Search Results - "Sousa, Mónica Mendes"

The Actin-Binding Protein α-Adducin Is Required for Maintaining Axon Diameter by Leite, Sérgio Carvalho, Sampaio, Paula, Sousa, Vera Filipe, Nogueira-Rodrigues, Joana, Pinto-Costa, Rita, Peters, Luanne Laurel, Brites, Pedro, Sousa, Mónica Mendes

“…The actin-binding protein adducin was recently identified as a component of the neuronal subcortical cytoskeleton. Here, we analyzed mice lacking adducin to…”
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The Regulation of Axon Diameter: From Axonal Circumferential Contractility to Activity-Dependent Axon Swelling by Costa, Ana Rita, Pinto-Costa, Rita, Sousa, Sara Castro, Sousa, Mónica Mendes

“…In the adult nervous system axon caliber varies widely amongst different tracts. When considering a given axon, its diameter can further fluctuate in space and…”
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Early axonal loss accompanied by impaired endocytosis, abnormal axonal transport, and decreased microtubule stability occur in the model of Krabbe's disease by Teixeira, Carla Andreia, Miranda, Catarina Oliveira, Sousa, Vera Filipe, Santos, Telma Emanuela, Malheiro, Ana Rita, Solomon, Melani, Maegawa, Gustavo H, Brites, Pedro, Sousa, Mónica Mendes

“…Abstract In Krabbe's disease (KD), a leukodystrophy caused by β-galactosylceramidase deficiency, demyelination and a myelin-independent axonopathy contributes…”
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The role of brain-derived neurotrophic factor (BDNF) in the development of neurogenic detrusor overactivity (NDO) by Frias, Bárbara, Santos, João, Morgado, Marlene, Sousa, Mónica Mendes, Gray, Susannah M Y, McCloskey, Karen D, Allen, Shelley, Cruz, Francisco, Cruz, Célia Duarte

“…Neurogenic detrusor overactivity (NDO) is a well known consequence of spinal cord injury (SCI), recognizable after spinal shock, during which the bladder is…”
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Axonal pathology in Krabbe's disease: The cytoskeleton as an emerging therapeutic target by Nogueira-Rodrigues, Joana, Brites, Pedro, Sousa, Mónica Mendes

“…In Krabbe's disease (KD), demyelination and myelin‐independent axonal and neuronal defects contribute to the severe neuropathology. The toxic substrate that…”
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ApoA-I cleaved by transthyretin has reduced ability to promote cholesterol efflux and increased amyloidogenicity by Liz, Márcia Almeida, Gomes, Cláudio M., Saraiva, Maria João, Sousa, Mónica Mendes

“…A fraction of plasma transthyretin (TTR) circulates in HDL through binding to apolipoprotein A-I (apoA-I). Moreover, TTR is able to cleave the C terminus of…”
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Evidence for Early Cytotoxic Aggregates in Transgenic Mice for Human Transthyretin Leu55Pro by Sousa, Mónica Mendes, Fernandes, Rui, Palha, Joana Almeida, Taboada, Ana, Vieira, Paulo, Saraiva, Maria João

“…Familial amyloidotic polyneuropathy (FAP) is a lethal autosomal dominant disorder characterized by systemic extracellular deposition of transthyretin (TTR)…”
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Familial Amyloid Polyneuropathy: Receptor for Advanced Glycation End Products-Dependent Triggering of Neuronal Inflammatory and Apoptotic Pathways by Sousa, Monica Mendes, Du Yan, Shi, Fernandes, Rui, Guimaraes, Antonio, Stern, David, Saraiva, Maria Joao

“…Familial amyloid polyneuropathy (FAP) is a neurodegenerative disorder associated with extracellular deposition of mutant transthyretin (TTR) amyloid fibrils,…”
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Primary bone marrow mesenchymal stromal cells rescue the axonal phenotype of Twitcher mice by Miranda, Catarina Oliveira, Teixeira, Carla Andreia, Sousa, Vera Filipe, Santos, Telma Emanuela, Liz, Márcia Almeida, Marques, Ana Maio, Pinto-do-Ó, Perpétua, Sousa, Mónica Mendes

“…Krabbe's disease (KD) is a demyelinating disorder caused by the deficiency of lysosomal galactocerebrosidase (GALC), affecting both the central (CNS) and the…”
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Up-regulation of the extracellular matrix remodeling genes, biglycan, neutrophil gelatinase-associated lipocalin, and matrix metalloproteinase-9 in familial amyloid polyneuropathy by Sousa, Mónica Mendes, do Amaral, José Barbas, Guimarães, António, Saraiva, Maria João

“…ABSTRACT Familial amyloid polyneuropathy (FAP) is characterized by extracellular deposition of transthyretin (TTR) aggregates and amyloid fibrils, particularly…”
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Neuronal Intrinsic Regenerative Capacity: The Impact of Microtubule Organization and Axonal Transport by Murillo, Blanca, Mendes Sousa, Mónica

“…ABSTRACT In the adult vertebrate central nervous system, axons generally fail to regenerate. In contrast, peripheral nervous system axons are able to form a…”
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Coherent-hybrid STED: high contrast sub-diffraction imaging using a bi-vortex depletion beam by Pereira, António, Sousa, Mafalda, Almeida, Ana C., Ferreira, Luísa T., Costa, Ana Rita, Cruz, Marco Gabriel Novais, Ferrás, Cristina, Sousa, Mónica Mendes, Sampaio, Paula, Belsley, M., Maiato, Helder

“…Stimulated emission depletion (STED) fluorescence microscopy squeezes an excited spot well below the wavelength scale using a doughnut-shaped depletion beam…”
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Transthyretin in high density lipoproteins: association with apolipoprotein A-I by Sousa, M M, Berglund, L, Saraiva, M J

“…Previous studies have revealed the presence of transthyretin (TTR) on lipoproteins. To further address this issue, we fractionated plasma lipoproteins from 9…”
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Deposition of Transthyretin in Early Stages of Familial Amyloidotic Polyneuropathy : Evidence for Toxicity of Nonfibrillar Aggregates by Mendes Sousa, Monica, Cardoso, Isabel, Fernandes, Rui, Guimaraes, Antonio, Saraiva, Maria Joao

“…Familial amyloidotic polyneuropathy (FAP) is a neurodegenerative disorder characterized by extracellular deposition of transthyretin (TTR) amyloid fibrils,…”
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Regenerative medicine for the treatment of spinal cord injury: more than just promises? by Pêgo, Ana Paula, Kubinova, Sarka, Cizkova, Dasa, Vanicky, Ivo, Mar, Fernando Milhazes, Sousa, Mónica Mendes, Sykova, Eva

“…Spinal cord injury triggers a complex set of events that lead to tissue healing without the restoration of normal function due to the poor regenerative…”
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Apolipoprotein AI and Transthyretin as Components of Amyloid Fibrils in a Kindred with apoAI Leu178His Amyloidosis by de Sousa, Mónica Mendes, Vital, Claude, Ostler, Dominique, Fernandes, Rui, Pouget-Abadie, Jean, Carles, Dominique, Saraiva, Maria João

“…We found a new C-terminal amyloidogenic variant of apolipoprotein AI (apoAI), Leu178His in a French kindred, associated with cardiac and larynx amyloidosis and…”
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Advances and pitfalls of cell therapy in metabolic leukodystrophies by Miranda, Catarina Oliveira, Brites, Pedro, Mendes Sousa, Mónica, Teixeira, Carla Andreia

“…Leukodystrophies are a group of disorders characterized by myelin dysfunction, either at the level of myelin formation or maintenance, that affect the central…”
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The intriguing nature of dorsal root ganglion neurons: Linking structure with polarity and function by Nascimento, Ana Isabel, Mar, Fernando Milhazes, Sousa, Mónica Mendes

“…Dorsal root ganglion (DRG) neurons are the first neurons of the sensory pathway. They are activated by a variety of sensory stimuli that are then transmitted…”
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Systemic Delivery of Bone Marrow‐Derived Mesenchymal Stromal Cells Diminishes Neuropathology in a Mouse Model of Krabbe's Disease by Miranda, Catarina Oliveira, Teixeira, Carla Andreia, Liz, Márcia Almeida, Sousa, Vera Filipe, Franquinho, Filipa, Forte, Giancarlo, Di Nardo, Paolo, Pinto‐Do‐Ó, Perpétua, Sousa, Mónica Mendes

“…In Krabbe's disease, a demyelinating disorder, add‐on strategies targeting the peripheral nervous system (PNS) are needed, as it is not corrected by…”
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Transthyretin knockouts are a new mouse model for increased neuropeptide Y by Nunes, Ana Filipa, Saraiva, Maria João, Sousa, Mónica Mendes

“…Transthyretin (TTR) has access to the brain and nerve through the blood and cerebrospinal fluid. To investigate TTR function in nervous system homeostasis,…”
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