Ivacaftor attenuates gentamicin-induced ototoxicity through the CFTR-Nrf2-HO1/NQO1 pathway
The objective of this study was to estimate the effect of the CFTR activator ivacaftor on gentamicin-induced ototoxicity and determine its mechanism.METHODS: The hair cell count was analyzed by Myosin 7a staining. Apoptosis was analyzed by TUNEL Apoptosis Kit. Cellular reactive oxygen species (ROS) level was detected by DCFH-DA probes. The Nrf2 related proteins expression levels were analyzed by western blot.RESULTS: An in vitro cochlear explant model showed that gentamicin caused ROS accumulation in sensory hair cells and induced apoptosis, and this effect was alleviated by pretreatment with ivacaftor. Western blotting sh...
Source: Redox Report : communications in free radical research - April 2, 2024 Category: Biochemistry Authors: Rui Hu Fan Wu Yi-Qing Zheng Source Type: research

Non-coding regulatory sRNAs from bacteria of the Burkholderia cepacia complex
Appl Microbiol Biotechnol. 2024 Apr 2;108(1):280. doi: 10.1007/s00253-024-13121-6.ABSTRACTSmall non-coding RNAs (sRNAs) are key regulators of post-transcriptional gene expression in bacteria. Hundreds of sRNAs have been found using in silico genome analysis and experimentally based approaches in bacteria of the Burkholderia cepacia complex (Bcc). However, and despite the hundreds of sRNAs identified so far, the number of functionally characterized sRNAs from these bacteria remains very limited. In this mini-review, we describe the general characteristics of sRNAs and the main mechanisms involved in their action as regulato...
Source: Applied Microbiology and Biotechnology - April 2, 2024 Category: Microbiology Authors: Gon çalo R Matos Joana R Feliciano Jorge H Leit ão Source Type: research

Ivacaftor attenuates gentamicin-induced ototoxicity through the CFTR-Nrf2-HO1/NQO1 pathway
The objective of this study was to estimate the effect of the CFTR activator ivacaftor on gentamicin-induced ototoxicity and determine its mechanism.METHODS: The hair cell count was analyzed by Myosin 7a staining. Apoptosis was analyzed by TUNEL Apoptosis Kit. Cellular reactive oxygen species (ROS) level was detected by DCFH-DA probes. The Nrf2 related proteins expression levels were analyzed by western blot.RESULTS: An in vitro cochlear explant model showed that gentamicin caused ROS accumulation in sensory hair cells and induced apoptosis, and this effect was alleviated by pretreatment with ivacaftor. Western blotting sh...
Source: Redox Report : communications in free radical research - April 2, 2024 Category: Biochemistry Authors: Rui Hu Fan Wu Yi-Qing Zheng Source Type: research

STUB1 exacerbates calcium oxalate-induced kidney injury by modulating reactive oxygen species-mediated cellular autophagy via regulating CFTR ubiquitination
This study investigated the role of STIP1 homology and U-box protein 1 (STUB1), an E3 ubiquitin ligase, and cystic fibrosis transmembrane conductance regulator (CFTR), a chloride channel, in CaOx-related renal damage and autophagy regulation.  HK-2 cells were treated with various doses of CaOx monohydrate (COM) to simulate kidney injury in vitro. Cell viability, reactive oxygen species (ROS) production, and apoptosis were assessed. The regulation of CFTR ubiquitination by STUB1 was confirmed by immunoprecipitation. An in vivo model was established by injecting mice with glyoxylate. COM treatment dose-dependently decrease...
Source: Urolithiasis - April 2, 2024 Category: Urology & Nephrology Source Type: research

Glycemic Variability and the Thickness of Retinal Layers in Cystic Fibrosis Patients with and without Cystic Fibrosis Related Diabetes
CONCLUSION: Although we investigated a rather small number of patients, we obtained evidence that intraretinal neurodegenerative changes occur in each of our subgroups (CFNG, CFAG, CFRD). Beyond this, our results favor the detrimental role of additional diabetes, as the deviations from healthy controls were most pronounced in the CFRD group and are similar to those seen in patients suffering from type 1 or type 2 diabetes.PMID:38557392 | DOI:10.1080/02713683.2024.2333770 (Source: Cell Research)
Source: Cell Research - April 1, 2024 Category: Cytology Authors: Luisa S Saegebrecht Martin R öhlig Friederike Schaub Manfred Ballmann Oliver Stachs Dagmar-C Fischer Source Type: research

Glycemic Variability and the Thickness of Retinal Layers in Cystic Fibrosis Patients with and without Cystic Fibrosis Related Diabetes
CONCLUSION: Although we investigated a rather small number of patients, we obtained evidence that intraretinal neurodegenerative changes occur in each of our subgroups (CFNG, CFAG, CFRD). Beyond this, our results favor the detrimental role of additional diabetes, as the deviations from healthy controls were most pronounced in the CFRD group and are similar to those seen in patients suffering from type 1 or type 2 diabetes.PMID:38557392 | DOI:10.1080/02713683.2024.2333770 (Source: Current Eye Research)
Source: Current Eye Research - April 1, 2024 Category: Opthalmology Authors: Luisa S Saegebrecht Martin R öhlig Friederike Schaub Manfred Ballmann Oliver Stachs Dagmar-C Fischer Source Type: research

Glycemic Variability and the Thickness of Retinal Layers in Cystic Fibrosis Patients with and without Cystic Fibrosis Related Diabetes
CONCLUSION: Although we investigated a rather small number of patients, we obtained evidence that intraretinal neurodegenerative changes occur in each of our subgroups (CFNG, CFAG, CFRD). Beyond this, our results favor the detrimental role of additional diabetes, as the deviations from healthy controls were most pronounced in the CFRD group and are similar to those seen in patients suffering from type 1 or type 2 diabetes.PMID:38557392 | DOI:10.1080/02713683.2024.2333770 (Source: Current Eye Research)
Source: Current Eye Research - April 1, 2024 Category: Opthalmology Authors: Luisa S Saegebrecht Martin R öhlig Friederike Schaub Manfred Ballmann Oliver Stachs Dagmar-C Fischer Source Type: research

Prevalence and Clinical Impact of Respiratory Viral Infections from the STOP2 Study of Cystic Fibrosis Pulmonary Exacerbations
Annals of the American Thoracic Society,Volume 21, Issue 4, Page 595-603, April 2024. (Source: Annals of the American Thoracic Society)
Source: Annals of the American Thoracic Society - April 1, 2024 Category: Respiratory Medicine Authors: Christina S. Thornton Lindsay J. Caverly Linda M. Kalikin Lisa A. Carmody Scott McClellan William LeBar Don B. Sanders Natalie E. West Christopher H. Goss Patrick A. Flume Sonya L. Heltshe Donald R. VanDevanter John J. LiPuma Source Type: research

Nonantibiotic Management of Nontuberculous Mycobacteria in Non –Cystic Fibrosis Bronchiectasis: Natural or Nonsense?
Annals of the American Thoracic Society,Volume 21, Issue 4, Page 543-545, April 2024. (Source: Annals of the American Thoracic Society)
Source: Annals of the American Thoracic Society - April 1, 2024 Category: Respiratory Medicine Authors: Katherine B. Hisert Jerry A. Nick Source Type: research

Association of Neutrophil –Lymphocyte Ratio with Clinical Outcomes in Patients with Cystic Fibrosis
(Source: Indian Journal of Pediatrics)
Source: Indian Journal of Pediatrics - April 1, 2024 Category: Pediatrics Source Type: research

Outcomes of Combined Liver-Lung Transplant in Pediatric Patients with Cystic Fibrosis: An ISHLT Study
This study aims to analyze pre-transplant predictors and post-transplant outcomes of ConLi-LTx and lung transplant (LTx) only recipients. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , B. Langlais, B. Aqel, J. D'Cunha, P. Reck dos Santos Source Type: research

Assessing the Relationship Between Pre-Operative Opioid Use and Outcomes of Patients with Cystic Fibrosis Undergoing Lung Transplantation
Purpose: Pain syndromes are well characterized in patients with cystic fibrosis (CF) with up to 53% requiring opioid use. Recent evidence suggests that preoperative opioid use is associated with higher complication rates and worse survival outcomes for patients undergoing lung transplantation (LT) however this has not been well characterized among patients with CF undergoing LT who might require longer and higher opioid dosing compared to patients with other indications for LT. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , J.M. Ruck, J.S. Ha, E. Bush, C. Merlo Source Type: research

Successful NTM Eradication and Lung Transplantation in Pediatric CF Patients
We present two pediatric cystic fibrosis (CF) patients with MABSC lung disease who underwent successful lung transplantation without NTM recurrence. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , C. Burton, O.G. Vanderkooi, M. McKinney, D. Lien, N. Avdimiretz Source Type: research

Paediatric Lung Transplantation for Childhood Interstitial Lung Disease Shows Favorable Outcome Compared with LuTx for Cystic Fibrosis or Pulmonary Hypertension
Purpose: Childhood interstitial lung disease (ChILD) is an umbrella term for more than 200 different rare diseases with a significant morbidity and mortality. Lung transplantation (LuTx) is an established treatment option for ChILD in patients with endstage lung disease. There is limited data on the indications and outcomes following LuTx in ChILD. The aim of this study is to evaluate the long term outcome in ChILD-patients following LuTx and its possible interrelation with the type and severity of the underlying disease. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , F. L änger, F. Ius, C. Müller, K. Schütz, D. Bobylev, J. Salman, N. Schwerk, J. Carlens Source Type: research