The presence of a lupus anticoagulant was evaluated in patients with Bechet's disease by the kaolin clotting time method. Four percents (three patients) of 69 patients analyzed were found positive for the lupus anticoagulant. However, no statistically significant association existed between the presence of this antibody and the presence of thrombosis, clinical activity, clinical type, antinuclear antibodies and the positive VDRL test.
Behcet Syndrome/*immunology ; Cardiolipins/immunology ; Female ; Human ; Lupus Coagulation Inhibitor/*analysis ; Male

BACKGROUND: Anti-cardiolipin antibody (Anti-CL Ab) is one of the various anti-phospholipid antibodies (Anti-PL Abs) and found in the plasma of patients with systemic lupus erythematosus (SLE), atherosclerosis, and other infectious diseases. While anti-PL Abs found in the sera of patients with infectious diseases bind directly to CL, binding of anti-PL Abs to CL circulating in the sera of patients with autoimmune diseases is mediated by beta2-glycoprotein 1 (beta2-GP1). The purpose of this study is to investigate the effect of beta2-GP1 on the antigen binding assay of anti-CL Abs present in the sera of patients with atherosclerosis, which has been known as one of autoimmune diseases. METHODS: ELISA was performed with sera containing anti-CL Abs from three patients with atherosclerosis in the presence or absence of beta2-GP1 or FBS. RESULTS: Reactivity of anti-CL Abs to CL was increased in the presence of beta2-GP1 or FBS in a dose dependent manner. CONCLUSION: beta2-GP1 or FBS could be used as co-factor in CL ELISA with anti-CL Abs present in the sera of patients with atherosclerosis. It is suggested that anti-CL Abs found in atherosclerosis patients are similar in terms of antigen binding property to those circulating in the patients with autoimmune diseases, not to infectious diseases.
Antibodies* ; Atherosclerosis ; Autoimmune Diseases ; Cardiolipins* ; Communicable Diseases ; Enzyme-Linked Immunosorbent Assay ; Humans ; Lupus Erythematosus, Systemic ; Plasma

Barth syndrome (BTHS) is a rare genetic disorder characterized by various types of cardiomyopathy, neutropenia, failure to thrive, skeletal myopathy, and 3-methylglutaconic aciduria. BTHS is caused by loss-of-function mutations in the tafazzin (TAZ) gene located on chromosome Xq28, leading to cardiolipin deficiency. We report a 13-month-old boy with BTHS who had a novel de novo mutation in the TAZ gene. To the best of our knowledge, this is the first reported case of a BTHS patient with a de novo mutation in Korea. This report will contribute towards expanding the knowledge on the mutation spectrum of the TAZ gene in BTHS.
Barth Syndrome* ; Cardiolipins ; Cardiomyopathies ; Failure to Thrive ; Humans ; Infant ; Korea ; Male ; Muscular Diseases ; Neutropenia

Antibodies to cardiolipin and other phospholipid have been associated with recurrent thrombotic events, including ischemic strokes, especially in children and young adults. Recently it has been shown that anti-beta2-glycoprotein I antibodies may be more specific in predicting thrombosis. We report a case of anterior spinal artery syndrome with elevated titer of antibodies to beta2-glycoprotein I in young adult.
Anterior Spinal Artery Syndrome ; Antibodies ; Antiphospholipid Syndrome ; beta 2-Glycoprotein I ; Cardiolipins ; Child ; Humans ; Stroke ; Thrombosis ; Young Adult

The mitochondrial respiratory chain consists of 5 enzyme complexes that are responsible for ATP generation. The paradigm of the electron transport chain as discrete enzymes diffused in the inner mitochondrial membrane has been replaced by the solid state supercomplex model wherein the respiratory complexes associate with each other to form supramolecular complexes. Defects in these supercomplexes, which have been shown to be functionally active and required for forming stable respiratory complexes, have been associated with many genetic and neurodegenerative disorders demonstrating their biomedical significance. In this review, we will summarize the functional and structural significance of supercomplexes and provide a comprehensive review of their assembly and the assembly factors currently known to play a role in this process.
Adenosine Triphosphate ; metabolism ; Arylamine N-Acetyltransferase ; metabolism ; Cardiolipins ; metabolism ; Electron Transport ; Humans ; Mitochondria ; enzymology ; metabolism ; Multienzyme Complexes ; chemistry ; metabolism

Mitochondria participate in various intracellular metabolic pathways such as generating intracellular ATP, synthesizing several essential molecules, regulating calcium homeostasis, and producing the cell's reactive oxygen species (ROS). Emerging studies have demonstrated newly discovered roles of mitochondria, which participate in the regulation of innate immune responses by modulating NLRP3 inflammasomes. Here, we review the recently proposed pathways to be involved in mitochondria-mediated regulation of inflammasome activation and inflammation: 1) mitochondrial ROS, 2) calcium mobilization, 3) nicotinamide adenine dinucleotide (NAD+) reduction, 4) cardiolipin, 5) mitofusin, 6) mitochondrial DNA, 7) mitochondrial antiviral signaling protein. Furthermore, we highlight the significance of mitophagy as a negative regulator of mitochondrial damage and NLRP3 inflammasome activation, as potentially helpful therapeutic approaches which could potentially address uncontrolled inflammation.
Adenosine Triphosphate ; Calcium ; Cardiolipins ; DNA, Mitochondrial ; Homeostasis ; Immunity, Innate ; Inflammasomes* ; Inflammation ; Metabolic Networks and Pathways ; Mitochondria* ; Mitochondrial Degradation ; NAD ; Reactive Oxygen Species

OBJECTIVETo explore the effects of mitochondrial pathways on apoptosis in colon carcinoma cells induced by Tumor necrosis factor related apoptosis inducing ligand and offer evidences for TRAIL application in clinic.

METHODSApoptosis, integration of mitochondria (including DeltaPsim, cardiolipin), activity of Caspase-9 and release of cytochrome c in colon carcinoma cells SW1116 treated with TRAIL, were detected by means of flowcytometry, fluorometer method and western-blot at the different time point.

RESULTSAfter treated with TRAIL for 4 hours, the apoptosis index was 32.98%, and the damage of mitochondria occurred with DeltaPsim, cardiolipin decreased, and the activity of Caspase-9 and cytochrome c increased. The Caspase-9 activity at 24 hour was (48.12+/-2.21)micromol.L(-1).h(-1).mg(-1) protein. Mitochondrial damage induced by TRAIL could be inhibited by Caspase inhibitor Z-VAD. fmk.

CONCLUSIONMitochondrial pathways involved in the apoptosis of colon carcinoma cell induced by TRAIL. Cytochrome c was released and Caspase-9 was activated in the Caspase-dependent manner after the damage of mitochondrial.

Apoptosis ; Cardiolipins ; metabolism ; Caspase 9 ; metabolism ; Cell Line, Tumor ; Cytochromes c ; metabolism ; Humans ; Mitochondria ; metabolism ; Mitochondrial Membranes ; metabolism ; Receptors, TNF-Related Apoptosis-Inducing Ligand ; metabolism ; TNF-Related Apoptosis-Inducing Ligand ; metabolism