Reversal of a full-length mutant huntingtin neuronal cell phenotype by chemical inhibitors of polyglutamine-mediated aggregation 

    Wang, Jin; Ginés Padrós, Silvia; MacDonald, Marcy E.; Gusella, James F. (2009-03-20)

    Background: Huntington's disease (HD) is an inherited neurodegenerative disorder triggered by an expanded polyglutamine tract in huntingtin that is thought to confer a new conformational property on this large protein. The ...

    Muscle protein waste in tumor-bearing rats is effectively antagonized by a beta 2-adrenergic agonist (clenbuterol). Role of the ATP-ubiquitin-dependent proteolytic pathway. 

    Costelli, Paola; García Martínez, Celia; Llovera i Tomàs, Marta; Carbó Carbó, Neus; López-Soriano, Francisco J.; Agell i Jané, Neus; Tessitore, Luciana; Baccino, Francesco M.; Argilés Huguet, Josep Ma. (2009-05-14)

    Tissue protein hypercatabolism (TPH) is a most important feature in cancer cachexia, particularly with regard to the skeletal muscle. The rat ascites hepatoma Yoshida AH-130 is a very suitable model system for studying the ...

    Platelet-activating factor causes ventilation-perfusion mismatch in humans 

    Rodríguez-Roisin, Robert; Félez Flor, Miguel Ángel; Chung, K. Fan, 1951-; Barberà i Mir, Joan Albert; Wagner, P. D. (Peter D.); Cobos Carbó, Albert; Barnes, Peter J.; Roca Torrent, Josep (2009-05-14)

    We hypothesized that platelet-activating factor (PAF), a potent inflammatory mediator, could induce gas exchange abnormalities in normal humans. To this end, the effect of aerosolized PAF (2 mg/ml solution; 24 micrograms) ...

    Defective TNF-alpha-mediated hepatocellular apoptosis and liver damage in acidic sphingomyelinase knockout mice 

    García Ruiz, Carmen; Colell Riera, Anna; Marí García, Montserrat; Morales Muñoz, Albert; Calvo Ademuz, Maria; Enrich Bastús, Carles; Fernández-Checa Torres, José Carlos (2009-05-15)

    This study addressed the contribution of acidic sphingomyelinase (ASMase) in TNF-alpha-mediated hepatocellular apoptosis. Cultured hepatocytes depleted of mitochondrial glutathione (mGSH) became sensitive to TNF-alpha, ...

    Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase 

    Borrell Pagès, Maria; Canals i Coll, Josep M.; Cordelières, Fabrice P.; Parker, J. Alex; Pineda Martí, José Ramón; Grange, Ghislaine; Bryson, Elzbieta A.; Guillermier, Martine; Hirsch, Etienne; Hantraye, Philippe; Cheetham, Michael E.; Néri, Christian; Alberch i Vié, Jordi, 1959-; Brouillet, Emmanuel; Saudou, Frédéric; Humbert, Sandrine (2009-05-15)

    There is no treatment for the neurodegenerative disorder Huntington disease (HD). Cystamine is a candidate drug; however, the mechanisms by which it operates remain unclear. We show here that cystamine increases levels of ...

    Nolz1 promotes striatal neurogenesis through the regulation of retinoic acid signaling 

    Urbán Avellaneda, Noelia; Martín Ibáñez, Raquel; Herranz, Cristina; Esgleas, Miriam; Crespo, Empar; Pardo Muñoz, Mònica; Crespo-Enríquez, Ivan; Méndez-Gómez, Héctor R.; Waclaw, Ronald; Chatzi, Christina; Álvarez, Susana; Álvarez, Rosana; Duester, Gregg; Campbell, Kenneth; Lera, Ángel R. de; Vicario Abejón, Carlos; Martínez, Salvador; Alberch i Vié, Jordi, 1959-; Canals i Coll, Josep M. (2010-09-21)

    Background: Nolz1 is a zinc finger transcription factor whose expression is enriched in the lateral ganglionic eminence (LGE), although its function is still unknown. Results: Here we analyze the role of Nolz1 during LGE ...

    Effects of prolonged ethanol intake and malnutrition on rat pancreas 

    López Blanco, José Manuel; Bombí, Josep Antoni; Valderrama Labarca, Rodrigo; Giménez Lagunas, América; Parés Darnaculleta, Albert; Caballeria Rovira, Joan; Imperial Ródenas, Santiago; Navarro Colás, Salvador (2011-07-07)

    Nutritional factors, especially the protein and fat content of the diet, may change pancreatic morphology after ethanol induced injury. This study was performed to delineate the combined effects of a low fat diet and ...

    Immunoelectron microscopic localization of transforming growth factor alpha in rat colon 

    Pérez Tomás, Ricardo E.; Culleré i Luengo, F. Xavier; Asbert i Alsina, Mònica; Díaz Ruiz, Carmen (2011-07-07)

    Transforming growth factor alpha (TGF alpha) is a polypeptide, which binds to the epidermal growth factor receptor to carry out its function related to cell proliferation and differentiation. The ultrastructural localisation ...

    Conditional BDNF release under pathological conditions improves Huntington's disease pathology by delaying neuronal dysfunction 

    Giralt Torroella, Albert; Carreton, Olga; Lao-Peregrin, Cristina; Martín, Eduardo D.; Alberch i Vié, Jordi, 1959- (2011-11-15)

    Background Brain-Derived Neurotrophic Factor (BDNF) is the main candidate for neuroprotective therapy for Huntington's disease (HD), but its conditional administration is one of its most challenging problems. Results Here ...

    Increased 90-kDa ribosomal S6 kinase (Rsk) activity is protective against mutant huntingtin toxicity 

    Xifró i Collsamata, Xavier; Anglada Huguet, Marta; Rué Cabré, Laura; Saavedra, Ana; Pérez Navarro, Esther; Alberch i Vié, Jordi, 1959- (2011-11-30)

    Background The 90-kDa ribosomal S6 kinase (Rsk) family is involved in cell survival. Rsk activation is regulated by sequential phosphorylations controlled by extracellular signal-regulated kinase (ERK) 1/2 and 3-phosphoi ...

    Cholesterol transport from late endosomes to the Golgi regulates t-SNARE trafficking, assembly, and function 

    Reverter Martín, Meritxell; Rentero Alfonso, Carles; Vilà de Muga, Sandra; Álvarez-Guaita, Anna; Mulay, Vishwaroop; Wood, Peta; Monastyrskaya, Katia; Pol i Sorolla, Albert; Tebar Ramon, Francesc; Blasi Cabús, Joan; Grewal, Thomas; Enrich Bastús, Carles; Cairns, Rose (2012-02-02)

    Cholesterol regulates plasma membrane (PM) association and functioning of syntaxin-4 and soluble N-ethylmaleimide-sensitive fusion protein 23 (SNAP23) in the secretory pathway. However, the molecular mechanism and cellular ...

    Gp74 a membrane glycoprotein of the cis-Golgi network that cycles through the endoplasmic reticulum and intermediate compartment 

    Alcalde, José; Egea Guri, Gustavo; Sandoval, Ignacio V. (2012-04-27)

    A monoclonal antibody CC92 (IgM), raised against a fraction of rat liver enriched in Golgi membranes, recognizes a novel Endo H-resistant 74-kD membrane glycoprotein (gp74). The bulk of gp74 is confined to the cis-Golgi ...

    PDMP blocks brefeldin a-induced retrograde membrane transport from Golgi to ER: evidence for involvement of calcium homeostasis and dissociation from sphingolipid metabolism 

    Kok, Jan Willem; Babià i Díez, Teresa; Filipenau, Catalin M.; Nelemans, Adriaan; Egea Guri, Gustavo; Hoekstra, Dick (2012-04-27)

    In this study, we show that an inhibitor of sphingolipid biosynthesis, d,l-threo-1-phenyl-2- decanoylamino-3-morpholino-1-propanol (PDMP), inhibits brefeldin A (BFA)-induced retrograde membrane transport from Golgi to ...

    Regulation of protein transport from the Golgi complex to the endoplasmic reticulum by CDC42 and N-WASP. 

    Luna Moñux, Ana; Matas Guadix, Olga Belén; Martínez-Menárguez, José Ángel; Mato Matute, Maria Eugènia; Durán Serrano, Juan Manuel; Ballesta, José; Way, Michael; Egea Guri, Gustavo (2012-04-27)

    Actin is involved in the organization of the Golgi complex and Golgi-to-ER protein transport in mammalian cells. Little, however, is known about the regulation of the Golgi-associated actin cytoskeleton. We provide evidence ...

    Myosin motors and not actin comets are mediators of the actin-based Golgi-to-endoplasmic reticulum protein transport 

    Durán Serrano, Juan Manuel; Valderrama i Alfonso, Ferran; Castel i Gil, Susanna; Magdalena, Juana; Tomás, Mónica; Hosoya, Hiroshi; Renau Piqueras, Jaime; Malhotra, Vivek; Egea Guri, Gustavo (2012-04-27)

    We have previously reported that actin filaments are involved in protein transport from the Golgi complex to the endoplasmic reticulum. Herein, we examined whether myosin motors or actin comets mediate this transport. To ...

    Diacylglycerol is required for the formation of COPI vesicles in the Golgi-to-ER transport pathway 

    Sarri Plans, Elisabet; Martínez Rodríguez, Susana Eva; Jiménez, Nuria; Claro Izaguirre, Enrique; Mérida, Isabel; Burger, Koert N. J.; Egea Guri, Gustavo; Fernández Ulibarri, Inés; Vilella, Montserrat; Lázaro Diégez, Francisco (2012-04-27)

    Diacylglycerol is necessary for trans-Golgi network (TGN) to cell surface transport, but its functional relevance in the early secretory pathway is unclear. Although depletion of diacylglycerol did not affect ER-to-Golgi ...

    Vacuole membrane protein 1 is an endoplasmic reticulum protein required for organelle biogenesis, protein secretion, and development 

    Calvo-Garrido, Javier; Carilla-Latorre, Sergio; Lázaro Diégez, Francisco; Egea Guri, Gustavo; Escalante, Ricardo (2012-04-27)

    Vacuole membrane protein 1 (Vmp1) is membrane protein of unknown molecular function that has been associated with pancreatitis and cancer. The social amoeba Dictyostelium discoideum has a vmp1-related gene that we identified ...

    Mutant Huntingtin impairs post-Golgi trafficking to lysosomes by delocalizing optineurin/Rab8 complex from the Golgi apparatus 

    Toro, Daniel del; Alberch i Vié, Jordi, 1959-; Lázaro Diégez, Francisco; Martín Ibáñez, Raquel; Xifró i Collsamata, Xavier; Egea Guri, Gustavo; Canals i Coll, Josep M. (2012-04-27)

    Huntingtin regulates post-Golgi trafficking of secreted proteins. Here, we studied the mechanism by which mutant huntingtin impairs this process. Colocalization studies and Western blot analysis of isolated Golgi membranes ...

    Clearance of a hirano body-like F-actin aggresome generated by jasplakinolide 

    Lázaro Diégez, Francisco; Knecht, Erwin; Egea Guri, Gustavo (2012-04-27)

    Addendum to: Lázaro-Diéguez F, Aguado C, Mato E, Sánchez-Ruíz Y, Esteban I, Alberch J, Knecht E, Egea G. Dynamics of an F-actin aggresome generated by the actin-stabilizing toxin jasplakinolide. J Cell Sci 2008; 121:1415-25.

    Activation of Srk1 by the Mitogen-activated Protein Kinase Sty1/Spc1 Precedes Its Dissociation from the Kinase and Signals Its Degradation 

    López Avilés, Sandra; Lambea Martínez, Eva; Moldón Vara, Alberto; Grande Robles, Maribel; Fajardo, Alba; Rodríguez-Gabriel, Miguel A.; Hidalgo Hernando, Elena; Aligué i Alemany, Rosa Maria (2012-05-09)

    Control of cell cycle progression by stress-activated protein kinases (SAPKs) is essential for cell adaptation to extracellular stimuli. The Schizosaccharomyces pombe SAPK Sty1/Spc1 orchestrates general changes in gene ...