Progressive alterations in polysomal architecture and activation of ribosome stalling relief factors in a mouse model of Huntington's disease
In this study we examined the architecture of polysomes in their native brain context in striatal tissue from the zQ175 knock-in mouse model of HD. We performed 3D electron tomography of high-pressure frozen and freeze-substituted striatal tissue from HD models and corresponding controls at different ages. Electron tomography results revealed progressive remodelling towards a more compacted polysomal architecture in the mouse model, an effect that coincided with the emergence and progression of HD related symptoms. The aberrant polysomal architecture is compatible with ribosome stalling phenomena. In fact, we also detected...
Source: Neurobiology of Disease - April 2, 2024 Category: Neurology Authors: Eva Martin-Solana Irene Diaz-Lopez Yamina Mohamedi Ivan Ventoso Jose-Jesus Fernandez Maria Rosario Fernandez-Fernandez Source Type: research
Progressive alterations in polysomal architecture and activation of ribosome stalling relief factors in a mouse model of Huntington's disease
In this study we examined the architecture of polysomes in their native brain context in striatal tissue from the zQ175 knock-in mouse model of HD. We performed 3D electron tomography of high-pressure frozen and freeze-substituted striatal tissue from HD models and corresponding controls at different ages. Electron tomography results revealed progressive remodelling towards a more compacted polysomal architecture in the mouse model, an effect that coincided with the emergence and progression of HD related symptoms. The aberrant polysomal architecture is compatible with ribosome stalling phenomena. In fact, we also detected...
Source: Neurobiology of Disease - April 2, 2024 Category: Neurology Authors: Eva Martin-Solana Irene Diaz-Lopez Yamina Mohamedi Ivan Ventoso Jose-Jesus Fernandez Maria Rosario Fernandez-Fernandez Source Type: research
Auditory oddball responses in the human subthalamic nucleus and substantia nigra pars reticulata
Neurobiol Dis. 2024 Mar 30:106490. doi: 10.1016/j.nbd.2024.106490. Online ahead of print.ABSTRACTThe auditory oddball is a mainstay in research on attention, novelty, and sensory prediction. How this task engages subcortical structures like the subthalamic nucleus and substantia nigra pars reticulata is unclear. We administered an auditory OB task while recording single unit activity (35 units) and local field potentials (57 recordings) from the subthalamic nucleus and substantia nigra pars reticulata of 30 patients with Parkinson's disease undergoing deep brain stimulation surgery. We found tone modulated and oddball modu...
Source: Neurobiology of Disease - April 1, 2024 Category: Neurology Authors: Dallas Leavitt Frhan I Alanazi Tameem M Al-Ozzi Melanie Cohn Mojgan Hodaie Suneil K Kalia Andres M Lozano Luka Milosevic William D Hutchison Source Type: research
Auditory oddball responses in the human subthalamic nucleus and substantia nigra pars reticulata
Neurobiol Dis. 2024 Mar 30:106490. doi: 10.1016/j.nbd.2024.106490. Online ahead of print.ABSTRACTThe auditory oddball is a mainstay in research on attention, novelty, and sensory prediction. How this task engages subcortical structures like the subthalamic nucleus and substantia nigra pars reticulata is unclear. We administered an auditory OB task while recording single unit activity (35 units) and local field potentials (57 recordings) from the subthalamic nucleus and substantia nigra pars reticulata of 30 patients with Parkinson's disease undergoing deep brain stimulation surgery. We found tone modulated and oddball modu...
Source: Neurobiology of Disease - April 1, 2024 Category: Neurology Authors: Dallas Leavitt Frhan I Alanazi Tameem M Al-Ozzi Melanie Cohn Mojgan Hodaie Suneil K Kalia Andres M Lozano Luka Milosevic William D Hutchison Source Type: research
Sex-specific vulnerabilities in human astrocytes underpin the differential impact of palmitic acid
Neurobiol Dis. 2024 Mar 27:106489. doi: 10.1016/j.nbd.2024.106489. Online ahead of print.ABSTRACTObesity and neurometabolic diseases have been linked to neurodegenerative diseases. Our hypothesis is that the endogenous estrogenic component of human astrocytes plays a critical role in cell response during lipotoxic damage, given that obesity can disrupt hormonal homeostasis and cause brain inflammation. Our findings showed that high concentrations of palmitic acid (PA) significantly reduced cell viability more in male astrocytes, indicating sex-specific vulnerabilities. PA induced a greater increase in cytosolic reactive ox...
Source: Neurobiology of Disease - March 29, 2024 Category: Neurology Authors: Oscar Hidalgo-Lanussa Janneth Santos George E Barreto Source Type: research
Hippocampal Pyk2 regulates specific social skills: Implications for schizophrenia
Neurobiol Dis. 2024 Mar 27;194:106487. doi: 10.1016/j.nbd.2024.106487. Online ahead of print.ABSTRACTPyk2 has been shown previously to be involved in several psychological and cognitive alterations related to stress, Huntington's disease, and Alzheimer's disease. All these disorders are accompanied by different types of impairments in sociability, which has recently been linked to improper mitochondrial function. We hypothesize that Pyk2, which regulates mitochondria, could be associated with the regulation of mitochondrial dynamics and social skills. In the present manuscript, we report that a reduction of Pyk2 levels in ...
Source: Neurobiology of Disease - March 29, 2024 Category: Neurology Authors: Laura L ópez-Molina Anna Sancho-Balsells Omar Al-Massadi Enrica Montalban Jordi Alberch Bel én Arranz Jean-Antoine Girault Albert Giralt Source Type: research
Sex-specific vulnerabilities in human astrocytes underpin the differential impact of palmitic acid
Neurobiol Dis. 2024 Mar 27:106489. doi: 10.1016/j.nbd.2024.106489. Online ahead of print.ABSTRACTObesity and neurometabolic diseases have been linked to neurodegenerative diseases. Our hypothesis is that the endogenous estrogenic component of human astrocytes plays a critical role in cell response during lipotoxic damage, given that obesity can disrupt hormonal homeostasis and cause brain inflammation. Our findings showed that high concentrations of palmitic acid (PA) significantly reduced cell viability more in male astrocytes, indicating sex-specific vulnerabilities. PA induced a greater increase in cytosolic reactive ox...
Source: Neurobiology of Disease - March 29, 2024 Category: Neurology Authors: Oscar Hidalgo-Lanussa Janneth Santos George E Barreto Source Type: research
Hippocampal Pyk2 regulates specific social skills: Implications for schizophrenia
Neurobiol Dis. 2024 Mar 27;194:106487. doi: 10.1016/j.nbd.2024.106487. Online ahead of print.ABSTRACTPyk2 has been shown previously to be involved in several psychological and cognitive alterations related to stress, Huntington's disease, and Alzheimer's disease. All these disorders are accompanied by different types of impairments in sociability, which has recently been linked to improper mitochondrial function. We hypothesize that Pyk2, which regulates mitochondria, could be associated with the regulation of mitochondrial dynamics and social skills. In the present manuscript, we report that a reduction of Pyk2 levels in ...
Source: Neurobiology of Disease - March 29, 2024 Category: Neurology Authors: Laura L ópez-Molina Anna Sancho-Balsells Omar Al-Massadi Enrica Montalban Jordi Alberch Bel én Arranz Jean-Antoine Girault Albert Giralt Source Type: research
Sex-specific vulnerabilities in human astrocytes underpin the differential impact of palmitic acid
Neurobiol Dis. 2024 Mar 27:106489. doi: 10.1016/j.nbd.2024.106489. Online ahead of print.ABSTRACTObesity and neurometabolic diseases have been linked to neurodegenerative diseases. Our hypothesis is that the endogenous estrogenic component of human astrocytes plays a critical role in cell response during lipotoxic damage, given that obesity can disrupt hormonal homeostasis and cause brain inflammation. Our findings showed that high concentrations of palmitic acid (PA) significantly reduced cell viability more in male astrocytes, indicating sex-specific vulnerabilities. PA induced a greater increase in cytosolic reactive ox...
Source: Neurobiology of Disease - March 29, 2024 Category: Neurology Authors: Oscar Hidalgo-Lanussa Janneth Santos George E Barreto Source Type: research
Hippocampal Pyk2 regulates specific social skills: Implications for schizophrenia
Neurobiol Dis. 2024 Mar 27:106487. doi: 10.1016/j.nbd.2024.106487. Online ahead of print.ABSTRACTPyk2 has been shown previously to be involved in several psychological and cognitive alterations related to stress, Huntington's disease, and Alzheimer's disease. All these disorders are accompanied by different types of impairments in sociability, which has recently been linked to improper mitochondrial function. We hypothesize that Pyk2, which regulates mitochondria, could be associated with the regulation of mitochondrial dynamics and social skills. In the present manuscript, we report that a reduction of Pyk2 levels in mous...
Source: Neurobiology of Disease - March 29, 2024 Category: Neurology Authors: Laura L ópez-Molina Anna Sancho-Balsells Omar Al-Massadi Enrica Montalban Jordi Alberch Bel én Arranz Jean-Antoine Girault Albert Giralt Source Type: research
Proteomics insights into fragile X syndrome: Unraveling molecular mechanisms and therapeutic avenues
Neurobiol Dis. 2024 Mar 26:106486. doi: 10.1016/j.nbd.2024.106486. Online ahead of print.ABSTRACTFragile X Syndrome (FXS) is a neurodevelopment disorder characterized by cognitive impairment, behavioral challenges, and synaptic abnormalities, with a genetic basis linked to a mutation in the FMR1 (Fragile X Messenger Ribonucleoprotein 1) gene that results in a deficiency or absence of its protein product, Fragile X Messenger Ribonucleoprotein (FMRP). In recent years, mass spectrometry (MS) - based proteomics has emerged as a powerful tool to uncover the complex molecular landscape underlying FXS. This review provides a comp...
Source: Neurobiology of Disease - March 28, 2024 Category: Neurology Authors: Diana A Abbasi Elizabeth Berry-Kravis Xinyu Zhao Stephanie M Cologna Source Type: research
Corrigendum to "Upregulation of tripeptidyl-peptidase 1 by 3-hydroxy-(2,2)-dimethyl butyrate, a brain endogenous ligand of PPAR α: Implications for late-infantile Batten disease therapy" [Neurobiology of Disease 127 (2019) 362-373]
Neurobiol Dis. 2024 Mar 27:106479. doi: 10.1016/j.nbd.2024.106479. Online ahead of print.NO ABSTRACTPMID:38548486 | DOI:10.1016/j.nbd.2024.106479 (Source: Neurobiology of Disease)
Source: Neurobiology of Disease - March 28, 2024 Category: Neurology Authors: Sudipta Chakrabarti Sujyoti Chandra Avik Roy Madhuchhanda Kundu Kalipada Pahan Source Type: research
Proteomics insights into fragile X syndrome: Unraveling molecular mechanisms and therapeutic avenues
Neurobiol Dis. 2024 Mar 26:106486. doi: 10.1016/j.nbd.2024.106486. Online ahead of print.ABSTRACTFragile X Syndrome (FXS) is a neurodevelopment disorder characterized by cognitive impairment, behavioral challenges, and synaptic abnormalities, with a genetic basis linked to a mutation in the FMR1 (Fragile X Messenger Ribonucleoprotein 1) gene that results in a deficiency or absence of its protein product, Fragile X Messenger Ribonucleoprotein (FMRP). In recent years, mass spectrometry (MS) - based proteomics has emerged as a powerful tool to uncover the complex molecular landscape underlying FXS. This review provides a comp...
Source: Neurobiology of Disease - March 28, 2024 Category: Neurology Authors: Diana A Abbasi Elizabeth Berry-Kravis Xinyu Zhao Stephanie M Cologna Source Type: research
Corrigendum to "Upregulation of tripeptidyl-peptidase 1 by 3-hydroxy-(2,2)-dimethyl butyrate, a brain endogenous ligand of PPAR α: Implications for late-infantile Batten disease therapy" [Neurobiology of Disease 127 (2019) 362-373]
Neurobiol Dis. 2024 Mar 27:106479. doi: 10.1016/j.nbd.2024.106479. Online ahead of print.NO ABSTRACTPMID:38548486 | DOI:10.1016/j.nbd.2024.106479 (Source: Neurobiology of Disease)
Source: Neurobiology of Disease - March 28, 2024 Category: Neurology Authors: Sudipta Chakrabarti Sujyoti Chandra Avik Roy Madhuchhanda Kundu Kalipada Pahan Source Type: research
Investigation of microglial diversity in a LRRK2 G2019S mouse model of Parkinson's disease
Neurobiol Dis. 2024 Mar 23:106481. doi: 10.1016/j.nbd.2024.106481. Online ahead of print.ABSTRACTMicroglia contribute to the outcomes of various pathological conditions including Parkinson's disease (PD). Microglia are heterogenous, with a variety of states recently identified in aging and neurodegenerative disease models. Here, we delved into the diversity of microglia in a preclinical PD model featuring the G2019S mutation in LRRK2, a known pathological mutation associated with PD. Specifically, we investigated the 'dark microglia' (DM) and the 'disease-associated microglia' (DAM) which present a selective enrichment of ...
Source: Neurobiology of Disease - March 25, 2024 Category: Neurology Authors: L Iovino J Vander Zwaag G Kaur M Khakpour V Giusti M Donadon A Chiavegato L Tenorio-Lopes E Greggio M E Tremblay L Civiero Source Type: research
