GM1-Ganglioside Accumulation at the Mitochondria-Associated ER Membranes Links ER Stress to Ca2+-Dependent Mitochondrial Apoptosis
Sano, Renata, Annunziata, Ida, Patterson, Annette, Moshiach, Simon, Gomero, Elida, Opferman, Joseph, Forte, Michael, d'Azzo, Alessandra
Published in Molecular cell (13.11.2009)
Published in Molecular cell (13.11.2009)
Get full text
Journal Article
Altered GM1 catabolism affects NMDAR-mediated Ca2+ signaling at ER-PM junctions and increases synaptic spine formation in a GM1-gangliosidosis model
Weesner, Jason A., Annunziata, Ida, van de Vlekkert, Diantha, Robinson, Camenzind G., Campos, Yvan, Mishra, Ashutosh, Fremuth, Leigh E., Gomero, Elida, Hu, Huimin, d’Azzo, Alessandra
Published in Cell reports (Cambridge) (28.05.2024)
Published in Cell reports (Cambridge) (28.05.2024)
Get full text
Journal Article
Alix-mediated assembly of the actomyosin–tight junction polarity complex preserves epithelial polarity and epithelial barrier
Campos, Yvan, Qiu, Xiaohui, Gomero, Elida, Wakefield, Randall, Horner, Linda, Brutkowski, Wojciech, Han, Young-Goo, Solecki, David, Frase, Sharon, Bongiovanni, Antonella, d’Azzo, Alessandra
Published in Nature communications (23.06.2016)
Published in Nature communications (23.06.2016)
Get full text
Journal Article
Regulated lysosomal exocytosis mediates cancer progression
Machado, Eda, White-Gilbertson, Shai, van de Vlekkert, Diantha, Janke, Laura, Moshiach, Simon, Campos, Yvan, Finkelstein, David, Gomero, Elida, Mosca, Rosario, Qiu, Xiaohui, Morton, Christopher L, Annunziata, Ida, d'Azzo, Alessandra
Published in Science advances (01.12.2015)
Published in Science advances (01.12.2015)
Get full text
Journal Article
Excessive exosome release is the pathogenic pathway linking a lysosomal deficiency to generalized fibrosis
van de Vlekkert, Diantha, Demmers, Jeroen, Nguyen, Xinh-Xinh, Campos, Yvan, Machado, Eda, Annunziata, Ida, Hu, Huimin, Gomero, Elida, Qiu, Xiaohui, Bongiovanni, Antonella, Feghali-Bostwick, Carol A, d'Azzo, Alessandra
Published in Science advances (01.07.2019)
Published in Science advances (01.07.2019)
Get full text
Journal Article
AAV-mediated gene therapy for galactosialidosis: A long-term safety and efficacy study
Hu, Huimin, Mosca, Rosario, Gomero, Elida, van de Vlekkert, Diantha, Campos, Yvan, Fremuth, Leigh E., Brown, Scott A., Weesner, Jason A., Annunziata, Ida, d’Azzo, Alessandra
Published in Molecular therapy. Methods & clinical development (10.12.2021)
Published in Molecular therapy. Methods & clinical development (10.12.2021)
Get full text
Journal Article
Preclinical Enzyme Replacement Therapy with a Recombinant β-Galactosidase-Lectin Fusion for CNS Delivery and Treatment of GM1-Gangliosidosis
Weesner, Jason Andrew, Annunziata, Ida, Yang, Tianhong, Acosta, Walter, Gomero, Elida, Hu, Huimin, van de Vlekkert, Diantha, Ayala, Jorge, Qiu, Xiaohui, Fremuth, Leigh Ellen, Radin, David N, Cramer, Carole L, d'Azzo, Alessandra
Published in Cells (Basel, Switzerland) (01.08.2022)
Published in Cells (Basel, Switzerland) (01.08.2022)
Get full text
Journal Article
Galactosialidosis: preclinical enzyme replacement therapy in a mouse model of the disease, a proof of concept
Cadaoas, Jaclyn, Hu, Huimin, Boyle, Gabrielle, Gomero, Elida, Mosca, Rosario, Jayashankar, Kartika, Machado, Mike, Cullen, Sean, Guzman, Belle, van de Vlekkert, Diantha, Annunziata, Ida, Vellard, Michel, Kakkis, Emil, Koppaka, Vish, d’Azzo, Alessandra
Published in Molecular therapy. Methods & clinical development (12.03.2021)
Published in Molecular therapy. Methods & clinical development (12.03.2021)
Get full text
Journal Article
Muscle degeneration in neuraminidase 1-deficient mice results from infiltration of the muscle fibers by expanded connective tissue
Zanoteli, Edmar, de Vlekkert, Diantha van, Bonten, Erik J., Hu, Huimin, Mann, Linda, Gomero, Elida M., Harris, A. John, Ghersi, Giulio, d'Azzo, Alessandra
Published in Biochimica et biophysica acta (01.07.2010)
Published in Biochimica et biophysica acta (01.07.2010)
Get full text
Journal Article
GM1-ganglioside accumulation at the mitochondria-associated ER membranes links ER stress to Ca(2+)-dependent mitochondrial apoptosis
Sano, Renata, Annunziata, Ida, Patterson, Annette, Moshiach, Simon, Gomero, Elida, Opferman, Joseph, Forte, Michael, d'Azzo, Alessandra
Published in Molecular cell (13.11.2009)
Published in Molecular cell (13.11.2009)
Get full text
Journal Article
Lysosomal NEU1 deficiency affects amyloid precursor protein levels and amyloid-β secretion via deregulated lysosomal exocytosis
Annunziata, Ida, Patterson, Annette, Helton, Danielle, Hu, Huimin, Moshiach, Simon, Gomero, Elida, Nixon, Ralph, d’Azzo, Alessandra
Published in Nature communications (14.11.2013)
Published in Nature communications (14.11.2013)
Get full text
Journal Article
AAV-mediated gene therapy in a mouse model of sialidosis
van de Vlekkert, Diantha, Hu, Huimin, Weesner, Jason A., Gomero, Elida M., d'Azzo, Alessandra
Published in Molecular genetics and metabolism (01.02.2023)
Published in Molecular genetics and metabolism (01.02.2023)
Get full text
Journal Article
AAV-mediated gene therapy for sialidosis
van de Vlekkert, Diantha, Hu, Huimin, Weesner, Jason A., Fremuth, Leigh E., Brown, Scott A., Lu, Meifen, Gomero, Elida, Campos, Yvan, Sheppard, Heather, d’Azzo, Alessandra
Published in Molecular therapy (03.07.2024)
Published in Molecular therapy (03.07.2024)
Get full text
Journal Article
Distinct functions of dimeric and monomeric scaffold protein Alix in regulating F-actin assembly and loading of exosomal cargo
Qiu, Xiaohui, Campos, Yvan, van de Vlekkert, Diantha, Gomero, Elida, Tanwar, Ajay C., Kalathur, Ravi, Weesner, Jason A., Bongiovanni, Antonella, Demmers, Jeroen, d’Azzo, Alessandra
Published in The Journal of biological chemistry (01.10.2022)
Published in The Journal of biological chemistry (01.10.2022)
Get full text
Journal Article
Preclinical enzyme replacement therapy with a recombinant β-galactosidase-lectin fusion for CNS delivery and treatment of GM1-gangliosidosis
Weesner, Jason A., van de Vlekkert, Diantha, Acosta, Walter L., Yang, Tianhong, Gomero, Elida M., Hu, Huimin, Radin, David, Cramer, Carole L., d'Azzo, Alessandra
Published in Molecular genetics and metabolism (01.02.2023)
Published in Molecular genetics and metabolism (01.02.2023)
Get full text
Journal Article
Chaperone mediated gene therapy for type I sialidosis
Annunziata, Ida, Hu, Huimin, Gomero, Elida, d'Azzo, Alessandra
Published in Molecular genetics and metabolism (01.02.2016)
Published in Molecular genetics and metabolism (01.02.2016)
Get full text
Journal Article
Altered GM1 catabolism affects NMDAR-mediated Ca 2+ signaling at ER-PM junctions and increases synaptic spine formation in a GM1-gangliosidosis model
Weesner, Jason A, Annunziata, Ida, van de Vlekkert, Diantha, Robinson, Camenzind G, Campos, Yvan, Mishra, Ashutosh, Fremuth, Leigh E, Gomero, Elida, Hu, Huimin, d'Azzo, Alessandra
Published in Cell reports (Cambridge) (28.05.2024)
Get full text
Published in Cell reports (Cambridge) (28.05.2024)
Journal Article
Preclinical Dose-Finding Study With a Liver-Tropic, Recombinant AAV-2/8 Vector in the Mouse Model of Galactosialidosis
Hu, Huimin, Gomero, Elida, Bonten, Erik, Gray, John T, Allay, Jim, Wu, Yanan, Wu, Jianrong, Calabrese, Christopher, Nienhuis, Arthur, d'Azzo, Alessandra
Published in Molecular therapy (01.02.2012)
Published in Molecular therapy (01.02.2012)
Get full text
Journal Article