Tumour necrosis factor ?, lipid peroxidation and NO• are increased and associated with decreased free-radical scavenging enzymes in patients with Weill-Marchesani syndrome
Küçük Resim Yok
Tarih
2004
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Hindawi Ltd
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
AIM: 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.
Açıklama
Anahtar Kelimeler
antioxidant enzymes, lipid peroxidation, nitric oxide, tumour necrosis factor alpha, Weill-Marchesani syndrome
Kaynak
Mediators of Inflammation
WoS Q Değeri
Q4
Scopus Q Değeri
Q2
Cilt
13
Sayı
3
