Glutathione levels in blood from ataxia telangiectasia patients suggest in vivo adaptive mechanisms to oxidative stress

Paolo Degan, Marco d'Ischia, Federico V. Pallardó, Adriana Zatterale, Alfredo Brusco, Rita Calzone, Simona Cavalieri, Kaan Kavakli, Ana Lloret, Paola Manini, Maria Antonietta Pisanti, Emilia Vuttariello, Giovanni Pagano

Research output: Contribution to journalArticle

12 Citations (Scopus)

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). Conclusion: 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.

Original languageEnglish
Pages (from-to)666-670
Number of pages5
JournalClinical Biochemistry
Volume40
Issue number9-10
DOIs
Publication statusPublished - Jun 2007

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Ataxia Telangiectasia
Oxidative stress
Glutathione Disulfide
Pyruvaldehyde
Glutathione
Oxidative Stress
Blood
Glyoxal
Reactive Oxygen Species
Plasmas
Biomarkers
Heterozygote
Parents
Homozygote
Leukocytes

Keywords

  • 8-Hydroxy-2′-deoxyguanosine
  • Ataxia telangiectasia
  • Glutathione
  • Glyoxal
  • Methylglyoxal
  • Oxidative stress

ASJC Scopus subject areas

  • Clinical Biochemistry

Cite this

Glutathione levels in blood from ataxia telangiectasia patients suggest in vivo adaptive mechanisms to oxidative stress. / Degan, Paolo; d'Ischia, Marco; Pallardó, Federico V.; Zatterale, Adriana; Brusco, Alfredo; Calzone, Rita; Cavalieri, Simona; Kavakli, Kaan; Lloret, Ana; Manini, Paola; Pisanti, Maria Antonietta; Vuttariello, Emilia; Pagano, Giovanni.

In: Clinical Biochemistry, Vol. 40, No. 9-10, 06.2007, p. 666-670.

Research output: Contribution to journalArticle

Degan, P, d'Ischia, M, Pallardó, FV, Zatterale, A, Brusco, A, Calzone, R, Cavalieri, S, Kavakli, K, Lloret, A, Manini, P, Pisanti, MA, Vuttariello, E & Pagano, G 2007, 'Glutathione levels in blood from ataxia telangiectasia patients suggest in vivo adaptive mechanisms to oxidative stress', Clinical Biochemistry, vol. 40, no. 9-10, pp. 666-670. https://doi.org/10.1016/j.clinbiochem.2007.03.013
Degan, Paolo ; d'Ischia, Marco ; Pallardó, Federico V. ; Zatterale, Adriana ; Brusco, Alfredo ; Calzone, Rita ; Cavalieri, Simona ; Kavakli, Kaan ; Lloret, Ana ; Manini, Paola ; Pisanti, Maria Antonietta ; Vuttariello, Emilia ; Pagano, Giovanni. / Glutathione levels in blood from ataxia telangiectasia patients suggest in vivo adaptive mechanisms to oxidative stress. In: Clinical Biochemistry. 2007 ; Vol. 40, No. 9-10. pp. 666-670.
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AU - d'Ischia, Marco

AU - Pallardó, Federico V.

AU - Zatterale, Adriana

AU - Brusco, Alfredo

AU - Calzone, Rita

AU - Cavalieri, Simona

AU - Kavakli, Kaan

AU - Lloret, Ana

AU - Manini, Paola

AU - Pisanti, Maria Antonietta

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AB - 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). Conclusion: 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.

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