title: Increased circulating levels of plasma ATP in cystic fibrosis patients.
abstract: Recent studies have shown that the cystic fibrosis transmembrane conductance regulator (CFTR), an ATP-binding cassette (ABC) transporter whose mutations are responsible for cystic fibrosis (CF), permeates ATP. However, little information is available concerning extracellular ATP concentrations in CF patients. Thus, the goal of this preliminary study was to determine the circulating levels of plasma ATP in CF patients. Circulating levels of plasma ATP were determined by the luciferin-luciferase assay in both CF patients and healthy volunteer control subjects. The two groups were compared using an analysis of variance. CF genotype and age, which ranged from 7 to 56 years, were also used to compare data by single-blind analysis. With comparable sample numbers, CF patients had statistically higher levels of circulating ATP (34%, P<0.01) when compared by analysis of covariance with the age of the subjects as the cofactor. The CF patients bearing the DeltaF508 genotype had a 54% (n=33, P<0.01) higher plasma ATP concentration compared to controls, while patients bearing other CF genotypes were similar to controls (n=10, P<0.4). We conclude that CF patients have higher circulating levels of ATP when compared to controls. Increased levels of plasma ATP, which is an important autocrine/paracrine hormone in many cell types, may be associated with chronic manifestations of the disease.
authors.affiliation: Renal Unit, Massachusetts General Hospital East, Charlestown, MA 02129, USA.
authors.forename: A S
authors.initials: AS
authors.lastname: Lader
authors.forename: A G
authors.initials: AG
authors.lastname: Prat
authors.forename: G R
authors.initials: GR
authors.lastname: Jackson
authors.forename: K L
authors.initials: KL
authors.lastname: Chervinsky
authors.forename: A
authors.initials: A
authors.lastname: Lapey
authors.forename: T B
authors.initials: TB
authors.lastname: Kinane
authors.forename: H F
authors.initials: HF
authors.lastname: Cantiello
mesh_terms: D000255:Adenosine Triphosphate / Q000097:blood*; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000704:Analysis of Variance; D002648:Child; D003550:Cystic Fibrosis / Q000097:blood* / Q000235:genetics; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005838:Genotype; D006720:Homozygote; D006801:Humans; D008875:Middle Aged; D009154:Mutation; D015203:Reproducibility of Results
chemical_list: C505032:CFTR protein, human; C093349:cystic fibrosis transmembrane conductance regulator delta F508; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D000255:Adenosine Triphosphate