Tumour necrosis factor ?, lipid peroxidation and NO• are increased and associated with decreased free-radical scavenging enzymes in patients with Weill-Marchesani syndrome

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dc.authoridbay karabulut, aysun/0000-0002-7873-2805
dc.authoridTürköz, Yusuf/0000-0001-5401-0720
dc.authoridCalis, Mustafa/0000-0002-1184-0772
dc.authorwosidEvereklioğlu, Cem/A-5370-2018
dc.authorwosidbay karabulut, aysun/HJP-0995-2023
dc.authorwosidTürköz, Yusuf/ABG-7931-2020
dc.contributor.authorEvereklioglu, C
dc.contributor.authorTurkoz, Y
dc.contributor.authorCalis, M
dc.contributor.authorDuygulu, F
dc.contributor.authorKarabulut, AB
dc.date.accessioned2024-08-04T20:30:44Z
dc.date.available2024-08-04T20:30:44Z
dc.date.issued2004
dc.departmentİnönü Üniversitesien_US
dc.description.abstractAIM: Weill-Marchesani syndrome (WMS) is a rare systemic disorder with both autosomal recessive and dominant inheritances. Accumulation of reactive oxygen species such as O-2(.-), H2O2 and OH. causes lipid peroxidation (LPO), whereas antioxidant enzymes (superoxide dismutase (SOD), glutathione peroxidase (GSHPx)) mediate defence against oxidative stress. Excess tumour necrosis factor (TNF)-alpha and NO. react with O-2(.-) and cause further antioxidant depletion with an increase in mutation frequency by H2O2. This study investigated the levels of SOD, GSHPx, catalase (CAT), TNF-alpha, NO. and LPO in patients with WMS. Methods: A group of 10 WMS patients (four males, six females; age, 26.5 +/- 19.0 years) and 10 age-matched and sex-matched controls (five males, five females; age, 27.3 +/- 18.2 years) were included. Serum TNF-alpha levels were determined by a spectrophotometer technique using immulite chemiluminescent immunometric assay. Malondialdehyde (MDA) was determined in plasma; CAT in red blood cells (RBCs), and SOD and GSHPx in both plasma and RBCs. Total serum NO. levels were evaluated by Griess reaction. Results: Mean levels of TNF-alpha (8.3 +/- 0.6 pg/ml) in WMS patients were significantly (p < 0.001) higher than controls (4.3 +/- 0.2 pg/ml). Plasma MDA levels in patients and controls were 5.4 +/- 0.8 and 1.8 +/- 0.6 mu mol/l, respectively, and the difference was significant (p = 0.0002). SOD and GSHPx activities were significantly lower in both RBCs and plasma of WMS than in controls (RBC-SOD, 3981.9 +/- 626.6 versus 5261.6 +/- 523.0 U/g haemoglobin (Hb), p = 0.0005; plasma-SOD, 529.4 +/- 49.3 versus 713.4 +/- 55.7 U/g protein, p = 0.0002; RBC-GSHPx, 682.7 +/- 42.0 versus 756.5 +/- 47.6 U/g Hb, p = 0.0011; plasma-GSHPx, 107.3 +/- 15.0 versus 131.4 +/- 19.7 U/g protein, p = 0.0113). In addition, serum NO center dot (NO2- + NO3-) levels were also significantly (p = 0.0002) increased in WMS patients (54.4 +/- 5.7 versus 26.9 +/- 6.7 mumol/l). RBC-CAT levels were similar between groups (125.6 +/- 21.3 versus 131.0 +/- 21.5 k/g Hb, p = 0.8798). Conclusions: The elevated LPO, TNF-alpha and NO. with decreased antioxidant enzyme activities indicated impaired antioxidative defence mechanisms with an oxidative injury and cell toxicity in WMS patients. The use of multiple antioxidants and free radical scavengers might be helpful in this genetic disorder.en_US
dc.identifier.doi10.1080/09511920410001713547
dc.identifier.endpage170en_US
dc.identifier.issn0962-9351
dc.identifier.issn1466-1861
dc.identifier.issue3en_US
dc.identifier.pmid15223607en_US
dc.identifier.scopus2-s2.0-4143060410en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage165en_US
dc.identifier.urihttps://doi.org/10.1080/09511920410001713547
dc.identifier.urihttps://hdl.handle.net/11616/94492
dc.identifier.volume13en_US
dc.identifier.wosWOS:000222224300005en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherHindawi Ltden_US
dc.relation.ispartofMediators of Inflammationen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectantioxidant enzymesen_US
dc.subjectlipid peroxidationen_US
dc.subjectnitric oxideen_US
dc.subjecttumour necrosis factor alphaen_US
dc.subjectWeill-Marchesani syndromeen_US
dc.titleTumour necrosis factor ?, lipid peroxidation and NO• are increased and associated with decreased free-radical scavenging enzymes in patients with Weill-Marchesani syndromeen_US
dc.typeArticleen_US

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