Cargando…

Autophagy delays progression of the two most frequent human monogenetic lethal diseases: cystic fibrosis and Wilson disease

Cystic fibrosis (CF) and Wilson disease (WD) are two monogenetic, recessively inherited lethal pathologies that are caused by ionic disequilibria. CF results from loss-of-function mutations in CF transmembrane conductance regulator (CFTR), a channel that conducts chloride across epithelial cell memb...

Descripción completa

Detalles Bibliográficos
Autores principales:	Maiuri, Luigi, Kroemer, Guido
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Impact Journals 2018
Materias:	Research Perspective
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326686/ https://www.ncbi.nlm.nih.gov/pubmed/30568028 http://dx.doi.org/10.18632/aging.101736

Ejemplares similares

Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis versus gluten-mediated inhibition in celiac disease
por: Maiuri, Luigi, et al.
Publicado: (2019)

Strategies for the etiological therapy of cystic fibrosis
por: Maiuri, Luigi, et al.
Publicado: (2017)

Targeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR Defect
por: Villella, Valeria Rachela, et al.
Publicado: (2013)

Aortic Valve Disease Is Not a Monogenetic Disorder
por: Rajamannan, Nalini M.
Publicado: (2017)

Autophagy suppresses the pathogenic immune response to dietary antigens in cystic fibrosis
por: Villella, Valeria R., et al.
Publicado: (2019)

The Multifaceted Roles of MicroRNAs in Cystic Fibrosis
por: De Palma, Fatima Domenica Elisa, et al.
Publicado: (2020)

Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis
por: Esposito, Speranza, et al.
Publicado: (2016)

Monogenetic Forms of Parkinson’s Disease – Bridging the Gap Between Genetics and Biomarkers
por: Tönges, Lars, et al.
Publicado: (2022)

Evaluation of autophagy inducers in epithelial cells carrying the ΔF508 mutation of the cystic fibrosis transmembrane conductance regulator CFTR
por: Zhang, Shaoyi, et al.
Publicado: (2018)

Evaluating the Genetics of Common Variable Immunodeficiency: Monogenetic Model and Beyond
por: de Valles-Ibáñez, Guillem, et al.
Publicado: (2018)

Future therapies for cystic fibrosis
por: Allen, Lucy, et al.
Publicado: (2023)

p.G970D is the most frequent CFTR mutation in Chinese patients with cystic fibrosis
por: Tian, Xinlun, et al.
Publicado: (2016)

A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease
por: Villella, Valeria R, et al.
Publicado: (2018)

Autophagy-dependent danger signaling and adaptive immunity to poorly immunogenic tumors
por: Kroemer, Guido, et al.
Publicado: (2016)

AKT as a therapeutic target for autophagy induction and cancer therapy
por: Wu, Qi, et al.
Publicado: (2021)

Monogenetic autoinflammatory syndromes and nephrology - therapy is usefull even in advanced kidney failure
por: Hohenstein-Scheibenecker, K, et al.
Publicado: (2015)

Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation
por: Ferrari, Eleonora, et al.
Publicado: (2017)

A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR
por: Tosco, A, et al.
Publicado: (2016)

Characterization of the most frequent ATP7B mutation causing Wilson disease in hepatocytes from patient induced pluripotent stem cells
por: Parisi, Silvia, et al.
Publicado: (2018)

The gliadin-CFTR connection: new perspectives for the treatment of celiac disease
por: Maiuri, Luigi, et al.
Publicado: (2019)

Aspirin induces autophagy via inhibition of the acetyltransferase EP300
por: Castoldi, Francesca, et al.
Publicado: (2018)

Developing Inhaled Antibiotics in Cystic Fibrosis: Current Challenges and Opportunities
por: Nichols, Dave P., et al.
Publicado: (2019)

One versus Many: Polymicrobial Communities and the Cystic Fibrosis Airway
por: Jean-Pierre, Fabrice, et al.
Publicado: (2021)

Correlation between the spatial distribution and colony size was common for monogenetic bacteria in laboratory conditions
por: Xue, Heng, et al.
Publicado: (2021)

Following Excitation/Inhibition Ratio Homeostasis from Synapse to EEG in Monogenetic Neurodevelopmental Disorders
por: Geertjens, Lisa, et al.
Publicado: (2022)

Socio-ecological impact of monogenetic volcanism in the La Garrotxa Volcanic Field (NE Iberia)
por: Revelles, Jordi, et al.
Publicado: (2023)

Ovarian endometrioma increases the embryo aneuploid rate: an analysis of 7092 biopsied blastocysts from fertile monogenetic disease carriers
por: Yan, Niwei, et al.
Publicado: (2023)

Evaluation of liver fibrosis in patients with Wilson’s disease
por: Przybyłkowski, Adam, et al.
Publicado: (2020)

Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation
por: Stefano, Daniela De, et al.
Publicado: (2014)

Defective proteostasis in celiac disease as a new therapeutic target
por: Maiuri, Luigi, et al.
Publicado: (2019)

Cystic Fibrosis–Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes
por: Putman, Melissa S., et al.
Publicado: (2023)

Cystic Fibrosis–Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes
por: Putman, Melissa S., et al.
Publicado: (2023)

Author Correction: Characterization of the most frequent ATP7B mutation causing Wilson disease in hepatocytes from patient induced pluripotent stem cells
por: Parisi, Silvia, et al.
Publicado: (2020)

With newer tools for gene editing, is it time to revisit genetic therapy for cystic fibrosis?
por: Gupta, Anuj, et al.
Publicado: (2020)

CFTR modulators: transformative therapies for cystic fibrosis
por: Dwight, Mary, et al.
Publicado: (2021)

Metabolic Shifts as the Hallmark of Most Common Diseases: The Quest for the Underlying Unity
por: Schwartz, Laurent, et al.
Publicado: (2021)

FOXO1 Content Is Reduced in Cystic Fibrosis and Increases with IGF-I Treatment
por: Smerieri, Arianna, et al.
Publicado: (2014)

Systemic autophagy in the therapeutic response to anthracycline-based chemotherapy
por: Castoldi, Francesca, et al.
Publicado: (2018)

Clinical characteristics of people with cystic fibrosis and frequent fungal infection
por: Poore, T. Spencer, et al.
Publicado: (2021)

Observations on Some of the Most Frequent and Important Diseases of the Heart
Publicado: (1809)

Cannot write session to /tmp/vufind_sessions/sess_8mp7op2scd9tot60h0e12ae6uj