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7 results on '"Comella Bolla, Andrea"'

2. Astrocyte dysfunction and neuronal network hyperactivity in a CRISPR engineered pluripotent stem cell model of frontotemporal dementia

Author

Swedish Society for Medical Research, Swedish Research Council, Swedish Alzheimer Foundation, Agencia Estatal de Investigación (España), Ministerio de Ciencia e Innovación (España), European Commission, Canals, Isaac [0000-0002-2689-268X], Comella Bolla, Andrea [0000-0001-9480-5050], Cepeda-Prado, E. [0000-0001-9781-3742], Avaliani, Natalia [0000-0002-2986-1520], Crowe, James A. [0000-0003-4487-9048], Oburoglu, Leal [0000-0003-0130-6602], Bruzelius, Andreas [0000-0002-7175-3590], Pajares, María A. [0000-0002-4714-9051], Pérez-Sala, Dolores [0000-0003-0600-665X], Heuer, Andreas [0000-0003-0300-7606], Rylander Ottosson, Daniella [0000-0002-9270-3576], Soriano, Jordi [0000-0003-2676-815X], Ahlenius, Henrik [0000-0001-8958-6148], Canals, Isaac, Comella Bolla, Andrea, Cepeda-Prado, E., Avaliani, Natalia, Crowe, James A., Oburoglu, Leal, Bruzelius, Andreas, King, Naomi, Pajares, María A., Pérez-Sala, Dolores, Heuer, Andreas, Rylander Ottosson, Daniella, Soriano, Jordi, Ahlenius, Henrik, Swedish Society for Medical Research, Swedish Research Council, Swedish Alzheimer Foundation, Agencia Estatal de Investigación (España), Ministerio de Ciencia e Innovación (España), European Commission, Canals, Isaac [0000-0002-2689-268X], Comella Bolla, Andrea [0000-0001-9480-5050], Cepeda-Prado, E. [0000-0001-9781-3742], Avaliani, Natalia [0000-0002-2986-1520], Crowe, James A. [0000-0003-4487-9048], Oburoglu, Leal [0000-0003-0130-6602], Bruzelius, Andreas [0000-0002-7175-3590], Pajares, María A. [0000-0002-4714-9051], Pérez-Sala, Dolores [0000-0003-0600-665X], Heuer, Andreas [0000-0003-0300-7606], Rylander Ottosson, Daniella [0000-0002-9270-3576], Soriano, Jordi [0000-0003-2676-815X], Ahlenius, Henrik [0000-0001-8958-6148], Canals, Isaac, Comella Bolla, Andrea, Cepeda-Prado, E., Avaliani, Natalia, Crowe, James A., Oburoglu, Leal, Bruzelius, Andreas, King, Naomi, Pajares, María A., Pérez-Sala, Dolores, Heuer, Andreas, Rylander Ottosson, Daniella, Soriano, Jordi, and Ahlenius, Henrik

Abstract

Frontotemporal dementia is the second most prevalent type of early-onset dementia and up to 40% of cases are familial forms. One of the genes mutated in patients is CHMP2B, which encodes a protein found in a complex important for maturation of late endosomes, an essential process for recycling membrane proteins through the endolysosomal system. Here, we have generated a CHMP2B-mutated human embryonic stem cell line using genome editing with the purpose to create a human in vitro Frontotemporal dementia disease model. To date, most studies have focused on neuronal alterations; however, we present a new co-culture system in which neurons and astrocytes are independently generated from human embryonic stem cells and combined in co-cultures. With this approach, we have identified alterations in the endolysosomal system of Frontotemporal dementia astrocytes, a higher capacity of astrocytes to uptake and respond to glutamate, and a neuronal network hyperactivity as well as excessive synchronization. Overall, our data indicates that astrocyte alterations precede neuronal impairments and could potentially trigger neuronal network changes, indicating the important and specific role of astrocytes in disease development.

Published
2023

3. Astrocyte dysfunction and neuronal network hyperactivity in a CRISPR engineered pluripotent stem cell model of frontotemporal dementia

Author

Canals, Isaac, primary, Comella-Bolla, Andrea, additional, Cepeda-Prado, Efrain, additional, Avaliani, Natalia, additional, Crowe, James A, additional, Oburoglu, Leal, additional, Bruzelius, Andreas, additional, King, Naomi, additional, Pajares, María A, additional, Pérez-Sala, Dolores, additional, Heuer, Andreas, additional, Rylander Ottosson, Daniella, additional, Soriano, Jordi, additional, and Ahlenius, Henrik, additional

Published
2023
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5. CD200 is up-regulated in R6/1 transgenic mouse model of Huntington's disease

Author

Ministerio de Ciencia, Innovación y Universidades (España), Agencia Estatal de Investigación (España), Instituto de Salud Carlos III, European Commission, Centro Investigación Biomédica en Red Enfermedades Neurodegenerativas (España), Red de Terapia Celular (España), Generalitat de Catalunya, CHDI Foundation, Miguez, Andrés [0000-0001-6014-3685], Straccia, Marco [0000-0003-3903-4163], Canals, Josep María [0000-0001-6829-7670], Comella Bolla, Andrea, Valente, Tony, Miguez, Andrés, Brito, Verónica, Ginés, Silvia, Solà, Carme, Straccia, Marco, Canals, Josep María, Ministerio de Ciencia, Innovación y Universidades (España), Agencia Estatal de Investigación (España), Instituto de Salud Carlos III, European Commission, Centro Investigación Biomédica en Red Enfermedades Neurodegenerativas (España), Red de Terapia Celular (España), Generalitat de Catalunya, CHDI Foundation, Miguez, Andrés [0000-0001-6014-3685], Straccia, Marco [0000-0003-3903-4163], Canals, Josep María [0000-0001-6829-7670], Comella Bolla, Andrea, Valente, Tony, Miguez, Andrés, Brito, Verónica, Ginés, Silvia, Solà, Carme, Straccia, Marco, and Canals, Josep María

Abstract

In Huntington’s disease (HD), striatal medium spiny neurons (MSNs) are particularly sensitive to the presence of a CAG repeat in the huntingtin (HTT) gene. However, there are many evidences that cells from the peripheral immune system and central nervous system (CNS) immune cells, namely microglia, play an important role in the etiology and the progression of HD. However, it remains unclear whether MSNs neurodegeneration is mediated by a non-cell autonomous mechanism. The homeostasis in the healthy CNS is maintained by several mechanisms of interaction between all brain cells. Neurons can control microglia activation through several inhibitory mechanisms, such as the CD200–CD200R1 interaction. Due to the complete lack of knowledge about the CD200–CD200R1 system in HD, we determined the temporal patterns of CD200 and CD200R1 expression in the neocortex, hippocampus and striatum in the HD mouse models R6/1 and HdhQ111/7 from pre-symptomatic to manifest stages. In order to explore any alteration in the peripheral immune system, we also studied the levels of expression of CD200 and CD200R1 in whole blood. Although CD200R1 expression was not altered, we observed and increase in CD200 gene expression and protein levels in the brain parenchyma of all the regions we examined, along with HD pathogenesis in R6/1 mice. Interestingly, the expression of CD200 mRNA was also up-regulated in blood following a similar temporal pattern. These results suggest that canonical neuronal–microglial communication through CD200–CD200R1 interaction is not compromised, and CD200 up-regulation in R6/1 brain parenchyma could represent a neurotrophic signal to sustain or extend neuronal function in the latest stages of HD as pro-survival mechanism.

Published
2019

7. Astrocyte dysfunction and neuronal network hyperactivity in a CRISPR engineered pluripotent stem cell model of frontotemporal dementia

Author

Isaac Canals, Andrea Comella-Bolla, Efrain Cepeda-Prado, Natalia Avaliani, James A Crowe, Leal Oburoglu, Andreas Bruzelius, Naomi King, María A Pajares, Dolores Pérez-Sala, Andreas Heuer, Daniella Rylander Ottosson, Jordi Soriano, Henrik Ahlenius, Swedish Society for Medical Research, Swedish Research Council, Swedish Alzheimer Foundation, Agencia Estatal de Investigación (España), Ministerio de Ciencia e Innovación (España), European Commission, Canals, Isaac, Comella Bolla, Andrea, Cepeda-Prado, E., Avaliani, Natalia, Crowe, James A., Oburoglu, Leal, Bruzelius, Andreas, Pajares, María A., Pérez-Sala, Dolores, Heuer, Andreas, Rylander Ottosson, Daniella, Soriano, Jordi, and Ahlenius, Henrik

Subjects
CRISPR/Cas9 genome editing, Astròcits, Stem cells, Electrophysiology, Cellular and Molecular Neuroscience, Psychiatry and Mental health, Neuronal network analysis, Neurology, Astrocytes, Human pluripotent stem cells, Electrofisiologia, Cèl·lules mare, Biological Psychiatry, Frontotemporal dementia
Abstract

31p.-7 fig. 1 graph. abst., Frontotemporal dementia is the second most prevalent type of early-onset dementia and up to 40% of cases are familial forms. One of the genes mutated in patients is CHMP2B, which encodes a protein found in a complex important for maturation of late endosomes, an essential process for recycling membrane proteins through the endolysosomal system. Here, we have generated a CHMP2B-mutated human embryonic stem cell line using genome editing with the purpose to create a human in vitro Frontotemporal dementia disease model. To date, most studies have focused on neuronal alterations; however, we present a new co-culture system in which neurons and astrocytes are independently generated from human embryonic stem cells and combined in co-cultures. With this approach, we have identified alterations in the endolysosomal system of Frontotemporal dementia astrocytes, a higher capacity of astrocytes to uptake and respond to glutamate, and a neuronal network hyperactivity as well as excessive synchronization. Overall, our data indicates that astrocyte alterations precede neuronal impairments and could potentially trigger neuronal network changes, indicating the important and specific role of astrocytes in disease development., This study was supported by funding from the Swedish Society for Medical Research (SSMF, S20-0003), Kockska, Segerfalk and Hardebo foundations to IC; the Swedish Research Council (VR:2018-02695), Swedish Alzheimer and Åhlen foundations to HA; the Swedish Research Council (VR:2016-01789) to AH; and the Agencia Estatal de Investigación, Ministerio de Ciencia e Innovación (RTI2018-097624-BI00) and European Regional Development Fund to DPS.

Published
2023

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