Valeria Rachela Villella
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View article: How Effectively Can Oxidative Stress and Inflammation Be Reversed When CFTR Function Is Pharmacologically Improved?
How Effectively Can Oxidative Stress and Inflammation Be Reversed When CFTR Function Is Pharmacologically Improved? Open
A critical challenge in the age of advanced modulator therapies is to understand and determine how effectively chronic oxidative stress and oxidative stress-induced inflammation can be reversed and physiological balance restored when CFTR …
View article: Effect of CFTR Modulators on Oxidative Stress and Autophagy in Non-CFTR-Expressing Cells
Effect of CFTR Modulators on Oxidative Stress and Autophagy in Non-CFTR-Expressing Cells Open
The triple combination therapy for cystic fibrosis (CF), including elexacaftor, tezacaftor and ivacaftor (ETI or Trikafta), has been shown to improve lung function and reduce pulmonary exacerbations, thereby enhancing the quality of life f…
View article: Multiple antimicrobial and immune-modulating activities of cysteamine in infectious diseases
Multiple antimicrobial and immune-modulating activities of cysteamine in infectious diseases Open
Infectious diseases are a major threat to global health and cause millions of deaths every year, particularly in developing countries. The emergence of multidrug resistance challenges current antimicrobial treatments, inducing uncertainty …
View article: The immune and microbial homeostasis determines the<i>Candida</i>–mast cells cross-talk in celiac disease
The immune and microbial homeostasis determines the<i>Candida</i>–mast cells cross-talk in celiac disease Open
Celiac disease (CD) is an autoimmune enteropathy resulting from an interaction between diet, genome, and immunity. Although many patients respond to a gluten-free diet, in a substantive number of individuals, the intestinal injury persists…
View article: Identification of an ultra-rare Alu insertion in the CFTR gene: Pitfalls and challenges in genetic test interpretation
Identification of an ultra-rare Alu insertion in the CFTR gene: Pitfalls and challenges in genetic test interpretation Open
Cystic fibrosis (CF) is a life-limiting genetic disorder characterized by defective chloride ion transport due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Early detection through newborn screening p…
View article: Food-dependent exercise-induced allergic reactions in Lipid Transfer Protein (LTP) hypersensitive subjects: new data and a critical reappraisal
Food-dependent exercise-induced allergic reactions in Lipid Transfer Protein (LTP) hypersensitive subjects: new data and a critical reappraisal Open
Background. Lipid transfer protein is the main cause of both primary food allergy and food-dependent exercise-induced allergic reactions (FDEIAR) in Italy. What characterizes LTP-hypersensitive patients with FDEIAR is still unclear.…
View article: Heterogeneity of sensitization profiles and clinical phenotypes among patients with seasonal allergic rhinitis in Southern European countries—The @IT.2020 multicenter study
Heterogeneity of sensitization profiles and clinical phenotypes among patients with seasonal allergic rhinitis in Southern European countries—The @IT.2020 multicenter study Open
Background Pollen allergy poses a significant health and economic burden in Europe. Disease patterns are relatively homogeneous within Central and Northern European countries. However, no study broadly assessed the features of seasonal all…
View article: In silico analysis and theratyping of an ultra-rare CFTR genotype (W57G/A234D) in primary human rectal and nasal epithelial cells
In silico analysis and theratyping of an ultra-rare CFTR genotype (W57G/A234D) in primary human rectal and nasal epithelial cells Open
View article: Correction to: L1077P CFTR pathogenic variant function rescue by Elexacaftor–Tezacaftor–Ivacaftor in cystic fbrosis patient-derived air–liquid interface (ALI) cultures and organoids: in vitro guided personalized therapy of non-F508del patients
Correction to: L1077P CFTR pathogenic variant function rescue by Elexacaftor–Tezacaftor–Ivacaftor in cystic fbrosis patient-derived air–liquid interface (ALI) cultures and organoids: in vitro guided personalized therapy of non-F508del patients Open
View article: Clinical severity of LTP syndrome is associated with an expanded IgE repertoire, FDEIA, FDHIH, and LTP mono reactivity
Clinical severity of LTP syndrome is associated with an expanded IgE repertoire, FDEIA, FDHIH, and LTP mono reactivity Open
Background. Lipid transfer proteins (LTP) allergy is often a challenge for clinicians. We evaluated a multiplex diagnostic approach with diverse cofactors to stratify LTP syndrome risk. Methods. Of the 1,831 participants scre…
View article: L1077P CFTR pathogenic variant function rescue by Elexacaftor–Tezacaftor–Ivacaftor in cystic fibrosis patient-derived air–liquid interface (ALI) cultures and organoids: in vitro guided personalized therapy of non-F508del patients
L1077P CFTR pathogenic variant function rescue by Elexacaftor–Tezacaftor–Ivacaftor in cystic fibrosis patient-derived air–liquid interface (ALI) cultures and organoids: in vitro guided personalized therapy of non-F508del patients Open
View article: Organic Electrochemical Transistor Immuno-Sensors for Spike Protein Early Detection
Organic Electrochemical Transistor Immuno-Sensors for Spike Protein Early Detection Open
The global COVID-19 pandemic has had severe consequences from the social and economic perspectives, compelling the scientific community to focus on the development of effective diagnostics that can combine a fast response and accurate sens…
View article: Theratyping of the Rare CFTR Genotype A559T in Rectal Organoids and Nasal Cells Reveals a Relevant Response to Elexacaftor (VX-445) and Tezacaftor (VX-661) Combination
Theratyping of the Rare CFTR Genotype A559T in Rectal Organoids and Nasal Cells Reveals a Relevant Response to Elexacaftor (VX-445) and Tezacaftor (VX-661) Combination Open
Despite the promising results of new CFTR targeting drugs designed for the recovery of F508del- and class III variants activity, none of them have been approved for individuals with selected rare mutations, because uncharacterized CFTR var…
View article: Factors predicting the outcome of allergen-specific nasal provocation test in children with grass pollen allergic rhinitis
Factors predicting the outcome of allergen-specific nasal provocation test in children with grass pollen allergic rhinitis Open
Background Nasal provocation testing (NPT) is a reference methodology to identify the culprit allergen in patients with allergic rhinitis. Selecting the right allergen for NPT is particularly difficult in poly-sensitized patients with seas…
View article: Immuno-Sensing at Ultra-Low Concentration of TG2 Protein by Organic Electrochemical Transistors
Immuno-Sensing at Ultra-Low Concentration of TG2 Protein by Organic Electrochemical Transistors Open
Transglutaminase 2 (TG2) is a ubiquitously expressed member of the transglutaminase family with Ca2+-dependent protein crosslinking activity. Its subcellular localization is crucial in determining its function, and indeed, TG2 is found in …
View article: Correction to: ‘Collective rotational motion of freely expanding T84 epithelial cell colonies’ (2023) by Ascione <i>et al.</i>
Correction to: ‘Collective rotational motion of freely expanding T84 epithelial cell colonies’ (2023) by Ascione <i>et al.</i> Open
View article: The STING/TBK1/IRF3/IFN type I pathway is defective in cystic fibrosis
The STING/TBK1/IRF3/IFN type I pathway is defective in cystic fibrosis Open
Cystic fibrosis (CF) is a rare autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The most common mutation is F508del-CFTR (ΔF) which leads the encoded ion channel toward…
View article: Collective rotational motion of freely expanding T84 epithelial cell colonies
Collective rotational motion of freely expanding T84 epithelial cell colonies Open
Coordinated rotational motion is an intriguing, yet still elusive mode of collective cell migration, which is relevant in pathological and morphogenetic processes. Most of the studies on this topic have been carried out on epithelial cells…
View article: Immuno-Biosensing Based on Organic Electrochemical Transistors for Anti-Spike Protein Early Detection
Immuno-Biosensing Based on Organic Electrochemical Transistors for Anti-Spike Protein Early Detection Open
View article: Collective rotational motion of freely-expanding T84 epithelial cell colonies
Collective rotational motion of freely-expanding T84 epithelial cell colonies Open
Coordinated rotational motion is an intriguing, yet still elusive mode of collective cell migration, which is relevant in pathological and morphogenetic processes. Most of the studies on this topic have been carried out on confined epithel…
View article: Anakinra restores cellular proteostasis by coupling mitochondrial redox balance to autophagy
Anakinra restores cellular proteostasis by coupling mitochondrial redox balance to autophagy Open
Autophagy selectively degrades aggregation-prone misfolded proteins caused by defective cellular proteostasis. However, the complexity of autophagy may prevent the full appreciation of how its modulation could be used as a therapeutic stra…
View article: Probiotics Supplements Reduce ER Stress and Gut Inflammation Associated with Gliadin Intake in a Mouse Model of Gluten Sensitivity
Probiotics Supplements Reduce ER Stress and Gut Inflammation Associated with Gliadin Intake in a Mouse Model of Gluten Sensitivity Open
Exposure to gluten, a protein present in wheat rye and barley, is the major inducer for human Celiac Disease (CD), a chronic autoimmune enteropathy. CD occurs in about 1% worldwide population, in genetically predisposed individuals bearing…
View article: Impaired cholesterol metabolism in the mouse model of cystic fibrosis. A preliminary study
Impaired cholesterol metabolism in the mouse model of cystic fibrosis. A preliminary study Open
This study aims to investigate cholesterol metabolism in a mouse model with cystic fibrosis (CF) by the comparison of affected homozygous versus wild type (WT) mice. In particular, we evaluated the effects of a diet enriched with cholester…
View article: Probiotics Supplements Reduce ER Stress and Gut Inflammation Associated With Gliadin Intake in Celiac Disease Mouse Model
Probiotics Supplements Reduce ER Stress and Gut Inflammation Associated With Gliadin Intake in Celiac Disease Mouse Model Open
Celiac disease (CD) is a permanent intolerance to dietary protein, gluten, from wheat rye and barley. It occurs in about 1% worldwide population, in genetically predisposed individuals bearing human leukocyte antigen (HLA) DQ2/DQ8. Gut epi…
View article: Impaired cholesterol metabolism in the mice model of cystic fibrosis. A vicious circle?
Impaired cholesterol metabolism in the mice model of cystic fibrosis. A vicious circle? Open
Patients with cystic fibrosis (CF) have low cholesterol absorption and, despite enhanced endogenous biosynthesis, low serum cholesterol. Herein, we investigated cholesterol metabolism in a murine CF model in comparison to wild type (WT) te…
View article: To Be or Not to Be a Pathogen: Candida albicans and Celiac Disease
To Be or Not to Be a Pathogen: Candida albicans and Celiac Disease Open
Celiac disease (CD) is an immune-mediated disorder triggered by the ingestion of gluten and characterized by reversible small-bowel mucosal atrophy in genetically predisposed subjects. Although the prevalence of CD has increased, many aspe…
View article: Succinate links mitochondria to deadly bacteria in cystic fibrosis
Succinate links mitochondria to deadly bacteria in cystic fibrosis Open
Cystic fibrosis (CF) is a life shortening–autosomal recessive disease caused by loss–of–function mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) that transports essential electrolytes in and out of speciali…
View article: Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis <i>versus</i> gluten-mediated inhibition in celiac disease
Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis <i>versus</i> gluten-mediated inhibition in celiac disease Open
Cystic Fibrosis (CF) is the most frequent lethal monogenetic disease affecting humans. CF is characterized by mutations in cystic fibrosis transmembrane conductance regulator (CFTR), a chloride channel whose malfunction triggers the activa…
View article: Cystic Fibrosis: New Insights into Therapeutic Approaches
Cystic Fibrosis: New Insights into Therapeutic Approaches Open
Since the identification of Cystic Fibrosis (CF) as a disease in 1938 until 2012, only therapies to treat symptoms rather than etiological therapies have been used to treat the disease. Over the last few years, new technologies have been d…
View article: Genistein antagonizes gliadin-induced CFTR malfunction in models of celiac disease
Genistein antagonizes gliadin-induced CFTR malfunction in models of celiac disease Open
In celiac disease (CD), an intolerance to dietary gluten/gliadin, antigenic gliadin peptides trigger an HLA-DQ2/DQ8-restricted adaptive Th1 immune response. Epithelial stress, induced by other non-antigenic gliadin peptides, is required fo…