ABSTRACT
BACKGROUND: Familial Mediterranean fever (FMF) is a genetically linked disorder common amongst races of the Eastern Mediterranean region. Typical symptoms include episodic pain syndrome extending throughout the chest or abdomen associated with histopathological signs of amyloidosis of the kidney. AIM: To investigate possible connections between the aseptic inflammation that occurs during pain crises and cell membrane structural and functional integrity in patients with FMF. METHODS: Oxidative stress parameters in 42 patients in remission and during a pain crisis were compared with 21 normal subjects. RESULTS: The patient group had significantly greater concentrations of chemiluminescent and thiobarbituric acid-reactive substances in the blood plasma and lower concentrations of alpha-tocopherol than the control group while in remission; these changes were exacerbated during pain crises. Analyses of the phospholipid composition of erythrocyte membranes showed significant increases in amounts of acidic phospholipids (phosphatidic acid, monophosphatidylinositol and cardiolipin) and lysophosphatidylcholine compared with healthy subjects. CONCLUSIONS: The pattern of differences in membrane phospholipid composition was consistent with increased oxidative stress in patients with FMF.
Subject(s)
Erythrocytes/metabolism , Familial Mediterranean Fever/etiology , Oxidative Stress , Phospholipids/metabolism , Erythrocyte Membrane/chemistry , Erythrocyte Membrane/metabolism , Familial Mediterranean Fever/metabolism , Humans , Phospholipids/analysisABSTRACT
Rat brain slices have been incubated in the presence of water-soluble synthetic peptide fragments corresponding to residues 1-9 and 1-15 of the N-terminus of immunophilin and the effects on the phospholipid composition examined. During a 2 h incubation in the presence of 1 nM, 0-1 mu M and 10 mu M concentrations of the peptides there were significant and dose-dependent decreases in the amounts of phosphatidylcholine and phosphatidylethanolamine and increases in the amounts of phosphatidylserine and, to a lesser extent, phosphatidylinositol, cardiolipin and lysophosphatidylcholine. The overall decrease in the neutral phospholipids and increase in the acidic phospholipids tended to counteract any change in the phospholipid composition of the tissue. The results are discussed in terms of the possible effects of immunophilin on modulating phospholipid turnover in brain cell membranes.
