochemical outcomes associated with LID. We first established an animal model in which 6-hydroxydopamine (6-OHDA)-lesioned mice were treated daily with L-dopa (10 mg/kg, i.p.) for 2 weeks; these mice developed stereotypical abnormal involuntary movements (AIMs). When the mice were pretreated with the NMDA antagonist, amantadine, we observed suppression of AIMs and reductions of phosphorylated ERK1/2 and NR2B in the striatum. We then took an optogenetic approach to manipulate glutamatergic activity...
Source: Neurobiology of Disease - February 24, 2024 Category: Neurology Authors: Yu-Ting Huang Ya-Wen Chen Tze-Yen Lin Jin-Chung Chen Source Type: research
Corrigendum to "Reducing huntingtin by immunotherapy delays disease progression in a mouse model of Huntington disease" [Neurobiology of Disease, 2024 Jan:190:106376]
Neurobiol Dis. 2024 Feb 23:106444. doi: 10.1016/j.nbd.2024.106444. Online ahead of print.NO ABSTRACTPMID:38402018 | DOI:10.1016/j.nbd.2024.106444 (Source: Neurobiology of Disease)
Source: Neurobiology of Disease - February 24, 2024 Category: Neurology Authors: Stefan Bartl Yuanyun Xie Nalini Potluri Ratnesh Kesineni Katlin Hencak Louisa Dal Cengio Katja Balazs Abid Oueslati Michela Parth Nina Salhat Alberto Siddu Oskar Smrzka Francesca Cicchetti G ünther Staffler Michael R Hayden Amber L Southwell Source Type: research
Role of soluble epoxide hydrolase in pain and depression comorbidity
Neurobiol Dis. 2024 Feb 21:106443. doi: 10.1016/j.nbd.2024.106443. Online ahead of print.ABSTRACTThe coexistence of chronic pain and depression in clinical practice places a substantial social burden and profoundly impacts in patients. Although a clear correlation exists, the underlying mechanism of comorbidity between chronic pain and depression remains elusive. Research conducted in recent decades has uncovered that soluble epoxide hydrolase, a pivotal enzyme in the metabolism of polyunsaturated fatty acids, plays a crucial role in inflammation. Interestingly, this enzyme is intricately linked to the development of both ...
Source: Neurobiology of Disease - February 23, 2024 Category: Neurology Authors: Yuchen Bu Siqi Yang Di Wang Suwan Hu Qi Zhang Zifeng Wu Chun Yang Source Type: research
The potential roles of salivary biomarkers in neurodegenerative diseases
Neurobiol Dis. 2024 Feb 19;193:106442. doi: 10.1016/j.nbd.2024.106442. Online ahead of print.ABSTRACTCurrent research efforts on neurodegenerative diseases are focused on identifying novel and reliable biomarkers for early diagnosis and insight into disease progression. Salivary analysis is gaining increasing interest as a promising source of biomarkers and matrices for measuring neurodegenerative diseases. Saliva collection offers multiple advantages over the currently detected biofluids as it is easily accessible, non-invasive, and repeatable, allowing early diagnosis and timely treatment of the diseases. Here, we review...
Source: Neurobiology of Disease - February 21, 2024 Category: Neurology Authors: Ling-Ling Jiao Hui-Lin Dong Meng-Meng Liu Peng-Lin Wu Yi Cao Yuan Zhang Fu-Gao Gao Huai-Yuan Zhu Source Type: research
The potential roles of salivary biomarkers in neurodegenerative diseases
Neurobiol Dis. 2024 Feb 19;193:106442. doi: 10.1016/j.nbd.2024.106442. Online ahead of print.ABSTRACTCurrent research efforts on neurodegenerative diseases are focused on identifying novel and reliable biomarkers for early diagnosis and insight into disease progression. Salivary analysis is gaining increasing interest as a promising source of biomarkers and matrices for measuring neurodegenerative diseases. Saliva collection offers multiple advantages over the currently detected biofluids as it is easily accessible, non-invasive, and repeatable, allowing early diagnosis and timely treatment of the diseases. Here, we review...
Source: Neurobiology of Disease - February 21, 2024 Category: Neurology Authors: Ling-Ling Jiao Hui-Lin Dong Meng-Meng Liu Peng-Lin Wu Yi Cao Yuan Zhang Fu-Gao Gao Huai-Yuan Zhu Source Type: research
