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Your search keyword '"Carrella, Diego"' showing total 4 results
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4 results on '"Carrella, Diego"'

1. Author Correction: Impaired mitophagy links mitochondrial disease to epithelial stress in methylmalonyl-CoA mutase deficiency

Author

Luciani, Alessandro, Schumann, Anke, Berquez, Marine, Chen, Zhiyong; https://orcid.org/0000-0003-0203-2930, Nieri, Daniela, Failli, Mario, Debaix, Huguette, Festa, Beatrice Paola, Tokonami, Natsuko, Raimondi, Andrea; https://orcid.org/0000-0002-4563-386X, Cremonesi, Alessio, Carrella, Diego, Forny, Patrick; https://orcid.org/0000-0003-1877-2976, Kölker, Stefan, Camassei, Francesca Diomedi, Diaz, Francisca, Moraes, Carlos T, Di Bernardo, Diego; https://orcid.org/0000-0002-1911-7407, Baumgartner, Matthias R; https://orcid.org/0000-0002-9270-0826, Devuyst, Olivier; https://orcid.org/0000-0003-3744-4767, Luciani, Alessandro, Schumann, Anke, Berquez, Marine, Chen, Zhiyong; https://orcid.org/0000-0003-0203-2930, Nieri, Daniela, Failli, Mario, Debaix, Huguette, Festa, Beatrice Paola, Tokonami, Natsuko, Raimondi, Andrea; https://orcid.org/0000-0002-4563-386X, Cremonesi, Alessio, Carrella, Diego, Forny, Patrick; https://orcid.org/0000-0003-1877-2976, Kölker, Stefan, Camassei, Francesca Diomedi, Diaz, Francisca, Moraes, Carlos T, Di Bernardo, Diego; https://orcid.org/0000-0002-1911-7407, Baumgartner, Matthias R; https://orcid.org/0000-0002-9270-0826, and Devuyst, Olivier; https://orcid.org/0000-0003-3744-4767

Published
2020

2. Impaired mitophagy links mitochondrial disease to epithelial stress in methylmalonyl-CoA mutase deficiency

Author

Luciani, Alessandro, Schumann, Anke, Berquez, Marine, Chen, Zhiyong; https://orcid.org/0000-0003-0203-2930, Nieri, Daniela, Failli, Mario, Debaix, Huguette, Festa, Beatrice Paola, Tokonami, Natsuko, Raimondi, Andrea; https://orcid.org/0000-0002-4563-386X, Cremonesi, Alessio; https://orcid.org/0000-0002-1524-4169, Carrella, Diego, Forny, Patrick; https://orcid.org/0000-0003-1877-2976, Kölker, Stefan, Diomedi Camassei, Francesca, Diaz, Francisca, Moraes, Carlos T, Di Bernardo, Diego; https://orcid.org/0000-0002-1911-7407, Baumgartner, Matthias R; https://orcid.org/0000-0002-9270-0826, Devuyst, Olivier; https://orcid.org/0000-0003-3744-4767, Luciani, Alessandro, Schumann, Anke, Berquez, Marine, Chen, Zhiyong; https://orcid.org/0000-0003-0203-2930, Nieri, Daniela, Failli, Mario, Debaix, Huguette, Festa, Beatrice Paola, Tokonami, Natsuko, Raimondi, Andrea; https://orcid.org/0000-0002-4563-386X, Cremonesi, Alessio; https://orcid.org/0000-0002-1524-4169, Carrella, Diego, Forny, Patrick; https://orcid.org/0000-0003-1877-2976, Kölker, Stefan, Diomedi Camassei, Francesca, Diaz, Francisca, Moraes, Carlos T, Di Bernardo, Diego; https://orcid.org/0000-0002-1911-7407, Baumgartner, Matthias R; https://orcid.org/0000-0002-9270-0826, and Devuyst, Olivier; https://orcid.org/0000-0003-3744-4767

Abstract

Deregulation of mitochondrial network in terminally differentiated cells contributes to a broad spectrum of disorders. Methylmalonic acidemia (MMA) is one of the most common inherited metabolic disorders, due to deficiency of the mitochondrial methylmalonyl-coenzyme A mutase (MMUT). How MMUT deficiency triggers cell damage remains unknown, preventing the development of disease-modifying therapies. Here we combine genetic and pharmacological approaches to demonstrate that MMUT deficiency induces metabolic and mitochondrial alterations that are exacerbated by anomalies in PINK1/Parkin-mediated mitophagy, causing the accumulation of dysfunctional mitochondria that trigger epithelial stress and ultimately cell damage. Using drug-disease network perturbation modelling, we predict targetable pathways, whose modulation repairs mitochondrial dysfunctions in patient-derived cells and alleviate phenotype changes in mmut-deficient zebrafish. These results suggest a link between primary MMUT deficiency, diseased mitochondria, mitophagy dysfunction and epithelial stress, and provide potential therapeutic perspectives for MMA.

Published
2020

3. Genetic and pharmacological regulation of the endocannabinoid CB1 receptor in Duchenne muscular dystrophy

Author

Ombretta Guardiola, Fabio Arturo Iannotti, Giuseppe Busetto, Ester Pagano, Simone Adinolfi, Silvia Consalvi, Diego Carrella, Gabriella Minchiotti, Pier Lorenzo Puri, Valentina Saccone, Fabiana Piscitelli, Raffaele Capasso, Vincenzo Di Marzo, Elisabetta Gazzerro, Iannotti, F abio Arturo, Pagano, Ester, Guardiola, Ombretta, Adinolfi, Simone, Saccone, Valentina, Consalvi, Silvia, Piscitelli, Fabiana, Gazzerro, Elisabetta, Busetto, Giuseppe, Carrella, Diego, Capasso, Raffaele, Puri Pier, Lorenzo, Minchiotti, Gabriella, and Di Marzo, Vincenzo

Subjects
0301 basic medicine, Cannabinoid receptor, Transcription, Genetic, Cellular differentiation, Duchenne muscular dystrophy, Endocannabinoidi, Sistema endocannabinoide, General Physics and Astronomy, Rimonabant, Receptor, Cannabinoid, CB1, Settore BIO/13 - BIOLOGIA APPLICATA, Muscular dystrophy, lcsh:Science, Receptor, Luciferases, Multidisciplinary, musculoskeletal, neural, and ocular physiology, PAX7 Transcription Factor, food and beverages, Endocannabinoid system, 3. Good health, Cell biology, medicine.anatomical_structure, lipids (amino acids, peptides, and proteins), Function and Dysfunction of the Nervous System, psychological phenomena and processes, medicine.drug, musculoskeletal diseases, Science, lipid signalling, Arachidonic Acids, Biology, Motor Activity, General Biochemistry, Genetics and Molecular Biology, Article, Glycerides, Diglycerides, 03 medical and health sciences, medicine, Animals, Humans, Regeneration, RNA, Messenger, Muscle, Skeletal, Muscle Cells, neuromuscular disease, Base Sequence, Skeletal muscle, General Chemistry, medicine.disease, Mice, Inbred C57BL, Muscular Dystrophy, Duchenne, 030104 developmental biology, HEK293 Cells, nervous system, Mice, Inbred mdx, lcsh:Q, Biomarkers, Endocannabinoids
Abstract

The endocannabinoid system refers to a widespread signaling system and its alteration is implicated in a growing number of human diseases. However, the potential role of endocannabinoids in skeletal muscle disorders remains unknown. Here we report the role of the endocannabinoid CB1 receptors in Duchenne’s muscular dystrophy. In murine and human models, CB1 transcripts show the highest degree of expression at disease onset, and then decline overtime. Similar changes are observed for PAX7, a key regulator of muscle stem cells. Bioinformatics and biochemical analysis reveal that PAX7 binds and upregulates the CB1 gene in dystrophic more than in healthy muscles. Rimonabant, an antagonist of CB1, promotes human satellite cell differentiation in vitro, increases the number of regenerated myofibers, and prevents locomotor impairment in dystrophic mice. In conclusion, our study uncovers a PAX7–CB1 cross talk potentially exacerbating DMD and highlights the role of CB1 receptors as target for potential therapies., The regenerative capacity of muscle stem cells is impaired in Duchenne muscular dystrophy (DMD). Here, the authors show that the endocannabinoid receptor CB1 is activated by PAX7 in muscle stem cells, and that pharmacological inhibition of CB1 promotes stem cell activation and ameliorates symptoms in DMD mouse models.

Published
2018
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4. Goblet Cell Hyperplasia Requires High Bicarbonate Transport To Support Mucin Release.

Author

Gorrieri G, Scudieri P, Caci E, Schiavon M, Tomati V, Sirci F, Napolitano F, Carrella D, Gianotti A, Musante I, Favia M, Casavola V, Guerra L, Rea F, Ravazzolo R, Di Bernardo D, and Galietta LJ

Subjects
Cells, Cultured, Chlorides metabolism, Cytokines metabolism, Gene Expression Profiling, Humans, Hydrogen-Ion Concentration, Bicarbonates metabolism, Goblet Cells metabolism, Hyperplasia pathology, Mucins metabolism
Abstract

Goblet cell hyperplasia, a feature of asthma and other respiratory diseases, is driven by the Th-2 cytokines IL-4 and IL-13. In human bronchial epithelial cells, we find that IL-4 induces the expression of many genes coding for ion channels and transporters, including TMEM16A, SLC26A4, SLC12A2, and ATP12A. At the functional level, we find that IL-4 enhances calcium- and cAMP-activated chloride/bicarbonate secretion, resulting in high bicarbonate concentration and alkaline pH in the fluid covering the apical surface of epithelia. Importantly, mucin release, elicited by purinergic stimulation, requires the presence of bicarbonate in the basolateral solution and is defective in cells derived from cystic fibrosis patients. In conclusion, our results suggest that Th-2 cytokines induce a profound change in expression and function in multiple ion channels and transporters that results in enhanced bicarbonate transport ability. This change is required as an important mechanism to favor release and clearance of mucus.

Published
2016
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