• The role of glial chloride channels in brain homeostasis.
  • Rare genetic diseases caused by chloride-channel dysfunction.
  • The involvement of BDNF-regulated genes in CNS development in animal models and in human neural-development pathologies such as autism spectrum disorders, early-onset epileptic encephalopathies and perinatal brain damage (hypoxia-ischaemia, IUGR, etc.).
  • Modelling the neurovascular niche using metabolic strategies and biomaterials to promote regeneration following a CNS lesion during development.

Postnatal development of the astrocyte perivascular MLC1/GlialCAM complex defines a temporal window for the gliovascular unit maturation

Gilbert A, Vidal XE, Estevez R, Cohen-Salmon M, Boulay AC

BRAIN STRUCTURE AND FUNCTION. (ISSN/ISBN: 1863-2653).

2019

Chloride Channels in Astrocytes: Structure, Roles in Brain Homeostasis and Implications in Disease

Elorza-Vidal X, Gaitán-Peñas H, Estévez R

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES. (ISSN/ISBN: 14220067). 20(5):

2019

Expression of LRRC8/VRAC Currents in Xenopus Oocytes: Advantages and Caveats.

Gaitán-Peñas H, Pusch M, Estévez R

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (ISSN/ISBN: 14220067). 19 (3)

2018

CLCN1 myotonia congenita mutation with a variable pattern of inheritance suggests a novel mechanism of dominant myotonia.

Gaitán-Peñas H, Armand-Ugón M, Macaya A, Estévez R

MUSCLE AND NERVE (ISSN/ISBN: 0148639X)

2018

Megalencephalic leukoencephalopathy with subcortical cysts: A personal biochemical retrospective.

Estévez R, Elorza-Vidal X, Gaitán-Peñas H, Pérez-Rius C, Armand-Ugón M, Alonso-Gardón M, Xicoy-Espaulella E, Sirisi S, Arnedo T, Capdevila-Nortes X, López-Hernández T, Montolio M, Duarri A, Teijido O, Barrallo-Gimeno A, Palacín M, Nunes V

EUROPEAN JOURNAL OF MEDICAL GENETICS (ISSN/ISBN: 17697212)

2018

Deficient LRRC8A-dependent volume-regulated anion channel activity is associated with male infertility in mice

Bao, Jianqiang; Perez, Carlos J.; Kim, Jeesun; Zhang, Huan; Murphy, Caitlin J.; Hamidi, Tewfik; Jaubert, Jean; Platt, Craig D.; Chou, Janet; Deng, Meichun; Zhou, Meng-Hua; Huang, Yuying; Gaitan-Penas, Hector; Guenet, Jean-Louis; Lin, Kevin; Lu, Yue; Chen, Taiping; Bedford, Mark T.; Dent, Sharon Y. R.; Richburg, John H.; Estevez, Raul; Pan, Hui-Lin; Geha, Raif S.; Shi, Qinghua; Benavides, Fernando;

JCI INSIGHT. (ISSN/ISBN: 23793708). 3(16):

2018

GlialCAM/MLC1 modulates LRRC8/VRAC currents in an indirect manner: Implications for megalencephalic leukoencephalopathy.

Elorza-Vidal X, Sirisi S, Gaitán-Peñas H, Pérez-Rius C, Alonso-Gardón M, Armand-Ugón M, Lanciotti A, Brignone MS, Prat E, Nunes V, Ambrosini E, Gasull X, Estévez R

NEUROBIOLOGY OF DISEASE. (ISSN/ISBN: 09699961). 11988-99

2018

Leukoencephalopathy-causing CLCN2 mutations are associated with impaired Cl- channel function and trafficking.

Gaitán-Peñas H, Apaja PM, Arnedo T, Castellanos A, Elorza-Vidal X, Soto D, Gasull X, Lukacs GL, Estévez R

JOURNAL OF PHYSIOLOGY (ISSN/ISBN: 00223751). 595 (22) : 6993-7008

2017

Depolarization causes the formation of a ternary complex between GlialCAM, MLC1 and ClC-2 in astrocytes: implications in megalencephalic leukoencephalopathy.

Sirisi S, Elorza-Vidal X, Arnedo T, Armand-Ugón M, Callejo G, Capdevila-Nortes X, López-Hernández T, Schulte U, Barrallo-Gimeno A, Nunes V, Gasull X, Estévez R

HUMAN MOLECULAR GENETICS (ISSN/ISBN: 09646906). 26 (13) : 2436-2450

2017

Novel Properties of LRRC8-Mediated VRAC Currents

Gradogna, Antonella; Gaitan-Penas, Hector; Laparra-Cuervo, Lara; Solsona, Carles; Fernandez-Duenas, Victor; Barrallo-Gimeno, Alejandro; Ciruela, Francisco; Lakadamyali, Melike; Estevez, Raul; Pusch, Michael;

BIOPHYSICAL JOURNAL (ISSN/ISBN: 00063495). 112 (3)

2017

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Possible therapeutic effect of Biotin to treat MLC disease. (Second pilot test)

Estevez Povedano, Raul (Coordinador del proyecto total, red o consorcio); Nunes Martinez, Virginia (Investigador principal (IP))

Ref.: C14075(SecondPhase)

Entitat finançadora:
  • MEDDAY SAS

2016-01-01 - 2016-12-31

Desarrollo y caracterización inicial de modelos animales del síndrome de Bartter

Estevez Povedano, Raul (Coordinador del proyecto total, red o consorcio); Nunes Martinez, Virginia (Investigador principal (IP))

Entitat finançadora:
  • CIBERER

2016-01-01 - 2016-01-01

Bases moleculares de patologías asociadas a canales de cloruro CLC y LRRC8.

Estevez Povedano, Raul (Participante)

Ref.: SAF2015-70377-R

Entitat finançadora:
  • MINISTERIO DE ECONOMÍA Y COMPETITIVIDAD

Second Pilot test with Frederic Sedel: Possible therapeutic effect of Biotin to treat MLC disease

Estevez Povedano, Raul (Investigador/a)

Ref.: 308497

Entitat finançadora:
  • MEDDAY SAS

Assessorament i la investigació aplicada en el camp de la neurogenètica

Estevez Povedano, Raul (Investigador/a)

Entitat finançadora:
  • DIVERSOS

Ajut per incentivar i consolidar la recerca d'excel·lència ja existent a les universitats públiques de Catalunya. Programa ICREA Academia 2014.

Estevez Povedano, Raul (Participante)

Evaluation of a new treatment for MLC disease

Estevez Povedano, Raul (Investigador/a)

Ref.: 307964

Entitat finançadora:
  • MEDDAY SAS

CLC chloride channels and megalencephalic leukoencephalopathy: molecular mechanisms and therapeutics

Estevez Povedano, Raul (Participante)

Fisiologia i patologia de la relació funcional glia-neurona

Estevez Povedano, Raul (Participante)

Ref.: 2014SGR1178

CLC cloride channels and megalencephalic leukoencephalopathy: molecular mechanisms and therapeutics

Estevez Povedano, Raul (Participante)

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Paper del canal de clorur ClC-2 en les patologies de la mielina

Estevez Povedano, Raul (Director) Doctorando: Tanit Arnedo Llena

2015

Bases Moleculars de la leucoencefalopatia megaencefàlica amb quist subcortical. Utilització de models animals i cel·lulars

Nunes Martinez, Virginia (Director) Doctorando: Sònia Sirisi Dolcet; Estevez Povedano, Raul (Director) Doctorando: Sònia Sirisi Dolcet

2014

Bases moleculars de la leucoencefalopatia amb quists subcorticals (MLC): utilització de models animals i cel.lulars

Estevez Povedano, Raul (Director) Doctorando: Sonia Sirisi Dolçet

2014

Estudis bioquímics i funcionals de les proteïnes implicades en la leucoencefalopatia megalencefàlica amb quists subcorticals

Estevez Povedano, Raul (Director) Doctorando: Xavier Capdevila-Nortes

2013

Aproximaciones bioquímicas y celulares a la fisiopatologia de la leucoencefalopatia megalencefalica

Estevez Povedano, Raul (Director) Doctorando: Tania López Hernández

2012

Avenços en la fisiopatologia de la Leucoencefalopatia Megalencefàlica ajb quists subcorticals.

Nunes Martinez, Virginia (Director) Doctorando: Anna Duarri Piqué; Estevez Povedano, Raul (Director) Doctorando: Anna Duarri Piqué

2010

Caracterización bioquímica y localización de la proteína MLC1, implicada en la leucoencefalopatia megalencefálica

Estevez Povedano, Raul (Director) Doctorando: Oscar Teijido

2007

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