title: Glutathione levels in blood from ataxia telangiectasia patients suggest in vivo adaptive mechanisms to oxidative stress.
abstract: OBJECTIVE
  To evaluate an in vivo pro-oxidant state in patients with ataxia telangiectasia (AT).
  
  
  METHODS
  A set of oxidative stress endpoints were measured in 9 AT homozygotes, 16 AT heterozygotes (parents) and 83 controls (grouped in age ranges as for patients and parents, respectively). The following analytes were measured: (a) leukocyte 8-hydroxy-2'-deoxyguanosine (8-OHdG); (b) blood glutathione (GSSG and GSH); and (c) plasma levels of glyoxal (Glx) and methylglyoxal (MGlx).
  
  
  RESULTS
  AT patients displayed a significant decrease in blood GSSG (p=0.012) and in MGlx plasma concentrations (p=0.012). A non-significant decrease in the GSSG:GSH ratio (p=0.1) and a non-significant increase in 8-OHdG and Glx levels were observed in AT patients vs. young controls (age range 4-35 years). AT heterozygotes failed to display any significant changes vs. adult controls (age range 36-68 years).
  
  
  CONCLUSIONS
  No significant increase in oxidative stress biomarkers was detected in blood from AT patients. The decrease in GSSG and MGlx levels in AT patients may suggest an adaptive response to a pro-oxidant state in AT-related target organs.
authors.affiliation: Italian National Cancer Institute, IST; I-16132 Genoa, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Degan
authors.forename: Marco
authors.initials: M
authors.lastname: d'Ischia
authors.forename: Federico V
authors.initials: FV
authors.lastname: Pallardó
authors.forename: Adriana
authors.initials: A
authors.lastname: Zatterale
authors.forename: Alfredo
authors.initials: A
authors.lastname: Brusco
authors.forename: Rita
authors.initials: R
authors.lastname: Calzone
authors.forename: Simona
authors.initials: S
authors.lastname: Cavalieri
authors.forename: Kaan
authors.initials: K
authors.lastname: Kavakli
authors.forename: Ana
authors.initials: A
authors.lastname: Lloret
authors.forename: Paola
authors.initials: P
authors.lastname: Manini
authors.forename: Maria Antonietta
authors.initials: MA
authors.lastname: Pisanti
authors.forename: Emilia
authors.initials: E
authors.lastname: Vuttariello
authors.forename: Giovanni
authors.initials: G
authors.lastname: Pagano
mesh_terms: D000080242:8-Hydroxy-2'-Deoxyguanosine; D000222:Adaptation, Physiological; D000293:Adolescent; D000328:Adult; D001260:Ataxia Telangiectasia / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D004249:DNA Damage; D003849:Deoxyguanosine / Q000031:analogs & derivatives / Q000097:blood; D005260:Female; D005978:Glutathione / Q000097:blood*; D006037:Glyoxal / Q000097:blood; D006801:Humans; D008297:Male; D018384:Oxidative Stress / Q000502:physiology*; D011765:Pyruvaldehyde / Q000097:blood
chemical_list: D006037:Glyoxal; D011765:Pyruvaldehyde; D000080242:8-Hydroxy-2'-Deoxyguanosine; D003849:Deoxyguanosine; D005978:Glutathione