6533b7d7fe1ef96bd1269053

RESEARCH PRODUCT

LSC Abstract – Increased oxidative stress leads to telomere shortening in children with alpha-1 antitrypsin deficiency

Pilar Codoñer-franchMaría Mercedes Navarro-garcíaSara PastorSilvia CastilloEstrella Fernández-fabrellasFrancisco Jose Dasi FernandezAmparo EscribanoFrancisco SanzAna Reula

subject

medicine.medical_specialtyAlpha 1-antitrypsin deficiencyLungbusiness.industryGlutathionemedicine.diseasemedicine.disease_causeGastroenterologyPathophysiologyTelomerechemistry.chemical_compoundLiver diseasemedicine.anatomical_structurechemistryLung diseaseInternal medicineImmunologymedicinebusinessOxidative stress

description

Background: Oxidative stress (OS) is involved in the pathophysiology of AATD (Escribano A. et al. Thorax 2015; 70:82-3). In addition, it has been shown that OS accelerates telomere shortening which is associated to higher emphysema risk in COPD patients. Rationale and aims: Since AATD is characterised by chronic OS, we hypothesise that telomere shortening would be accelerated in AATD patients and would be associated with higher risk of developing lung disease. This study is aimed to assess the OS profile, the enzymatic antioxidant defence mechanisms and telomere length (TL) in children with AATD and to study its association with AAT phenotypes. Methods: OS parameters, the activity of the main antioxidant enzymes and TL were prospectively measured in fifty-one children diagnosed with alpha-1 antitrypsin deficiency and thirty-eight control individuals. Results: AATD patients showed higher GSSG/GSH ratio (p=0.001; p Conclusions: Increased OS lead to accelerated telomere shortening in AATD patients. An association between OS, TL and risk of developing lung/liver disease is observed. Funding: SEPAR 2013 and ISCIII (PI14/02162) grants.

yearjournalcountryeditionlanguage
2016-09-01ERS Lung Science Conference 2016
https://doi.org/10.1183/13993003.congress-2016.pp115