title: Neutrophil glutamine deficiency in relation to genotype in children with cystic fibrosis.
abstract: Pulmonary disease in cystic fibrosis (CF) is characterized by a chronic neutrophil-dominated inflammation of lung tissue. Inasmuch as some amino acids (AA) play a pivotal role in various aspects of neutrophil metabolism, the aim of this study was to investigate a possible alteration of neutrophil AA metabolism and to evaluate its relation (if any) with the genotype. We performed plasma and neutrophil AA analysis in 26 CF patients with known genotype, 10 patients with non-CF bronchiectasis, and 20 normal subjects. The CF group showed a significant decrease of free intracellular neutrophil glutamine (Gln) content compared with controls and the non-CF bronchiectasis group. In the latter group, levels of neutrophil Gln were significantly lower compared with the controls. Amino acid plasma concentration in non-CF bronchiectasis showed a decrease of Gln and taurine compared with controls. Neutrophil Gln content showed values significantly lower in CF patients with severe mutations (class I, II, and III mutations) compared with mild mutations (class IV and V mutations). Results of our study add further evidence for intrinsic neutrophil alterations that could play an important role in the pathogenesis of chronic pulmonary disease in CF patients.
authors.affiliation: Department of Pediatrics, University of Rome La Sapienza, Italy. patdeufemia@inwind.it
authors.forename: Patrizia
authors.initials: P
authors.lastname: D'Eufemia
authors.forename: Roberto
authors.initials: R
authors.lastname: Finocchiaro
authors.forename: Mauro
authors.initials: M
authors.lastname: Celli
authors.forename: Julia
authors.initials: J
authors.lastname: Tote
authors.forename: Valentina
authors.initials: V
authors.lastname: Ferrucci
authors.forename: Anna
authors.initials: A
authors.lastname: Zambrano
authors.forename: Patrizia
authors.initials: P
authors.lastname: Troiani
authors.forename: Serena
authors.initials: S
authors.lastname: Quattrucci
mesh_terms: D000293:Adolescent; D000596:Amino Acids / Q000097:blood / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000235:genetics* / Q000276:immunology / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism; D005260:Female; D005838:Genotype; D005973:Glutamine / Q000097:blood / Q000172:deficiency*; D006801:Humans; D008297:Male; D009154:Mutation; D009504:Neutrophils / Q000378:metabolism*
chemical_list: D000596:Amino Acids; C505032:CFTR protein, human; D005973:Glutamine; D019005:Cystic Fibrosis Transmembrane Conductance Regulator