Results 131 to 140 of about 2,946,385 (264)

Systemic delivery of AAV-GFM1 corrects COXPD1 molecular alterations in Gfm1 R671C/− mice

EMBO Molecular Medicine
Hepatoencephalopathy due to mutations in the nuclear gene GFM1, known as combined oxidative phosphorylation (OXPHOS) deficiency type I (COXPD1), is an autosomal recessive mitochondrial disease caused by defects or deficiency of the mitochondrial ...
Miguel Molina-Berenguer, Diego Herrero-Martínez, Antoni Vallbona-Garcia, Ferran Vila-Julià, Yolanda Cámara, África Vales, Gloria González-Aseguinolaza, Javier Torres-Torronteras, Ramon Martí   +8 more
doaj   +1 more source

Neurologic Manifestations of Long COVID Affect Adult Females More Severely Than Males

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To characterize differences in neurologic manifestations of postacute sequelae of SARS‐CoV‐2 infection (Neuro‐PASC) between females and males. Methods Cross‐sectional study of the first consecutive 261 posthospitalization Neuro‐PASC (PNP) and 2068 nonhospitalized Neuro‐PASC (NNP) patients evaluated at the Neuro‐COVID clinic between ...
Hannah Kopinsky, Melissa Lopez, Janet Miller, Millenia Jimenez, Eric M. Liotta, Igor J. Koralnik   +5 more
wiley   +1 more source

Mitochondrial diseases

, 2016
Mitochondrial diseases are a group of genetic disorders that are characterized by defects in oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) and mitochondrial DNA (mtDNA) that encode structural mitochondrial proteins ...
Koga Y., Chinnery PF, DiMauro S., Hirano M, McFarland R, Suomalainen A, Zeviani M., Koga Y, Gorman GS, Suomalainen A., Chinnery P. F., Zeviani M, Hirano M., DiMauro S, Gorman G. S., Turnbull DM, McFarland R., Turnbull D. M., Thorburn D. R., Thorburn DR   +19 more
core   +2 more sources

Quantitative MRI Uncovers Subtle Cortical Damage in Myelin Oligodendrocyte Glycoprotein Antibody‐Associated Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To determine whether myelin‐sensitive quantitative MRI reveals microstructural abnormalities in normal‐appearing cortex (NACtx) in myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD), indicating that conventional MRI underestimates remission residual cortical injury.
Valentina Camera, Agnese Tamanti, Silvia Messina, Nicola Dall'Osto, Teresa Maltempo, Stefano Ziccardi, Matteo Foschi, Maria Grazia Piscaglia, Diana Ferraro, Francesco Crescenzo, Francesca Rossi, Albulena Bajrami, Sabrina Marangoni, Damiano Marastoni, Francesca Benedetta Pizzini, Maria Isabel Leite, Roberta Magliozzi, Patrick Waters, Massimiliano Calabrese, Jacqueline Palace, Ruth Geraldes   +20 more
wiley   +1 more source

Thalamic nuclei volumes are related to disease stage in patients with amyotrophic lateral sclerosis

Frontiers in Neuroscience
ObjectiveTo explore atrophy patterns in thalamic nuclei at different phases of amyotrophic lateral sclerosis (ALS) and determine any correlations between thalamic nucleus volume and either cognitive impairments or motor disabilities.MethodsWe used the ...
Tianrui Wen, Tianrui Wen, Jun Zhu, Jun Zhu, Sujuan Sun, Sujuan Sun, Yujing Chen, Yujing Chen, Ninglu Gao, Ninglu Gao, Mingjie Ma, Xinyue Chen, Shuangwu Liu, Shuangwu Liu, Pengfei Lin, Yan Deng   +15 more
doaj   +1 more source

Baseline Neuroinflammation Stratifies TSPO‐PET Response to Disease‐Modifying Therapy in Multiple Sclerosis

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To investigate which baseline clinical and imaging characteristics best predict TSPO‐PET‐measurable reduction in glial activation following treatment of multiple sclerosis (MS), to utilize this information for designing more efficient biomarker‐based clinical trials targeting glial activation.
Marlene T. Morch, Markus Matilainen, Giulia N. Baldrighi, Marjo Nylund, Maija Saraste, Laura Airas   +5 more
wiley   +1 more source

Intracerebral Hemorrhage Induces Monocyte TNF Signaling in Patients That Is Suppressed by BAF312

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Intracerebral hemorrhage (ICH) causes high morbidity and mortality, with neurotoxic inflammation driven by infiltrating monocytes. This study is an in‐depth longitudinal examination of the immune response during the first week of ICH in the presence and absence of the immunomodulatory drug BAF312 (Siponimod).
Jonathan Howard DeLong, Sebastian Díaz‐Pérez, Kevin Navin Sheth, Jang‐Ho Cha, C. J. Malanga, Patricia G. M. Wagner, Nicole Pezous, Aurelie Hanin, Kyle B. Walsh, H. E. Hinson, Lauren Hachmann Sansing   +10 more
wiley   +1 more source

Vascular Disease, Non‐Steroidal Anti‐Inflammatory Drugs and Risk of Parkinson's Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objectives A history of vascular disease has been shown to be associated with an increased risk of Parkinson's disease, but this may be due to reverse causation. In addition, non‐steroidal anti‐inflammatory drugs are thought to reduce Parkinson's disease risk, but evidence is inconsistent and confounding by prior vascular disease is possible ...
Clare Wotton, Carol Hermon, Sarah Floud, Gillian Reeves, Isobel Barnes   +4 more
wiley   +1 more source

Proteomic analysis of cellular models of neurodegeneration and mitochondrial dysfunction [PDF]

, 2009
Mitochondrial dysfunction is thought to contribute to neurodegenerative processes. As an example, dysfunction of complex I of the electron transport chain has been observed in Parkinson’s disease patients and 1-methyl-4-phenyl-1,2,3,6 tetrahydropyridine (
Burté, F, Burte, Florence
core  

Mitochondria transplantation transiently rescues cerebellar neurodegeneration improving mitochondrial function and reducing mitophagy in mice

Nature Communications
Cerebellar ataxia is the primary manifestation of cerebellar degenerative diseases, and mitochondrial dysfunction in Purkinje cells (PCs) plays a critical role in disease progression.
Shu-Jiao Li, Qian-Wen Zheng, Jie Zheng, Jin-Bao Zhang, Hui Liu, Jing-Jing Tie, Kun-Long Zhang, Fei-Fei Wu, Xiao-Dong Li, Shuai Zhang, Xin Sun, Yan-Ling Yang, Ya-Yun Wang   +12 more
doaj   +1 more source