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13 p, 8.3 MB |
Microglial response promotes neurodegeneration in the Ndufs4 KO mouse model of Leigh syndrome
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Aguilar, Kevin (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Comes, Gemma (Universitat Autònoma de Barcelona. Departament de Biologia Cel·lular, de Fisiologia i d'Immunologia) ;
Canal Capdevila, Carla (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Quintana Romero, Albert (Universitat Autònoma de Barcelona. Departament de Biologia Cel·lular, de Fisiologia i d'Immunologia) ;
Sanz, Elisenda (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Hidalgo Pareja, Juan (Universitat Autònoma de Barcelona. Departament de Biologia Cel·lular, de Fisiologia i d'Immunologia)
Leigh syndrome is a mitochondrial disease characterized by neurodegeneration, neuroinflammation, and early death. Mice lacking NDUFS4, a mitochondrial complex I subunit (Ndufs4 KO mice), have been established as a good animal model for studying human pathology associated with Leigh syndrome. [...]
2022 - 10.1002/glia.24234
GLIA, Vol. 70, Issue 11 (November 2022) , p. 2032-2044
2 documents
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2.
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16 p, 1.9 MB |
Interleukin-4 and interleukin-13 induce different metabolic profiles in microglia and macrophages that relate with divergent outcomes after spinal cord injury
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Amo Aparicio, Jesús (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Garcia Garcia, Joana (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Francos Quijorna, Isaac (Regeneration Group, Wolfson Centre for Age-Related Diseases, IoPPN, King's College London) ;
Urpi, Andrea (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Esteve-Codina, Anna (Universitat Pompeu Fabra) ;
Gut, Marta (Universitat Pompeu Fabra) ;
Quintana Romero, Albert (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Lopez-Vales, Ruben (Universitat Autònoma de Barcelona. Institut de Neurociències)
Background: Microglia and macrophages adopt a pro-inflammatory phenotype after spinal cord injury (SCI), what is thought to contribute to secondary tissue degeneration. We previously reported that this is due, in part, to the low levels of anti-inflammatory cytokines, such as IL-4. [...]
2021 - 10.7150/thno.65203
Theranostics, Vol. 11 Num. 20 (october 2021) , p. 9805-9820
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3.
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14 p, 2.9 MB |
Mitochondrial Proteome of Affected Glutamatergic Neurons in a Mouse Model of Leigh Syndrome
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Gella, Alejandro (Universitat Autònoma de Barcelona. Departament de Bioquímica i de Biologia Molecular) ;
Prada-Dacasa, Patricia (Universitat Autònoma de Barcelona. Departament de Biologia Cel·lular, de Fisiologia i d'Immunologia) ;
Carrascal Pérez, Montserrat (Institut d'Investigacions Biomèdiques de Barcelona) ;
Urpi, Andrea (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
González-Torres, Melania (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Abian, Joaquin (Institut d'Investigacions Biomèdiques de Barcelona) ;
Sanz, Elisenda (Universitat Autònoma de Barcelona. Departament de Biologia Cel·lular, de Fisiologia i d'Immunologia) ;
Quintana Romero, Albert (Universitat Autònoma de Barcelona. Departament de Biologia Cel·lular, de Fisiologia i d'Immunologia)
Defects in mitochondrial function lead to severe neuromuscular orphan pathologies known as mitochondrial disease. Among them, Leigh Syndrome is the most common pediatric presentation, characterized by symmetrical brain lesions, hypotonia, motor and respiratory deficits, and premature death. [...]
2020 - 10.3389/fcell.2020.00660
Frontiers in Cell and Developmental Biology, Vol. 8 (july 2020)
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26 p, 4.7 MB |
Defined neuronal populations drive fatal phenotype in a mouse model of leigh syndrome
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Bolea Tomás, Irene (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Gella, Alejandro (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Sanz, Elisenda (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Prada Dacasa, Patricia (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Menardy, Fabien (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Bard, Angela M. (Seattle Children's Research Institute) ;
Machuca Márquez, Pablo (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Eraso-Pichot, Abel (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Mòdol Caballero, Guillem (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Navarro, X. (Xavier) (Xavier) (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Kalume, Frank (Department of Pharmacology. University of Washington) ;
Quintana Romero, Albert (Universitat Autònoma de Barcelona. Institut de Neurociències)
Mitochondrial deficits in energy production cause untreatable and fatal pathologies known as mitochondrial disease (MD). Central nervous system affectation is critical in Leigh Syndrome (LS), a common MD presentation, leading to motor and respiratory deficits, seizures and premature death. [...]
2019 - 10.7554/eLife.47163
eLife, Vol. 8 (august 2019) , p. e47163
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13 p, 1.8 MB |
Interleukin-6, a Major Cytokine in the Central Nervous System
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Erta Cañabate, Maria (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Quintana Romero, Albert (University of Washington. Departament of Chemistry) ;
Hidalgo Pareja, Juan (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Universitat Autònoma de Barcelona.
Departament de Biologia Cel·lular, de Fisiologia i d'Immunologia
Interleukin-6 (IL-6) is a cytokine originally identified almost 30 years ago as a B-cell differentiation factor, capable of inducing the maturation of B cells into antibody-producing cells. As with many other cytokines, it was soon realized that IL-6 was not a factor only involved in the immune response, but with many critical roles in major physiological systems including the nervous system. [...]
2012 - 10.7150/ijbs.4679
International journal of biological sciences, Vol. 8, Num. 9 (2012) , p. 1254-1266
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7.
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9 p, 846.3 KB |
Striatal GPR88 modulates foraging efficiency
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Rainwater, Aundrea (Howard Hughes Medical Institute (Maryland, Estats Units d'Amèrica)) ;
Sanz Iglesias, Elisenda (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Palmiter, Richard D. (Howard Hughes Medical Institute (Maryland, Estats Units d'Amèrica)) ;
Quintana Romero, Albert (Universitat Autònoma de Barcelona. Institut de Neurociències)
The striatum is anatomically and behaviorally implicated in behaviors that promote efficient foraging. To investigate this function, we studied instrumental choice behavior in mice lacking GPR88, a striatum-enriched orphan G-protein-coupled receptor that modulates striatal medium spiny neuron excitability. [...]
2017 - 10.1523/JNEUROSCI.2439-16.2017
The Journal of neuroscience, Vol. 37, Núm. 33 (August 2017) , p. 7939-7947
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8.
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8 p, 2.2 MB |
Loss of mitochondrial Ndufs4 in striatal medium spiny neurons mediates progressive motor impairment in a mouse model of leigh syndrome
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Chen, Byron (University of Washington. Howard Hughes Medical Institute) ;
Hui, Jessica (Seattle Children's Research Institute (Seattle, Estats Units d'Amèrica)) ;
Montgomery, Kelsey S. (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Gella, Alejandro (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Bolea Tomás, Irene (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Sanz Iglesias, Elisenda (Universitat Autònoma de Barcelona. Institut de Neurociències) ;
Palmiter, Richard D. (Howard Hughes Medical Institute (Maryland, Estats Units d'Amèrica)) ;
Quintana Romero, Albert (Universitat Autònoma de Barcelona. Institut de Neurociències)
Inability of mitochondria to generate energy leads to severe and often fatal myoencephalopathies. Among these, Leigh syndrome (LS) is one of the most common childhood mitochondrial diseases; it is characterized by hypotonia, failure to thrive, respiratory insufficiency and progressive mental and motor dysfunction, leading to early death. [...]
2017 - 10.3389/fnmol.2017.00265
Frontiers in molecular neuroscience, Vol. 10 (August 2017) , article 265
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9 p, 1.4 MB |
Severely impaired learning and altered neuronal morphology in mice lacking NMDA receptors in medium spiny neurons
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Beutler, Lisa R. (University of Washington. Department of Genome Sciences) ;
Eldred, Kiara C. (Howard Hughes Medical Institute (Estats Units d'Amèrica)) ;
Quintana Romero, Albert (University of Washington. Department of Biochemistry) ;
Keene, C. Dirk (University of Washington. Department of Pathology) ;
Rose, Shannon E. (University of Washington. Department of Pathology) ;
Postupna, Nadia (University of Washington. Department of Pathology) ;
Montine, Thomas J. (University of Washington. Department of Pathology) ;
Palmiter, Richard D. (University of Washington. Department of Genome Sciences)
The striatum is composed predominantly of medium spiny neurons (MSNs) that integrate excitatory, glutamatergic inputs from the cortex and thalamus, and modulatory dopaminergic inputs from the ventral midbrain to influence behavior. [...]
2011 - 10.1371/journal.pone.0028168
PloS one, Vol. 6 Num. 11 (November 2011) , p. e28168
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5 p, 216.6 KB |
Altered Anesthetic Sensitivity of Mice Lacking Ndufs4, a Subunit of Mitochondrial Complex I
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Quintana Romero, Albert (University of Washington. Howard Hughes Medical Institute and Department of Biochemistry) ;
Morgan, Philip G. (University of Washington. Department of Anesthesiology and Pain Medicine) ;
Kruse, Shane E. (University of Washington. Howard Hughes Medical Institute and Department of Biochemistry) ;
Sedensky, Margaret M. (Seattle Children's Research Institute)
Anesthetics are in routine use, yet the mechanisms underlying their function are incompletely understood. Studies in vitro demonstrate that both GABAA and NMDA receptors are modulated by anesthetics, but whole animal models have not supported the role of these receptors as sole effectors of general anesthesia. [...]
2012 - 10.1371/journal.pone.0042904
PloS one, Vol. 7, Num. 8 (2012) , p. e42904
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