High Frequencies of Regulatory T Cells Are Associated with Less Chronic Lung Allograft Dysfunction in Cystic Fibrosis Patients After Combined Lung and Liver Transplantation

In this study we investigated regulatory t cells after combined lung and liver transplantation, or after isolated lung transplantation, in cystic fibrosis patients and correlated the findings with long term outcomes
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research

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Many disease-causing mutations impair protein stability. Here, we explore a thermodynamic strategy to correct the disease-causing F508del mutation in the human cystic fibrosis transmembrane conductance regulator (hCFTR). F508del destabilizes nucleotide-binding domain 1 (hNBD1) in hCFTR relative to an aggregation-prone intermediate. We developed a fluorescence self-quenching assay for compounds that prevent aggregation of hNBD1 by stabilizing its native conformation. Unexpectedly, we found that dTTP and nucleotide analogs with exocyclic methyl groups bind to hNBD1 more strongly than ATP and preserve electrophysiological fun...
Source: Journal of Biological Chemistry - Category: Chemistry Authors: Tags: Molecular Biophysics Source Type: research
Publication date: Available online 5 October 2018Source: The Journal of Molecular DiagnosticsAuthor(s): Jenny L. Kerschner, Sujana Ghosh, Alekh Paranjapye, Wilmel R. Cosme, Marie-Pierre Audrézet, Miyuki Nakakuki, Hiroshi Ishiguro, Claude Férec, Johanna Rommens, Ann HarrisIt is estimated that up to 5% of cystic fibrosis transmembrane conductance regulator (CFTR) pathogenic alleles are unidentified. Some of these errors may lie in noncoding regions of the locus and affect gene expression. To identify regulatory element variants in the CFTR locus, SureSelect-targeted enrichment of 460 kb encompassing the gene wa...
Source: The Journal of Molecular Diagnostics - Category: Pathology Source Type: research
Cystic fibrosis (CF) ‐associated liver disease (CFLD) is a hepatobiliary complication of CF. Current diagnostic modalities rely on nonspecific assessments, whereas liver biopsy is the gold standard to assess severity of fibrosis. MicroRNAs (miRNAs) regulate liver disease pathogenesis and are proposed as diagnostic bio markers. We investigated the combined use of serum miRNAs and aspartate aminotransferase (AST) to platelet ratio (APRI) to diagnose and assess CFLD severity. This was a cross‐sectional cohort study of the circulatory miRNA signature of 124 children grouped by clinical, biochemical, and imaging ass essment...
Source: Hepatology - Category: Internal Medicine Authors: Tags: Original Article Source Type: research
Conclusions and clinical relevance: Proteomic analysis of the CF airway demonstrates a complex environment of proteins and pathways. This work provides evidence that aptamer ‐based proteomics can differentiate between groups and can determine endotypes within CF.This article is protected by copyright. All rights reserved
Source: Proteomics. Clinical Applications - Category: Biochemistry Authors: Tags: Research Article Source Type: research
Annals of the American Thoracic Society,Volume 15, Issue Supplement_3, Page S171-S176, November 2018.
Source: Annals of the American Thoracic Society - Category: Respiratory Medicine Authors: Source Type: research
Sarah Burgwin, of Totnes, Devon, said it was 'shameful' that six-year-old Katie Stafford was being denied access to Orkambi, which can improve lung function in sufferers of the disease.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
ConclusionsOlder age, lower FEV1 % of predicted, and lower MEP were independently linked to unfavorable outcomes. FACED and BSI were not accurate in predicting mortality in our cohort.
Source: Lung - Category: Respiratory Medicine Source Type: research
Source: Respiration - Category: Respiratory Medicine Source Type: research
In this study seven clinical M. abscessus complex strains and the M. abscessus reference strain ATCC19977 were used to find species-specific proteins for their use in immune assays. Six strains showed rough and smooth colony morphotypes simultaneously, two strains only showed rough mophotypes, resulting in 14 separate isolates. Clinical isolates were submitted to whole genome sequencing. Proteomic analysis was performed on bacterial lysates and culture supernatant of all 14 isolates. Species-specificity for M. abscessus complex was determined by a BLAST search for proteins present in all supernatants. Species-specific prot...
Source: Microbes and Infection - Category: Infectious Diseases Source Type: research
Sarah Burgwin wants the NHS to pay for Orkambi to treat her six-year-old daughter's cystic fibrosis.
Source: BBC News | Health | UK Edition - Category: Consumer Health News Source Type: news
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