Cardiolipins: Acidic phospholipids composed of two molecules of phosphatidic acid covalently linked to a molecule of glycerol. They occur primarily in mitochondrial inner membranes and in bacterial plasma membranes. They are the main antigenic components of the Wassermann-type antigen that is used in nontreponemal SYPHILIS SERODIAGNOSIS.Mitochondria: Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)Glycerophospholipids: Derivatives of phosphatidic acid in which the hydrophobic regions are composed of two fatty acids and a polar alcohol is joined to the C-3 position of glycerol through a phosphodiester bond. They are named according to their polar head groups, such as phosphatidylcholine and phosphatidylethanolamine.Mitochondrial ADP, ATP Translocases: A class of nucleotide translocases found abundantly in mitochondria that function as integral components of the inner mitochondrial membrane. They facilitate the exchange of ADP and ATP between the cytosol and the mitochondria, thereby linking the subcellular compartments of ATP production to those of ATP utilization.Barth Syndrome: Rare congenital X-linked disorder of lipid metabolism. Barth syndrome is transmitted in an X-linked recessive pattern. The syndrome is characterized by muscular weakness, growth retardation, DILATED CARDIOMYOPATHY, variable NEUTROPENIA, 3-methylglutaconic aciduria (type II) and decreases in mitochondrial CARDIOLIPIN level. Other biochemical and morphological mitochondrial abnormalities also exist.Fatty Acids: Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)Oxidative Phosphorylation: Electron transfer through the cytochrome system liberating free energy which is transformed into high-energy phosphate bonds.Dictionaries, MedicalDictionaries as Topic: Lists of words, usually in alphabetical order, giving information about form, pronunciation, etymology, grammar, and meaning.Dictionaries, ChemicalPhospholipids: Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides see GLYCEROPHOSPHOLIPIDS) or sphingosine (SPHINGOLIPIDS). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system.Syphilis: A contagious venereal disease caused by the spirochete TREPONEMA PALLIDUM.Transferases (Other Substituted Phosphate Groups): A class of enzymes that transfers substituted phosphate groups. EC 2.7.8.Lactalbumin: A major protein fraction of milk obtained from the WHEY.Cell Membrane: The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.Phosphatidylserines: Derivatives of phosphatidic acids in which the phosphoric acid is bound in ester linkage to a serine moiety. Complete hydrolysis yields 1 mole of glycerol, phosphoric acid and serine and 2 moles of fatty acids.Phosphatidylcholines: Derivatives of phosphatidic acids in which the phosphoric acid is bound in ester linkage to a choline moiety. Complete hydrolysis yields 1 mole of glycerol, phosphoric acid and choline and 2 moles of fatty acids.Membrane Lipids: Lipids, predominantly phospholipids, cholesterol and small amounts of glycolipids found in membranes including cellular and intracellular membranes. These lipids may be arranged in bilayers in the membranes with integral proteins between the layers and peripheral proteins attached to the outside. Membrane lipids are required for active transport, several enzymatic activities and membrane formation.Fluorescent Treponemal Antibody-Absorption Test: Serologic assay that detects antibodies to Treponema pallidum, the etiologic agent of syphilis. After diluting the patient's serum to remove non-specific antibodies, the serum is mixed on a glass slide with Nichol's strain of Treponema pallidum. An antigen-antibody reaction occurs if the test is positive and the bound antibodies are detected with fluoresceinated antihuman gamma-globulin antibody.Abortion, Habitual: Three or more consecutive spontaneous abortions.Purpura, Thrombocytopenic, Idiopathic: Thrombocytopenia occurring in the absence of toxic exposure or a disease associated with decreased platelets. It is mediated by immune mechanisms, in most cases IMMUNOGLOBULIN G autoantibodies which attach to platelets and subsequently undergo destruction by macrophages. The disease is seen in acute (affecting children) and chronic (adult) forms.Purpura, Thrombocytopenic: Any form of purpura in which the PLATELET COUNT is decreased. Many forms are thought to be caused by immunological mechanisms.Sjogren's Syndrome: Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis.Lupus Erythematosus, Systemic: A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.Arthritis, Psoriatic: A type of inflammatory arthritis associated with PSORIASIS, often involving the axial joints and the peripheral terminal interphalangeal joints. It is characterized by the presence of HLA-B27-associated SPONDYLARTHROPATHY, and the absence of rheumatoid factor.Metabolomics: The systematic identification and quantitation of all the metabolic products of a cell, tissue, organ, or organism under varying conditions. The METABOLOME of a cell or organism is a dynamic collection of metabolites which represent its net response to current conditions.Magnetic Resonance Spectroscopy: Spectroscopic method of measuring the magnetic moment of elementary particles such as atomic nuclei, protons or electrons. It is employed in clinical applications such as NMR Tomography (MAGNETIC RESONANCE IMAGING).Syndrome: A characteristic symptom complex.Genetic Diseases, X-Linked: Genetic diseases that are linked to gene mutations on the X CHROMOSOME in humans (X CHROMOSOME, HUMAN) or the X CHROMOSOME in other species. Included here are animal models of human X-linked diseases.Mass Spectrometry: An analytical method used in determining the identity of a chemical based on its mass using mass analyzers/mass spectrometers.Attention Deficit Disorder with Hyperactivity: A behavior disorder originating in childhood in which the essential features are signs of developmentally inappropriate inattention, impulsivity, and hyperactivity. Although most individuals have symptoms of both inattention and hyperactivity-impulsivity, one or the other pattern may be predominant. The disorder is more frequent in males than females. Onset is in childhood. Symptoms often attenuate during late adolescence although a minority experience the full complement of symptoms into mid-adulthood. (From DSM-V)Methylphenidate: A central nervous system stimulant used most commonly in the treatment of ATTENTION DEFICIT DISORDER in children and for NARCOLEPSY. Its mechanisms appear to be similar to those of DEXTROAMPHETAMINE. The d-isomer of this drug is referred to as DEXMETHYLPHENIDATE HYDROCHLORIDE.Learning Disorders: Conditions characterized by a significant discrepancy between an individual's perceived level of intellect and their ability to acquire new language and other cognitive skills. These disorders may result from organic or psychological conditions. Relatively common subtypes include DYSLEXIA, DYSCALCULIA, and DYSGRAPHIA.Internet: A loose confederation of computer communication networks around the world. The networks that make up the Internet are connected through several backbone networks. The Internet grew out of the US Government ARPAnet project and was designed to facilitate information exchange.Adolescent Medicine: A branch of medicine pertaining to the diagnosis and treatment of diseases occurring during the period of ADOLESCENCE.Governing Board: The group in which legal authority is vested for the control of health-related institutions and organizations.Current Procedural Terminology: Descriptive terms and identifying codes for reporting medical services and procedures performed by PHYSICIANS. It is produced by the AMERICAN MEDICAL ASSOCIATION and used in insurance claim reporting for MEDICARE; MEDICAID; and private health insurance programs (From CPT 2002).Immunoglobulin G: The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.Glycerol: A trihydroxy sugar alcohol that is an intermediate in carbohydrate and lipid metabolism. It is used as a solvent, emollient, pharmaceutical agent, and sweetening agent.Glycerol Kinase: An enzyme that catalyzes the formation of glycerol 3-phosphate from ATP and glycerol. Dihydroxyacetone and L-glyceraldehyde can also act as acceptors; UTP and, in the case of the yeast enzyme, ITP and GTP can act as donors. It provides a way for glycerol derived from fats or glycerides to enter the glycolytic pathway. EC 2.7.1.30.Cytochrome c Group: A group of cytochromes with covalent thioether linkages between either or both of the vinyl side chains of protoheme and the protein. (Enzyme Nomenclature, 1992, p539)Electron Transport: The process by which ELECTRONS are transported from a reduced substrate to molecular OXYGEN. (From Bennington, Saunders Dictionary and Encyclopedia of Laboratory Medicine and Technology, 1984, p270)Cytochromes c: Cytochromes of the c type that are found in eukaryotic MITOCHONDRIA. They serve as redox intermediates that accept electrons from MITOCHONDRIAL ELECTRON TRANSPORT COMPLEX III and transfer them to MITOCHONDRIAL ELECTRON TRANSPORT COMPLEX IV.Electron Transport Complex IV: A multisubunit enzyme complex containing CYTOCHROME A GROUP; CYTOCHROME A3; two copper atoms; and 13 different protein subunits. It is the terminal oxidase complex of the RESPIRATORY CHAIN and collects electrons that are transferred from the reduced CYTOCHROME C GROUP and donates them to molecular OXYGEN, which is then reduced to water. The redox reaction is simultaneously coupled to the transport of PROTONS across the inner mitochondrial membrane.Cytochromes: Hemeproteins whose characteristic mode of action involves transfer of reducing equivalents which are associated with a reversible change in oxidation state of the prosthetic group. Formally, this redox change involves a single-electron, reversible equilibrium between the Fe(II) and Fe(III) states of the central iron atom (From Enzyme Nomenclature, 1992, p539). The various cytochrome subclasses are organized by the type of HEME and by the wavelength range of their reduced alpha-absorption bands.Cytidine Diphosphate Diglycerides: The ester of diacylglycerol with the terminal phosphate of cytidine diphosphate. It serves as an intermediate in the biosynthesis of phosphatidylethanolamine and phosphatidylserine in bacteria.Phosphatidylglycerols: A nitrogen-free class of lipids present in animal and particularly plant tissues and composed of one mole of glycerol and 1 or 2 moles of phosphatidic acid. Members of this group differ from one another in the nature of the fatty acids released on hydrolysis.Diacylglycerol Cholinephosphotransferase: An enzyme that catalyzes the synthesis of phosphatidylcholines from CDPcholine and 1,2-diacylglycerols. EC 2.7.8.2.Antibodies, Anticardiolipin: Antiphospholipid antibodies found in association with systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC;), ANTIPHOSPHOLIPID SYNDROME; and in a variety of other diseases as well as in healthy individuals. The antibodies are detected by solid-phase IMMUNOASSAY employing the purified phospholipid antigen CARDIOLIPIN.Diagnostic Uses of Chemicals: Chemicals administered to patients in order to diagnose or study the pathology of medical conditions, diseases, or syndromes.Autoantibodies: Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.beta 2-Glycoprotein I: A 44-kDa highly glycosylated plasma protein that binds phospholipids including CARDIOLIPIN; APOLIPOPROTEIN E RECEPTOR; membrane phospholipids, and other anionic phospholipid-containing moieties. It plays a role in coagulation and apoptotic processes. Formerly known as apolipoprotein H, it is an autoantigen in patients with ANTIPHOSPHOLIPID ANTIBODIES.United States Food and Drug Administration: An agency of the PUBLIC HEALTH SERVICE concerned with the overall planning, promoting, and administering of programs pertaining to maintaining standards of quality of foods, drugs, therapeutic devices, etc.Antiphospholipid Syndrome: The presence of antibodies directed against phospholipids (ANTIBODIES, ANTIPHOSPHOLIPID). The condition is associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses. In pregnancy it can cause abortion. Of the phospholipids, the cardiolipins show markedly elevated levels of anticardiolipin antibodies (ANTIBODIES, ANTICARDIOLIPIN). Present also are high levels of lupus anticoagulant (LUPUS COAGULATION INHIBITOR).

Nitric-oxide-induced apoptosis in human leukemic lines requires mitochondrial lipid degradation and cytochrome C release. (1/1010)

We have previously shown that nitric oxide (NO) stimulates apoptosis in different human neoplastic lymphoid cell lines through activation of caspases not only via CD95/CD95L interaction, but also independently of such death receptors. Here we investigated mitochondria-dependent mechanisms of NO-induced apoptosis in Jurkat leukemic cells. NO donor glycerol trinitrate (at the concentration, which induces apoptotic cell death) caused (1) a significant decrease in the concentration of cardiolipin, a major mitochondrial lipid; (2) a downregulation in respiratory chain complex activities; (3) a release of the mitochondrial protein cytochrome c into the cytosol; and (4) an activation of caspase-9 and caspase-3. These changes were accompanied by an increase in the number of cells with low mitochondrial transmembrane potential and with a high level of reactive oxygen species production. Higher resistance of the CD95-resistant Jurkat subclone (APO-R) cells to NO-mediated apoptosis correlated with the absence of cytochrome c release and with less alterations in other mitochondrial parameters. An inhibitor of lipid peroxidation, trolox, significantly suppressed NO-mediated apoptosis in APO-S Jurkat cells, whereas bongkrekic acid (BA), which blocks mitochondrial permeability transition, provided only a moderate antiapoptotic effect. Transfection of Jurkat cells with bcl-2 led to a complete block of apoptosis due to the prevention of changes in mitochondrial functions. We suggest that the mitochondrial damage (in particular, cardiolipin degradation and cytochrome c release) induced by NO in human leukemia cells plays a crucial role in the subsequent activation of caspase and apoptosis.  (+info)

Enzymatic properties of vesicle-reconstituted human cytochrome P450SCC (CYP11A1) differences in functioning of the mitochondrial electron-transfer chain using human and bovine adrenodoxin and activation by cardiolipin. (2/1010)

The recently reported heterologous expression and purification of both human cytochrome P450SCC and adrenodoxin [Woods, S.T., Sadleir, J., Downs, T., Triantopoulos, T., Haedlam, M.J. & Tuckey, R.C. (1998) Arch. Biochem. Biophys. 353, 109-115] has enabled us to perform studies with the membrane-reconstituted human enzymes to better understand the side-chain cleavage reaction in humans. Human P450SCC was successfully reconstituted into dioleoylphosphatidylcholine vesicles with and without cardiolipin and its enzymatic properties characterized in the membrane-bound state. Enhancement of the P450SCC activity and significant activation by cardiolipin were observed when human adrenodoxin instead of bovine adrenodoxin was used as electron donor. In the absence of cardiolipin, Km for cholesterol was decreased twice in the case of human adrenodoxin indicating enhanced cholesterol binding. On the other hand, in the presence of cardiolipin in the membrane both Km and V for cholesterol were decreased with human adrenodoxin as electron donor. Kinetic analysis of the interaction between human P450SCC and its redox partners provided evidence for enhanced binding of the human electron donor to human P450SCC indicated by both an increased V and decreased Kd for human adrenodoxin compared with the values with bovine adrenodoxin. Because no similar effects were observed in Tween 20 micelles, these results suggest that the phospholipid membrane may play an important role in the interaction of human adrenodoxin with human P450SCC.  (+info)

Phospholipid composition of Rickettsia prowazeki grown in chicken embryo yolk sacs. (3/1010)

The phospholipid composition and phospholipid fatty acid composition of purified Rickettsia prowazeki were determined. The lipid phosphorous content was 6.8 +/- 1.3 microgram/mg of total rickettsial protein. The major phospholipid was phosphatidylethanolamine (60 to 70%); phosphatidylglycerol constituted 20%, and phosphatidylcholine constituted 15%. Small amounts of phosphatidylserine and cardiolipin were detected. The principal fatty acids were 18:1, 16:1, and 16:0. The fatty acid composition of the phosphatidylcholine in the rickettsial extracts was very different than that of the other rickettsial phosphatides and very similar to that of normal yolk sac phosphatidylcholine. The specific of the phosphatidylcholine of rickettsiae grown in the presence of 32P was markedly lower than that of phosphatidylethanolamine and phosphatidylglycerol. It is suggested that the phosphatidylcholine in the rickettsial extract is yolk sac derived and either tightly absorbed or exchanged into the rickettsial membrane.  (+info)

Conformationally altered beta 2-glycoprotein I is the antigen for anti-cardiolipin autoantibodies. (4/1010)

Anti-cardiolipin autoantibodies (aCL) induce thrombosis and recurrent fetal death. These antibodies require a 'cofactor', identified as beta 2-glycoprotein I (beta 2-GPI), to bind phospholipids. We show here that aCL can bind beta 2-GPI in the absence of phospholipid. Binding of aCL to beta 2-GPI is dependent upon the beta 2-GPI being immobilized on an appropriate surface including cardiolipin, irradiated polystyrene and nitrocellulose membrane. This effect cannot be explained by increased antigen density of beta 2-GPI immobilized on these surfaces. Rather, conformational changes that occur following the interaction of beta 2-GPI with phospholipid render this protein antigenic to aCL. Liquid-phase beta 2-GPI was not antigenic for aCL. Thus, aCL cannot bind circulating beta 2-GPI. These findings may explain why patients with aCL can remain healthy for many years but then undergo episodes of thrombosis or fetal loss without changes in their circulating aCL profile, as the triggering event for these pathologies can be predicted to be one that renders beta 2-GPI antigenic for aCL.  (+info)

Transbilayer movement and distribution of spin-labelled phospholipids in the inner mitochondrial membrane. (5/1010)

The transmembrane diffusion and equilibrium distribution of spin-labelled phosphatidylethanolamine (PE*), phosphatidylcholine (PC*) and cardiolipin (CL*) were investigated in purified mitochondrial inner membranes using electron spin resonance spectroscopy. Using the back exchange technique, we found that the outside-inside movement of PE* and PC* in beef-heart inner mitochondrial membranes was rapid (t1/2 in the range 10-15 min at 30 degrees C). The steady-state distributions in non-energised mitoplasts were approximately 30% in the inner leaflet for PC* and 39% for PE*. Within the limits of probe concentration that can possibly be used in these experiments, the initial velocity of the inward movement was not saturable with respect to the amount of analogue added to the membranes, suggesting that the spin-labelled phospholipids diffused passively between the two leaflets of the inner mitochondrial membrane. In energised mitoplasts, PC* behaviour was not affected, PE* diffused approximately two times faster toward the inner monolayer but reached the same plateau. Treatment of energised mitochondria with N-ethylmaleimide did not affect PC* diffusion, while the kinetics of PE* internalisation became identical to that of PC*. Similar results were found when PC* and PE* movements were studied in mitoplasts from beef heart, rat liver or yeast. The spin-labelled cardiolipin, which possesses four long chains, had to be introduced in the mitoplast with some ethanol. After equilibration (t1/2 of the order of 13 min at 30 degrees C), the transmembrane distribution suggested that approximately half of the cardiolipin analogue remained in the outer leaflet. These results do not allow us to determine if a specific protein (or flippase) is involved in the phospholipid transmembrane traffic within inner mitochondrial membranes, but they show that lipids can rapidly flip through the mitochondrial membrane.  (+info)

Involvement of Arg-328, Arg-334 and Arg-342 of DnaA protein in the functional interaction with acidic phospholipids. (6/1010)

We reported previously that three basic amino acids (Arg-360, Arg-364 and Lys-372) of DnaA protein are essential for its functional interaction with cardiolipin. In this study, we examined the effect of mutation of some basic amino acids in a potential amphipathic helix (from Lys-327 to Ile-345) of DnaA protein on this interaction. ATP binding to the mutant DnaA protein, in which Arg-328, Arg-334 and Arg-342 were changed to acidic amino acids, was less inhibited by cardiolipin than that of the wild-type protein, as was the case for mutant DnaA protein with mutations of Arg-360, Arg-364 and Lys-372. A mutant DnaA protein with mutations of all six basic amino acids showed the most resistance to the inhibition of ATP binding by cardiolipin. These results suggest that Arg-328, Arg-334 and Arg-342, like Arg-360, Arg-364 and Lys-372, are also involved in the functional interaction between DnaA protein and acidic phospholipids.  (+info)

Maternal cardiolipin, beta 2-glycoprotein-I and prothrombin antibody expression in high-risk pregnancies with bilateral abnormal uterine artery Doppler waveforms. (7/1010)

OBJECTIVE: To compare the frequency of maternal serum antiphospholipid antibodies (to cardiolipin, beta 2-glycoprotein I and prothrombin) in pregnancies presenting with bilateral abnormal uterine artery Doppler waveforms. DESIGN: Retrospective analysis of stored serum. SUBJECTS: Cases comprised 47 singleton pregnancies with bilateral abnormal uterine artery Doppler waveforms at 24 weeks of gestation, followed from 20 weeks, and controls were 100 healthy pregnancies with normal uterine artery Doppler waveforms. METHODS: Ultrasound examination utilized a 5-MHz curvilinear transabdominal transducer with pulsed and color Doppler facilities. Antiphospholipid antibodies were analyzed by ELISA methodology, and reference ranges were established using the geometric mean +/- 2 SD of healthy non-pregnant adults. Human chorionic gonadotropin (hCG) levels were obtained from patient notes. RESULTS: Anticardiolipin antibodies were detected in 11 (23%) of the cases (IgG, n = 7; IgM, n = 6) compared with ten (10%) of the controls (p < 0.05). Low titer anticardiolipin IgG (range, 5.5-35.3; median, 6.3 GPL units) and anticardiolipin IgM (range, 3.4-14.7; median, 5.3 MPL units) were detected in cases. Amongst the cases, adverse perinatal outcomes were more common in the presence of raised levels of anticardiolipin antibodies. Anti-beta 2-glycoprotein I IgG was not detected in any of the cases. Antiprothrombin IgG was not detected, but antiprothrombin IgM occurred in 10.6% of cases compared with 2% of controls. CONCLUSIONS: Women with persistent bilateral abnormal uterine artery. Doppler waveforms in mid-gestation were more likely to express raised levels of anticardiolipin antibodies than healthy controls with normal uteroplacental perfusion. Anticardiolipin antibodies without anti-beta 2-glycoprotein I binding may be involved in the pathogenesis of uteroplacental ischemia in a proportion of high-risk pregnancies.  (+info)

Polar lipids of four Listeria species containing L-lysylcardiolipin, a novel lipid structure, and other unique phospholipids. (8/1010)

The membrane lipids of Listeria innocua, Listeria monocytogenes, Listeria seeligeri and Listeria welshimeri were fractionated on DEAE-cellulose and purified by chromatography on silica gel and/or preparative TLC. The lipid structures were elucidated by chemical and chromatographic means. The polar lipid composition of the four listeria species was similar. Phospholipids predominated. They consisted of phosphatidylglycerol, L-lysylphosphatidylglycerol, cardiolipin [bis(phosphatidyl)glycerol] and L-lysylcardiolipin. A phospholipid more polar than cardiolipin, possibly two L-lysyl derivatives of it, sn-glycero-1-phosphoglycolipid, its D-alanyl derivative, and polyprenol phosphate were also detected. Towards the end of exponential growth, the relative amounts of cardiolipin and L-lysylcardiolipin increased, approaching 47-78% lipid phosphorus with a ratio of L-lysylcardiolipin to cardiolipin of 0.25-1.6. As shown by fast atom bombardment-mass spectrometry, cardiolipin and L-lysylcardiolipin consisted of five molecular species due to various fatty acid combinations. L-lysylcardiolipin has so far not been found in nature. It belongs to the recently discovered class of substituted cardiolipins. Its occurrence in the four listeria species tested shows that it is a characteristic lipid component of the L. monocytogenes line of descent. Further studies on the lipid pattern of members of the other descent line are required to decide whether lysylcardiolipin can serve as a genus-specific chemotaxonomic marker for listeriae.  (+info)

Targeting mitochondrial cardiolipin and the cytochrome c/cardiolipin complex to promote electron transport and optimize mitochondrial ATP synthesis. Academic Article ...
CL(i-12:0/i-24:0/i-17:0/i-17:0) is a cardiolipin (CL). Cardiolipins are sometimes called double phospholipids because they have four fatty acid tails, instead of the usual two. They are glycerophospholipids in which the O1 and O3 oxygen atoms of the central glycerol moiety are each linked to one 1,3-diacylglyerol chain. Their general formula is OC(COP(O)(=O)OC[[email protected]@H](CO[R1])O[R2])COP(O)(=O)OC[[email protected]@H](CO[R3])O[R4], where R1-R4 are four fatty acyl chains. CL(i-12:0/i-24:0/i-17:0/i-17:0) contains one chain of 10-methylundecanoic acid at the C1 position, one chain of 22-methyltricosanoic acid at the C2 position, two chains of 15-methylhexadecanoic acid at the C3 and C4 positions fatty acids. Cardiolipins are known to be present in all mammalian cells, especially cells with a high number of mitochondria. De novo synthesis of Cardiolipins begins with condensing phosphatidic acid (PA) with cytidine-5-triphosphate (CTP) to form cytidine-diphosphate-1,2-diacyl-sn-glycerol (CDP-DG). Glycerol-3-phosphate ...
CL(18:2(9Z,12Z)/18:1(9Z)/18:1(9Z)/16:0) is a cardiolipin (CL). Cardiolipins are sometimes called double phospholipids because they have four fatty acid tails, instead of the usual two. They are glycerophospholipids in which the O1 and O3 oxygen atoms of the central glycerol moiety are each linked to one 1,3-diacylglyerol chain. Their general formula is OC(COP(O)(=O)OC[[email protected]@H](CO[R1])O[R2])COP(O)(=O)OC[[email protected]@H](CO[R3])O[R4], where R1-R4 are four fatty acyl chains. CL(18:2(9Z,12Z)/18:1(9Z)/18:1(9Z)/16:0) contains one chain of (9Z,12Z-octadecadienoyl) at the C1 position, two chains of (9Z-octadecenoyl) at the C2 and C3 positions, one chain of hexadecanoic acid at the C4 position fatty acids. Cardiolipins are known to be present in all mammalian cells, especially cells with a high number of mitochondria. De novo synthesis of Cardiolipins begins with condensing phosphatidic acid (PA) with cytidine-5-triphosphate (CTP) to form cytidine-diphosphate-1,2-diacyl-sn-glycerol (CDP-DG). Glycerol-3-phosphate is ...
Cardiolipin is found predominantly in the membranes of mitochondria and bacteria. It is a unique diphosphatidylglycerol containing four acyl groups. In animal tissues 80% of the acyl groups are composed of linoleic acid (18:2(n-6)).

The identification of Barth′s syndrome, a disease associated with abnormalities in the fatty acid composition of cardiolipin causing cardiomyopathy and neutropenia, has displayed the importance of cardiolipin in mitochondrial functionality. Cardiolipin effects mitochondrial bioenergetics by providing essential structural and functional support to proteins localized within the mitochondria. Specifically, interaction of cardiolipin with cytochrome oxidase and adenine nucleotide translocase is required in order for both of these mitochondrial enzymes to maintain activity.

Cardiolipin has been shown to perform a role in a number of other specific biological activities, including oxidative phosphorylation, apoptosis, cholesterol translocation and gene
Genetic analysis (chromosome/ Karyotype analysis): Blood samples should be taken from man and woman and tested for some probable abnormalities in the chromosomes, (missing chromosomes or additions to each chromosome, and translocations of chromosomes (in which all chromosomes are present but in different locations).. Anti Phospholipid Antibodies (APA) - cardiolipins are proteins found in many cells in your body. They help regulate blood flow throughout the body. However, when your body looks at the cardiolipins as an enemy or invader, it will attack them by creating antibodies . The three main groups anticardiolipins are: IgG, IgM, IgA.. Anti Cardiolipin Antibodies (ACA)- cells that attack the nuclei of other cells in your body. This is a mistake made by the body, thinking the good guys are the bad guys. Low levels should cause no problems.. Anti Nuclear Antibodies (ANA)- Again this is your body mistaking the good for the bad. Only this time it attacks the nuclei of the cell(s).. Lupus Anti ...
Acyl-CoA:lysocardiolipin acyltransferase. Possesses both lysophosphatidylinositol acyltransferase (LPIAT) and lysophosphatidylglycerol acyltransferase (LPGAT) activities. Recognizes both monolysocardiolipin and dilysocardiolipin as substrates with a preference for linoleoyl-CoA and oleoyl-CoA as acyl donors. Acts as a remodeling enzyme for cardiolipin, a major membrane polyglycerophospholipid. Converts lysophosphatidic acid (LPA) into phosphatidic acid (PA) with a relatively low activity. Required for establishment of the hematopoietic and endothelial lineages ...
The importance of the content of anionic phospholipids [cardiolipin (CL) and phosphatidylglycerol (PG)] in the osmotic adaptation and in the membrane structure of Bacillus subtilis cultures was investigated. Insertion mutations in the three putative cardiolipin synthase genes (ywiE, ywnE and ywjE) were obtained. Only the ywnE mutation resulted in a complete deficiency in cardiolipin and thus corresponds to a true clsA gene. The osmotolerance of a clsA mutant was impaired: although at NaCl concentrations lower than 1·2 M the growth curves were similar to those of its wild-type control, at 1·5 M NaCl (LBN medium) the lag period increased and the maximal optical density reached was lower. The membrane of the clsA mutant strain showed an increased PG content, at both exponential and stationary phase, but no trace of CL in either LB or LBN medium. As well as the deficiency in CL synthesis, the clsA mutant showed other differences in lipid and fatty acids content compared to the wild-type, suggesting a
1-MLCL(0:0/16:0/16:0/16:0) is a monolysocardiolipin (MLCL). Monolysocardiolipins have three fatty acid tails, instead of the usual two. 1-MLCL(0:0/16:0/16:0/16:0) contains one chain of at the C1 position, three chains of hexadecanoic acid at the C2, C3 and C4 positions. MLCL is present in eukaryotes as part of the metabolic cycle of mitochondrial lipids. Removal of one acyl chain from a cardiolipin results in generation of monolysocardiolipin (MLCL). MLCL has been used as an inter-mediate in the synthesis of spin-labeled CL to study the interaction of CL with mitochondrial enzymes. Because a role for MLCL has been suggested in apoptosis, this molecule has been used to study its interaction with various enzymes involved in lipid remodeling and apoptosis. There are two species of monolysocardiolipins, 1-MLCL which is missing a fatty acid in position R1 the and 2-MLCL which is missing a fatty acid in position R4 ...
The design of a human immunodeficiency virus-1 (HIV-1) immunogen that can induce broadly reactive neutralizing antibodies is a major goal of HIV-1 vaccine development. Although rare human monoclonal antibodies (mAbs) exist that broadly neutralize HIV-1, HIV-1 envelope immunogens do not induce these antibody specificities. Here we demonstrate that the two most broadly reactive HIV-1 envelope gp41 human mAbs, 2F5 and 4E10, are polyspecific autoantibodies reactive with the phospholipid cardiolipin. Thus, current HIV-1 vaccines may not induce these types of antibodies because of autoantigen mimicry of the conserved membrane-proximal epitopes of the virus. These results may have important implications for generating effective neutralizing antibody responses by using HIV-1 vaccines.. ...
Define cardiolipin. cardiolipin synonyms, cardiolipin pronunciation, cardiolipin translation, English dictionary definition of cardiolipin. n. A phospholipid usually obtained from beef heart, used in combination with phosphatidylcholine and cholesterol as an antigen to diagnose syphilis
CLSAs vision is that partners will enrich the CLSA research platform, promote and foster its development into a dynamic, rich, comprehensive and unique resource for aging research. This premium resource will result in beneficial rewards for the partners through facilitating rapid adoption of sound research into health practice, programs, policies, services and products that will contribute to healthy aging and enhanced quality of life for older adults. The CLSA has been successful in additional fundraising with a variety of partners to support the implementation and baseline data collection phase of the CLSA (2010-2015). The CLSA continues to develop partnerships for the first (2015-2018) and second (2018-2021) follow-ups to support and sustain the long-term vision of the CLSA. A full list of CLSA partnerships is available on the Partners section of the CLSA website.. ...
Another focus of our research on cytochrome c is the proteins interaction with anionic cardiolipin containing liposomes by CD, absorption and resonance Raman spectroscopy. The specific aim of this project is to fully characterize structure and energy landscape of oxidized and reduced cytochrome c after the binding to the surface of such liposomes and after dissociation form the surface. These liposome serve as a model for the innermembrane of mitochondria. This will lead us to understand why the protein gains peroxidase activity on membrane surface if challenged with hydrogen peroxide. A detailed study aimed at exploring the binding and subsequent conformational changes of ferricytochrome c to cardiolipin containing liposomes have revealed two protein binding sites. For liposomes with 20% cardiolipin binding via site 1 is insensitive to the addition of NaCl and mostly preserves the native state, including the Fe-M80 linkage. Binding via site 2, however, causes a conformational transition into a ...
The mitochondrial calcium uniporter is a Ca2+‐activated Ca2+ channel that is essential for dynamic modulation of mitochondrial function in response to cellular Ca2+ signals. It is regulated by two paralogous EF‐hand proteins-MICU1 and MICU2, but the mechanism is unknown. Here, we demonstrate that both MICU1 and MICU2 are stabilized by Ca2+. We reconstitute the MICU1-MICU2 heterodimer and demonstrate that it binds Ca2+ cooperatively with high affinity. We discover that both MICU1 and MICU2 exhibit affinity for the mitochondria‐specific lipid cardiolipin. We determine the minimum Ca2+ concentration required for disinhibition of the uniporter in permeabilized cells and report a close match with the Ca2+‐binding affinity of MICU1-MICU2. We conclude that cooperative, high‐affinity interaction of the MICU1-MICU2 complex with Ca2+ serves as an on-off switch, leading to a tightly controlled channel, capable of responding directly to cytosolic Ca2+ signals. ...
Mitochondrial lipids in neurodegeneration. . Biblioteca virtual para leer y descargar libros, documentos, trabajos y tesis universitarias en PDF. Material universiario, documentación y tareas realizadas por universitarios en nuestra biblioteca. Para descargar gratis y para leer online.
Ornithinyl ester of phosphatidyl glycerol was found to accumulate in Mycobacterium 607 under acidic conditions (pH 5.6) of growth or in cultures of ultraviolet-irradiated (320 to 420 nm) bacilli. There was a corresponding decrease in cardiolipin content of the organisms under these conditions. ...
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In vitro, the TRIAP1:PRELID3A complex mediates the transfer of phosphatidic acid (PA) between liposomes and probably functions as a PA transporter across the mitochondrion intermembrane space. Phosphatidic acid import is required for cardiolipin (CL) synthesis in the mitochondrial inner membrane.
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Tafazzin is a protein that in humans is encoded by the TAZ gene. Tafazzin is highly expressed in cardiac and skeletal muscle. It is involved in the metabolism of cardiolipin. Tafazzin functions as a phospholipid-lysophospholipid transacylase. The mutation of the tafazzin gene is associated with a number of clinical disorders including Barth syndrome (BTHS) (type II 3-Methylglutaconic aciduria), dilated cardiomyopathy (DCM), hypertrophic DCM, endocardial fibroelastosis, and left ventricular noncompaction (LVNC). Tafazzin is responsible for remodeling of a phospholipid cardiolipin (CL), the signature lipid of the mitochondrial inner membrane. As a result, BTHS patients exhibit defects in CL metabolism, including aberrant CL fatty acyl composition, accumulation of monolysocardiolipin (MLCL) and reduced total CL levels. The protein was identified by Italian scientists Silvia Bione et al. in 1996. Owing to the complex procedure required for the identification of tafazzin, the protein was named after ...
Quantitative and qualitative alterations of mitochondrial cardiolipin have been implicated in the pathogenesis of Barth syndrome, an X-linked cardioskeletal myopathy caused by a deficiency in tafazzin, an enzyme in the cardiolipin remodeling pathway. A tafazzin-deficient Drosophila model of Barth syndrome that is characterized by low cardiolipin (CL) concentration, abnormal cardiolipin fatty acyl composition, abnormal mitochondria, and poor motor function has been generated earlier. This study shows that tafazzin deficiency in Drosophila disrupts the final stage of spermatogenesis, spermatid individualization, and causes male sterility. This phenotype can be genetically suppressed by inactivation of the gene encoding a calcium-independent phospholipase A2, iPLA2-VIA, which also prevents cardiolipin depletion/monolysocardiolipin accumulation, although in wild-type flies inactivation of the iPLA2-VIA does not affect the molecular composition of cardiolipin. Furthermore, it was shown that treatment ...
Barth syndrome (BTHS) is an X-linked genetic condition, usually transmitted from mother to son (although there is a relatively high incidence of new mutations in Barth syndrome and one confirmed case report of a female Barth syndrome patient). A mother who is a carrier of a Barth syndrome mutation (the gene is named tafazzin - also called TAZ or G4.5) shows no signs or symptoms of this disorder herself, probably due to skewed X-chromosome inactivation.. There is a 50% chance that a boy born to a female carrier will have Barth syndrome, whereas girls born to a carrier have a 50% risk of being carriers themselves. All daughters of a male with Barth syndrome will be carriers, however no sons will be affected. Because there are proven non-carrier mothers, all mothers of Barth syndrome children should be tested in order to define the genetic risk in each family.. Any male child related through the female line to a Barth syndrome individual should be tested for the disorder, as there can be great ...
Anticardiolipin antibodies purified from serum from patients with systemic lupus erythematosus (SLE) by cardiolipin micelles were studied for their effects on lymphocytes and neutrophils. At a concentration of 160 micrograms/ml they markedly suppressed the [3H]thymidine incorporation of mononuclear cells stimulated by phytohaemagglutinin (4.9 (SEM 1.9%) of the control) and pokeweed mitogen (26.7 (10.5%) of the control). In addition, anticardiolipin antibodies changed the cell cycle of phytohaemagglutinin stimulated lymphocytes such that the S and G2+M phases were significantly diminished (G0/G1 = 64.62%, S = 20.59%, G2+M = 14.78% in the presence of normal human IgG v G0/G1 = 86.07%, S = 10.32%, G2+M = 3.59% in the presence of anticardiolipin antibodies). The suppression of lymphocyte proliferation by anticardiolipin antibodies was shown not to be caused by an alteration of T cell subpopulations. However, the interleukin 2 receptors on the cell surface and the soluble interleukin 2 receptors in ...
The results of this study show that the synthesis of the inner mitochondrial membrane phospholipid, cardiolipin, is markedly inhibited following infection of human embryonic lung or hamster embryo fibroblast cells with herpes simplex virus type 1 or 2. The synthesis of other phospholipids, i.e., phosphatidylcholine, phosphatidylethanolamine, sphingomyelin, and phosphatidylinositol, is relatively unaffected.
Tafazzin knockdown causes hypertrophy of neonatal cardiac myocytes (34), and mutation of the tafazzin gene causes dilated cardiomyopathy in Barth syndrome (64). Our work with neonatal cardiac fibroblasts (NVFs) showed that tafazzin knockdown increased ROS production, activated MAPKs including p42/44 and p38, stimulated transcriptional and translational factors, which in turn activated cell cycle regulators, and increased DNA and protein synthesis. On the other hand, tafazzin knockdown also decreased intracellular ATP, activated AMPK, and halted the energy-consuming process (i.e., cell proliferation), ultimately resulting in multinucleation, hypertrophy, and enhanced collagen secretion.. Tafazzin plays an important role in de novo cardiolipin synthesis and remodeling in the mitochondria. Tafazzin knockdown leads to reduced cardiolipin, which is consistent with previous studies involving yeast (28), Drosophila (72), and human skin fibroblasts (68). To be consistent with what is reported in mice ...
Hydrothermal experiments were conducted at ca. 1 to 7000 bars and 700 to 1250 °C in 121 rhyolitic to basaltic systems to determine Cl solubility in silicate melts, i.e., the maximum Cl concentration in melts that are saturated in a hydrosaline liquid with or without an aqueous or aqueous-carbonic vapor. The Cl concentration of melts increases with the Cl contents of the fluid unless the melt coexists with vapor plus hydrosaline liquid at fixed pressure and temperature; this phase assemblage buffers the Cl content of each phase with increasing Cl in the system. The Cl content of fluid(s)-saturated melts is independent of the CO2 concentration of the saline liquid ± vapor with up to 21 wt% CO2 in the fluid(s). The experiments show that Cl dissolution in aluminosilicate melts increases with temperature and pressure. Chlorine solubility is also a function of melt composition; it increases with the molar ([Al1/2+Ca1/2+Mg1/2+Na]/Si) of the melt.. These experimental data have been integrated with ...
Catalyzes the synthesis of cardiolipin (CL) (diphosphatidylglycerol) by specifically transferring a phosphatidyl group from CDP-diacylglycerol to phosphatidylglycerol (PG). CL is a key phospholipid in mitochondrial membranes and plays important roles in maintaining the functional integrity and dynamics of mitochondria under both optimal and stress conditions ...
Translocation of ions across biological membranes is an essential process of life. It is enabled by membrane proteins and relies on tight integration of these proteins into the lipid bilayer. The tight seal is the prerequisite for the generation of electrochemical proton gradients which are the key to biological energy conversion. It is now generally accepted that specific interactions between individual lipids and proteins are crucial for function and structural integrity of membrane proteins [1,2]. Of special interest is cardiolipin (CL), the signature lipid of mitochondria. CL is essential for the function of respiratory complex III and other mitochondrial membrane proteins and also for the integrity of respiratory supercomplexes. Yet, the molecular mechanisms and underlying structure-function relationships are not understood. Disruption of CL biosynthesis leads to the Barth syndrome. This cardio-skeletal myopathy is caused by mutations in the gene of the acyl transferase tafazzin resulting ...
Park, C E. and Wenner, C E., "Mitochondrial lipids of eld ascites tumor cells. Abstr." (1968). Subject Strain Bibliography 1968. 223 ...
AccessGUDID - ANTI-CARDIOLIPIN IgA (04260157080133)- Anti-Cardiolipin Screen is an ELISA test system for the quantitative measurement of IgA class autoantibodies against cardiolipin in human serum or plasma. This product is intended for professional in vitro diagnostic use only.
혈액투석 환자에서 혈전형성에 의한 동정맥루 폐쇄 (vascualr access occlusion)는 가장 흔한 합병증 중의 하나로 혈관 수술 기술의 발전, 동맥경화증의 치료 및 항 혈소판 제제 사용을 포함한 약물적 치료에도 불구하고 혈액투석 환자 중 입원일수의 5-50% 정도를 차지하는 것으로 되어 있다. Anticardiolipin Antibody(ACA) 와 lupus anticoagulant(LA)는 항 인지질 항체들로 혈관 폐쇄 및 반복적인 유산의 중요한 위험인자로 알려져 있으나 혈액투석 치료를 받는 환자에서의 정확한 양성율 및 동정맥루 폐쇄와의 상관관계에 관해서는 아직 많은 이견이 있는 상태이다. 이에 저자는 이화여자대학교 부속병원에서 유지 혈액투석 중인 환자 50명, 총 68예의 동정맥루 (52 AV fistula, 16 AV grafts)를 대상으로 항 cardiolipin 항체와 루프스 항응고인자를 측정하고 병력 및 임상 기록 검토를 통해 이러한 ...
1. The [32P]phosphate incorporated into the phospholipids of isolated rat hepatic cells is present in phosphatidic acid and to a smaller extent in phosphatidylinositol. 2. The ability to synthesize nitrogen-containing phospholipids is restored by adding a liver supernatant fraction, and it is suggested that the metabolic deficiency is caused by the leakage of cytoplasmic enzymes of the synthetase system from the cells. 3. Fortified cell preparations were pulse-labelled with [32P]phosphate, [Me-14C]choline, [2-14C]ethanolamine and [U-14C]inositol and the subsequent fate of the labelled microsomal and mitochondrial phospholipids followed. 4. A fall in the specific radioactivity of microsomal phospholipids and a rise in that of mitochondrial phospholipids is interpreted as providing evidence of a transfer of labelled phospholipid molecules from the synthetic site (endoplasmic reticulum) to the mitochondrial membranes in the intact cells. 5. The formation of the phospholipids of mitochondrial ...
Like many genetic disorders, Barth syndrome is quite variable among different families and sometimes even within a single sibship. Whereas at least 80% of known patients with Barth syndrome manifest all four principal diagnostic criteria at some time during childhood, any or possibly even all of the cardinal findings may be absent in a boy with a proven mutation in the Barth gene. This variability in presentation of symptoms and severity in phenotype makes Barth syndrome a difficult disorder to diagnose. In absence of a family history of related illnesses, the clinician is presented with the challenge to diagnose a child who inherits the disorder through a spontaneous mutation. The diagnosis of Barth syndrome should be considered for any child or adult found to have any one of its four cardinal clinical characteristics, and evaluation for the other diagnostic criteria should be undertaken by obtaining the following studies:. ...
Quality Cardiolipin ELISA kit from ELISA kits manufacturer and elisa kits suppliers: Cardiolipin IgG ELISA kit. Our kits are FDA-CE and ISO certified.
A Perry, Florida mom is featured in this months edition of Readers Digest for promoting awareness of Barth Syndrome. Her son suffers from the disease that attacks young children.
MILPITAS, Calif. /ePRNews/ Cardiolipin is an important phospholipid present in the inner mitochondrial membrane and constitutes about 20% of its total lipid composition. It is essential for several mitochondrial functions such as electron…
Prinn Panitchpakdi, Country Head for Thailand at CLSA, discusses the armys decision to begin paying almost $5 billion worth of rice subsidies owed to Thai farmers.
This test helps your healthcare provider diagnose clotting disorders and autoimmune diseases. It measures the concentration of antibodies related to a fat molecule in your blood.
This test helps your healthcare provider diagnose clotting disorders and autoimmune diseases. It measures the concentration of antibodies related to a fat molecule in your blood.
The McCurdy Family (clockwise from left): Steve, Will, Kate, andEliza (photo courtesy of the McCurdy family) For more on the McCurdys and the Barth Syndrome Foundation, tune in to NBCs TODAY show onMonday, February 16. From the day he was born in 1986, Will McCurdy was a bright, engaging, and alert child. Butwhen he [...]
The intestinal epithelial cells (IECs) that line the gut form a robust line of defense against ingested pathogens. We investigated the impact of infection with the enteric pathogen Citrobacter rodentium on mouse IEC metabolism using global proteomic and targeted metabolomics and lipidomics. The major signatures of the infection were upregulation of the sugar transporter Sglt4, aerobic glycolysis, and production of phosphocreatine, which mobilizes cytosolic energy. In contrast, biogenesis of mitochondrial cardiolipins, essential for ATP production, was inhibited, which coincided with increased levels of mucosal O2 and a reduction in colon-associated anaerobic commensals. In addition, IECs responded to infection by activating Srebp2 and the cholesterol biosynthetic pathway. Unexpectedly, infected IECs also upregulated the cholesterol efflux proteins AbcA1, AbcG8, and ApoA1, resulting in higher levels of fecal cholesterol and a bloom of Proteobacteria. These results suggest that C. rodentium ...
Barth Syndrome is the only known Mendelian disorder of cardiolipin remodeling, with characteristic clinical features of cardiomyopathy, skeletal myopathy, and neutropenia. While the primary biochemical defects of reduced mature cardiolipin and increased monolysocardiolipin are well-described, much of the downstream biochemical dysregulation has not been uncovered, and biomarkers are limited. In order to further expand upon the knowledge of the biochemical abnormalities in Barth Syndrome, we analyzed metabolite profiles in plasma from a cohort of individuals with Barth Syndrome compared to age-matched controls via 1H nuclear magnetic resonance spectroscopy and liquid chromatography-mass spectrometry. A clear distinction between metabolite profiles of individuals with Barth Syndrome and controls was observed, and was defined by an array of metabolite classes including amino acids and lipids. Pathway analysis of these discriminating metabolites revealed involvement of mitochondrial and ...
Background: Graves disease is an autoimmune disease, characterized by the presence of antibodies directed to TSH receptor or nearby regions as well as antibodies to double strands DNA (dsDNA) anticardiolipin and nuclear antibodies. This study evaluated anticardiolipin and rheumatoid factor, such as IgA and IgM antibodies in patients with Graves disease. Patients and methods: Anticardiolipin and rheumatoid factor were measured in sera of 84 patients (29 male, 55 female) with evidence of Graves disease and 41 healthy individuals (15 male, 26 female) with negative history of hyperthyroidism and other autoimmune diseases. Results: Mean level of anti cardiolipin antibody (ACLA) in patients and control groups were 0.192±0.11 and 0.087±0.200 optical density (OD) respectively. The level of IgM-Rhematoid factor (IgM-RF) of patients and healthy control groups was the same, whereas the mean IgA-RF levels in patients was significantly lower than control group. Conclusion: Anticardiolipin level in different
Barth syndrome (BTHS), also known as 3-Methylglutaconic aciduria type 2, is an X-linked genetic disorder. The disorder, which affects multiple body systems, has so far been found exclusively in males. It is named after Dutch pediatric neurologist Peter Barth.Wikipedia Rare mutations in the TAZ gene on the X chromosome may lead to Barth syndrome. ...
Identification of cardiolipin as the membrane receptor of mitochondrial creatine kinase and determination of the transverse distribution of cardiolipin accross the inner mitochondrial ...
In patients with BTHS, we found abnormal molecular compositions of several phospholipids, including cardiolipin, phosphatidylcholine, and phosphatidylethanolamine. The data indicate a maldistribution of fatty acids (i.e., fatty acids lost their preference for specific phospholipids). For instance, the characteristic predominance of linoleic acid in cardiolipin was missing in BTHS. Conversely, the content of linoleoyl was increased in phosphatidylcholine and phosphatidylethanolamine. The notion of misdirected fatty acids was consistent with the presumed acyltransferase defect in BTHS. The most striking consequence of this defect was the absence of tetralinoleoyl-cardiolipin, a major molecular species in several control tissues (24). However, the molecular composition of cardiolipin was altered even in lymphoblasts that did not contain any tetralinoleoyl-cardiolipin, suggesting a general impairment of fatty acid trafficking from and to cardiolipin. In phosphatidylcholine and ...
Treatment of Barth Syndrome by CARDIOlipin MANipulation (CARDIOMAN): A randomised placebo controlled pilot trial conducted by the nationally commissioned Barth Syndrome Service. Sponsor: University Hospitals Bristol NHS Foundation Trust. Status: Recruiting. Funding: National Institute for Health Research (NIHR) Efficacy and Mechanism Evaluation (EME) programme and the Barth Syndrome Foundation USA.. Barth syndrome is a rare, life threatening, genetic disease which affects young males. It is caused by abnormal fats (lipids) in the powerhouses of cells (mitochondria) and those who suffer with it often develop heart failure, heart rhythm abnormalities, bacterial infections, poor growth or feeding, weak muscles, developmental delay, severe exercise intolerance, lethargy and fatigue; all of which affect their daily life. Low white blood cell counts occur frequently due to intermittent or persistent reduction in numbers of the neutrophils that are responsible for fighting bacterial infections. This ...
Stealth BioTherapeutics announced that the FDA has granted Fast Track designation for its lead candidate, elamipretide, for the treatment of Barth syndrome.
2WW = 2-Week Wait 47XXY = Klinefelters Syndrome ACA = Anti-cardiolipin Antibodies ACTH = Adrenal Corticotropic Hormone AF = Aunt Flo (menstruation)...
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Fatty Acids, Lipids, and IsoprenoidsPhospholipidsGlycerolipid and Glycerophospholipid Metabolism in Bacteria Cardiolipin synthetase (EC 2.7.8.-) ...
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To the Editor: Misra and colleagues (1) reported an increased incidence of anticardiolipin antibodies in patients with infectious mononucleosis and postulated the existence of cross-reactions between infectious (viral) antigens and cardiolipin. We report here our experience concerning immunologic and biochemical similarities between Pneumocystis carinii organisms and cardiolipin. We observed that in the acquired immunodeficiency syndrome (AIDS), anticardiolipin antibodies occurred almost exclusively among those patients who had active P. carinii pneumonia (2). To test the cross-reactive hypothesis, we did several experiments.. In the first set of experiments, rabbits were immunized with an antigen from P. carinii in Freunds adjuvant prepared according ...
Dietary conditioning of juvenile trout changed the acyl chain composition of mitochondrial phospholipids and the oxidative capacities of muscle mitochondria. Trout were fed three diets differing only in fatty acid (FA) composition. The highly unsaturated 22:6 n-3 (DHA) accounted for 0.4, 14, and 30% of fatty acids in Diets 1, 2 and 3. After 10 weeks of growth, the dietary groups differed markedly in FA composition of mitochondrial phospholipids, with significant dietary effects for virtually all FA. Mean mitochondrial DHA levels were 19, 40 and 33% in trout fed Diets 1, 2 and 3. Mitochondrial oxidative capacities changed with diet, while mitochondrial concentrations of cytochromes and of the adenylate nucleotide translocase (nmol mg(1) protein) did not. Mitochondria from fish fed Diet 1 had higher non-phosphorylating (state 4) rates at 5 degrees C than those fed other diets. When phosphorylating (state 3) rates differed between dietary groups, rates at 5 and 15 degrees C were higher for fish fed the
Our data suggest that subsequent thrombo-occlusive events and death after focal cerebral ischemia may occur sooner and more frequently in patients found to have IgG aCL immunoreactivity ,40 GPL units at the time of their index focal cerebral ischemic event when compared with patients who harbor 10 to 40 GPL at the time of their index event. Therefore GPL ,40 may be an important prognostic marker for subsequent thrombo-occlusive events in patients with index focal cerebral ischemia. Patients with ,40 GPL also appear to have more features of the antiphospholipid syndrome: younger age at onset and more recurrent strokes at the time of diagnosis.21 Furthermore, being in the GPL ,40 group appears to confer a sixfold-increased risk of developing subsequent TIAs only compared with being in the GPL ≤40 group. TIAs do not generally have as equally bad an outcome as ischemic stroke, although many hemispheric TIAs are minor infarcts by persistent subtle signs or neuroimaging abnormalities, and cognitive ...
Lencel; P; Hardouin; P; Magne; D. Do cytokines induce vascular calcification by the mere stimulation of TNAP activity? Med Hypotheses. 2010 Dec;75(6):517-21. IF = 1.1. Fellah; BH; Delorme; B; Sohier; J; Magne; D; Hardouin; P; Layrolle; P. Macrophage and osteoblast responses to biphasic calcium phosphate microparticles. J Biomed Mater Res A. 2010 Jun 15;93(4):1588-95. IF = 3.4. Granjon; T; Maniti; O; Auchli; Y; Dahinden; P; Buchet; R; Marcillat; O; Dimroth; P. Structure-function relations in oxaloacetate decarboxylase complex. Fluorescence and infrared approaches to monitor oxomalonate and Na(+) binding effect. PLoS One. 2010 Jun 3;5(6):e10935. IF = 3.2. Maniti; O; Lecompte; MF; Marcillat; O; Vial; C; Granjon; T. Mitochondrial creatine kinase interaction with cardiolipin-containing biomimetic membranes is a two-step process involving adsorption and insertion. Eur Biophys J. 2010 Nov;39(12):1649-55. IF = 2.2. Gouttenoire; J; Bougault; C; Aubert-Foucher; E; Perrier; E; Ronzière; MC; Sandell; L; ...
In the present study, we explored sexual dimorphism of doxorubicin cardiotoxicity and energetic and signaling pathways that could be involved in these differences. Two clinically relevant cumulative doses of doxorubicin, either 14 mg/kg after 7 injections or 8 mg/kg after 4 injections were administrated to investigate sex differences in the cardiotoxicity of doxorubicin. Doxorubicin treatment resulted in sex differences characterized in males by (1) important weight loss and decrease in survival rate, (2) strong alterations of myocardial function, (3) decrease in energy signaling pathways, (4) downregulation of mitochondrial biogenesis, (5) decrease in cardiolipin content, (6) decrease in mitochondrial DNA content, and (7) and alteration of mitochondrial respiration. No sex differences were found for the oxidative stress response or for death markers, whereas mitochondrial dysfunction and mitochondrial protein expression were associated with early cardiotoxicity in males.. Several clinical ...
Wang et al (Nat Med. 2014 May 11. doi: 10.1038/nm.3545.) just published a fascinating set of experiences in which they created a model of a Barth Syndrome cardiac dysfunction by using "heart on chip" technology. This technology involves using Barth patient derived induced pluripotent stem cell derived cardiomyocytes seeded on micropatterned fibronectin rectangles in order to recapitulate cardiac tissue structure. From these experiments, the authors were able to derive information about sarcomere organization, contractile dysfunction among other relevant parameters. This model, and the success of these experiments, holds great promise in the development of therapies for cardiomyopathy, as well as creating a system in which phenotype/genotype correlations of specific gene mutations can be better understood.. Hilary Vernon, MD PhD. ...
Saha, N.,Kamboh, M.I.,Kelly, L.J.,Ferrell, R.E.,Tay, J.S. (1992). Apolipoprotein H (beta-2-glycoprotein I) polymorphism in Asians.. Human Biology 64 (4) : 617-621. [email protected] Repository ...
Free resource for searching and exporting immune epitopes. Includes more than 95% of all published infectious disease, allergy, autoimmune, and transplant epitope data.
Discover Canadas largest research platform on aging and health. The first major data release from the Canadian Longitudinal Study on Aging (CLSA) is underway. Researchers interested in accessing the CLSA platform are invited to visit the DataPreview Portal on the CLSA website for detailed information about the available data and the application process. The CLSA is the most comprehensive study of aging ever undertaken in Canada. The study will follow 50,000 individuals aged 45 to 85 for 20 years, collecting information about their physical, emotional and social health through telephone interviews, in-home interviews and physical assessments. To date, more than 42,000 participants have been recruited.. The CLSA data will enable researchers to investigate the biological and psychological changes that frequently accompany aging, as well as non-medical factors such as social and economic changes. The longitudinal data will also provide opportunities for sound research that will inform decisions to ...
Das APOH Gen kodiert das Apolipoprotein H, welches die Brücke zwischen Lipidstoffwechsel, Koagulation und Infektabwehr schlägt. Es wurde gefunden, dass einige SNPs eine Bedeutung für die Plasmakonzentration besitzen. Damit könnten sie auch einen Effekt auf die prothombotische Wirkung insbesondere bei Patienten mit Phospholipid-Antikörpern besitzen.. ...
Mitochondria do not have IF1 (all mitochondria), and unlike mammals, yeast ones do not have IF3! Also they seem to use mitochondria-specific initiation factor AEP3. Do mammals have AEP3 homologue? Worth checking... To make things more complicated, mitochondria have their own special mRNA-specific factors... and again, there is a catch. Yeast mRNA have long 5 UTRs (untranslated regions), and mammals have short, they basically have leaderless mRNAs (Id love to see a good reference for that! THIS is a little bit out of date...) - therefore I would expect that these mRNA-specific IFs work differently in yeast in mammals. So yeast and mammalian mitochondrial translation seems to have very, very different translational apparatus. Isnt is weird ...
Buy our Tafazzin / TAZ peptide (276-287). Ab45823 is a blocking peptide for ab40004 and has been validated in BL. Abcam provides free protocols, tips and…
August 15, 2012: We consulted with a fertility doctor who specializes in artificial insemination (also called IUI). He ordered a lot of blood work and procedures for me to finish out the diagnosis for my side of things. So far, I know that my estradiol and FSH are good. I also know that I am heterozygous for the MTHFR gene, which is involved with blood clots. That gene puts me at a higher risk for developing blood clots, but it turns out that another of my factors is wonky (will update when I know the name Update: It is the anticardiolipin antibody IGM that is elevated.) is a more significant risk. I also had an x-ray today (8/22) called an HSG. They injected contrast dye into my business and x-rayed to make sure that all my tubes and uterus are open and inviting. Good news--they are. We are still waiting on some of the blood work to come back (including my CF gene testing). Current medications: Prenatal with DHA, Vitamin D, Calcium, MTHFR capsule. Also, he explained why we would need to ...
BSD Medical appoints William S. Barth as Chief Financial Officer and Corporate Secretary. Mr. Barth commenced employment on December 10, 2012 and wil
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Our hematology staff suspect auto-immune pathology or vasculitis that is why they asked for ANCA , Anti cardiolipin IgM and IgG which give the following results +ve Anti-cardiolipin IgM and +ve Cytoplasmic ANCA , so and after consultation of DR/Amal el-beshlawy and DR/Samia salah they decide to start methy-prednisolone at low dose 5 mg/kg/day with gradual tapering but without response also…hematuria persist ...
Kit Component:- KN200329G1, HCLS1 gRNA vector 1 in pCas-Guide vector- KN200329G2, HCLS1 gRNA vector 2 in pCas-Guide vector- KN200329D, donor vector…
By Espen Barth Eide, Special to CNN Editors note: Espen Barth Eide is managing director of the World Economic Forum. This is the first in a series of articles from the World Economic Forum on the key challenges facing the world in 2015 as part of their Outlook on the Global Agenda.
Mitochondrial biogenesis is induced by low temperature in many fish species. For example, cold acclimation of Gasterosteus aculeatus (threespine stickleback) increases mitochondrial densities in oxidative skeletal muscle. Oxidative muscles of Antarctic icefishes (suborder Notothenioidei) also have high mitochondrial densities characterized by higher densities of phospholipids compared to red-blooded notothenioids. Mitochondrial biogenesis has been well studied in mammals yet it is unknown how mitochondrial phospholipid synthesis is regulated. I hypothesized that both activity and mRNA levels of glycerol-3-phosphate acyltransferase (GPAT), the rate-limiting enzyme in glycerolipid biosynthesis, would increase in oxidative muscle of stickleback, where mitochondrial biogenesis occurs, but not in liver, in response to cold acclimation, and that GPAT1 and /or GPAT2 mRNA levels would be higher in hearts of icefishes compared to red-blooded species. To test these hypotheses, maximal activity of GPAT and ...
Dont be scared! There a a lot of us walking around with these antiphospholipid antibodies that never affect our health. It isnt known why in maybe a third of us that has them they suddenly play up. They can even appear in pregnancy and disappear afterwards. Either the anticardiolipin tests or the lupus anticoagulant can be positive at any time or negative. Thats why both tests are usually done together. Levels can vary too just as the body goes about its normal business so both tests need to be done at an interval of at least 6-8 weeks to see if they are constantly present and at what levels. They are not always due to autoimmune disease either. Leverls can be abnormal in infectious or other diseases so often certain tests are also done which indicate if they are autoimmune in ...
This is a study about why some people have certain types of proteins in their blood, called anti-phospholipid antibodies. The presence of these antibodies and associated complications (e.g. blood clots) are known to change over time. The purpose of this study is to evaluate these changes and improve our ability to determine the long-term outcome of affected individuals ...
My GP kindly agreed to Level 1 tests which I had arranged in February to ensure that I had the results before seeing Dr Gorgy. The tests were for lupus anticoagulant, natural killer cells, rheumatoid arthritis, thyroid function, anticardiolipin antibodies, FSH, LH and oestradoil. The last 3 tests are not very useful because they were done on day 13 of my cycle because my GP insisted that it wasnt necessary to do this on days 2 to 4 of cycle as I had requested. Ive been told on the phone that all the tests were normal but I requested copies of the test results which I received yesterday. Looking at the results, if I read it correctly, I think they forgot to send blood specimen for the lupus anticoagulant. WTF?!? Also, it showed a bunch of tests that didnt look anything like the Level 1 immune tests I did previously in 2008 or the named tests. Maybe Dr Gorgy will make some sense of it all ...
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Several weak hits in gapped BLAST to PSGA sequences, e.g. residues 75-210 are 36% similar to PGSA_MYCPN. All similarities begin at around residue 75 of UU364. MG114, CT797, TP0256 and CT496 are predicted phosphatidylglycerophosphate synthases with similarities to UU364 beginning around residue 17 ...
I had an ALCAT test done a couple of years ago, which produced a scoresheet of bad foods for me, semi-bad, somewhat okay and okay ones (my short description), based on some leukocyte examinations und...
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Objective To investigate whether the risk allele for systemic lupus erythematosus (SLE) in the signal transducer and activator of transcription factor 4 (STAT4) gene, defined by the single nucleotide polymorphism (SNP) rs10181656(G), is associated with vascular events and/or presence of prothrombotic anti-phospholipid antibodies (aPL) in patients with SLE.. Methods Two independent groups of unrelated patients with SLE of Swedish ethnicity (n=424 and 154) were genotyped, and occurrence of previous manifestations of ischaemic heart disease (IHD), ischaemic cerebrovascular disease (ICVD) and venous thromboembolic events (VTE) was tabulated. aPL values were measured by ELISA. Matched controls (n=492 and 194) were genotyped.. Results The STAT4 risk allele was more frequent in patients with SLE with previous arterial events (combined OR (ORc)=1.5, 95% CI 1.1 to 2.0) compared to patients without such events. The association was mainly attributable to an accumulation of the risk allele among patients ...
Abstract: The confirmation of diagnosis of the Antiphospholipid Syndrome (APS) relies on laboratory tests. Current classification criteria for definite APS mandate the use of three "standardized" laboratory assays to detect antiphospholipid antibodies (aPL) [viz: anticardiolipin (aCL) IgG and IgM, anti-β(2) glycoprotein I (anti-β(2) GPI) antibodies IgG and IgM and/or a lupus anticoagulant (LAC)], when at least one of the two major clinical manifestations (thrombosis or pregnancy losses) are present. Although International Consensus Guidelines for the determination of LAC have been published and revised, the existence of "standardized" tests for detection of aCL and anti-β(2) GPI has remained elusive. In spite of the publication of several proposals, consensus documents and expert opinions, significant inter-assay and inter-laboratory variation in the results of both aCL and anti-β(2) GPI testing still exists, which affects the consistency of the diagnosis of APS. At the 13(th) International ...
MCLIP : The plasma membranes of mammalian cells are formed from phospholipids. Anionic phospholipids (eg, phosphatidylserine) are found on the cytoplasmic surface and neutral phospholipids (eg, phosphatidylcholine) predominate on the external surface. Membrane phospholipids participate in several important cellular functions including exchanging metabolites across membranes, transferring molecular signals and serving as a platform for the assembly of protein-lipid complexes.(1) Cellular activation is often accompanied by the translocation of anionic phospholipids to the external membrane surface. For example, during platelet-mediated blood coagulation, phosphatidylserine is translocated from the inner platelet membrane and provides a surface for the assembly of the prothrombinase enzyme complex that catalyzes the formation of thrombin.   Complexes of negatively charged (anionic) phospholipids and endogenous plasma proteins provide epitopes recognized by natural autoantibodies.(2) Plasma from
The disclosure relates to a plasma etch chemistry which allows a near perfectly anisotropic etch of silicon. A Cl-containing compound such as HCl has HBr added thereto, readily allowing the anisotropic etching of silicon. This is due to the low volatility of SiBr4. The silicon surface facing the discharge is subjected to ion bombardment, allowing the volatilization (etching) of silicon as a Si-Cl-Br compound. The Br which adsorbs on the sidewalls of the etched silicon passivates them from the etching. This new plasma etch chemistry yields a very smooth etched surface, and the etch rate is relatively insensitive to the electrical conductivity of the silicon.
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Presently, the screening of syphilis is dependent mainly on serological tests. In the sexually transmitted disease clinic, syphilis serology is a basic screening test. The results VDRL test among of 150 HIV (82 males, 68 females) infected, regardless to immune status (CD4+ count), were studied. It was found that in 2 cases, the VDRL was biologically false reactive (VDRL positive, TPHA negative), who had CD4+ count ,200 /L, giving the incidence rate equal to 1.3 % (1.2 % for male and 1.5 % for female). ...
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Antiphospholipid syndrome is an autoimmune disease, in which "antiphospholipid antibodies" (anticardiolipin antibodies and lupus anticoagulant) react against proteins that bind to anionic phospholipids on plasma membranes. Like many autoimmune diseases, it is more common in women than in men. The exact cause is not known, but activation of the system of coagulation is evident. Clinically important antiphospholipid antibodies (those that arise as a result of the autoimmune process) are associated with thrombosis and vascular disease. The syndrome can be divided into primary (no underlying disease state) and secondary (in association with an underlying disease state) forms. Anti-ApoH and a subset of anti-cardiolipin antibodies bind to ApoH, which in turn inhibits Protein C, a glycoprotein with regulatory function upon the common pathway of coagulation (by degradating activated factor V). Lupus anticoagulant (LAC) antibodies bind to prothrombin, thus increasing its cleavage to thrombin, its active ...
Systemic Antiphospholipid Syndrome, Antiphospholipid Syndrome information and help pages for APS patients, doctors and medical professionals Antiphospholipid Syndrome - Systemic Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with typical laboratory abnormalities. These include persistently elevated levels of antibodies directed against membrane anionic phospholipids (ie, anticardiolipin [aCL] antibody, antiphosphatidylserine) or their associated plasma proteins, predominantly beta-2 glycoprotein I (apolipoprotein H), or evidence of a circulating anticoagulant.Multiple terms exist for APS. Unfortunately, some synonyms can be confusing. Lupus anticoagulant (LA) syndrome, for example, is misleading because patients may not ... Antiphospholipid Syndrome - Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with typical laboratory
Systemic Antiphospholipid Syndrome, Antiphospholipid Syndrome information and help pages for APS patients, doctors and medical professionals Antiphospholipid Syndrome - Systemic Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with typical laboratory abnormalities. These include persistently elevated levels of antibodies directed against membrane anionic phospholipids (ie, anticardiolipin [aCL] antibody, antiphosphatidylserine) or their associated plasma proteins, predominantly beta-2 glycoprotein I (apolipoprotein H), or evidence of a circulating anticoagulant.Multiple terms exist for APS. Unfortunately, some synonyms can be confusing. Lupus anticoagulant (LA) syndrome, for example, is misleading because patients may not ... Antiphospholipid Syndrome - Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with typical laboratory
Anticardiolipin antibodies (aCL) derived from the sera of individuals exhibiting the antiphospholipid syndrome (APS) directly bind to beta 2-glycoprotein I (beta 2-GPI), which is adsorbed to an oxidized polystyrene surface. Oxygen atoms were introduced on a polystyrene surface by irradiation with electron or gamma-ray radiation. X-ray photoelectron spectroscopy revealed the irradiated surfaces were oxidized to generate C-O and C = O moieties. aCL derived from either APS patients or (NZW x BXSB)F1 mice bound to beta 2-GPI coated on the irradiated plates, depending on the radiation dose. Antibody binding to beta 2-GPI on the irradiated plates was competitively inhibited by simultaneous addition of cardiolipin (CL)-coated latex beads mixed together with beta 2-GPI but were unaffected by addition of excess beta 2-GPI, CL micelles, or CL-coated latex beads alone. There was a high correlation between binding values of aCL in sera from 40 APS patients obtained by the anti-beta 2-GPI enzyme-linked ...
TY - JOUR. T1 - IgG/IgM antiphospholipid antibodies present in the classification criteria for the antiphospholipid syndrome: a critical review of their association with thrombosis. AU - Kelchtermans, H.. AU - Pelkmans, L.. AU - de Laat, B.. AU - Devreese, K. M.. PY - 2016/8. Y1 - 2016/8. KW - anti-cardiolipin. KW - anti-phospholipid antibody. KW - antiphospholipid syndrome. KW - (2)-glycoprotein I. KW - IgM. U2 - 10.1111/jth.13379. DO - 10.1111/jth.13379. M3 - Article. VL - 14. SP - 1530. EP - 1548. JO - Journal of Thrombosis and Haemostasis. JF - Journal of Thrombosis and Haemostasis. SN - 1538-7933. IS - 8. ER - ...
Antiphospholipid antibody syndrome is a fascinatingly complex disorder. It is an autoimmune disease, whereby the immune system attacks our own tissues. This article summarises the basic mechanisms of the condition and main diseases it is involved in. Learn about the diverse effects antiphospholipid antibody syndrome (APS) can have, from heart attack to miscarriage and how APS can be treated.
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, such as lupus anticoagulant, anticardiolipin antibodies and anti-β2-glycoprotein 1 antibodies. APS can present with a variety of clinical phenotypes, including thrombosis in the veins, arteries and microvasculature as well as obstetrical complications. The pathophysiological hallmark is thrombosis, but other factors such as complement activation might be important. Prevention of thrombotic manifestations associated with APS includes lifestyle changes and, in individuals at high risk, low-dose aspirin. Prevention and treatment of thrombotic events are dependent mainly on the use of vitamin K antagonists. Immunosuppression and anticomplement therapy have been used anecdotally but have not been adequately tested. Pregnancy morbidity includes unexplained recurrent early miscarriage, fetal death and late obstetrical manifestation such as pre-eclampsia, premature birth or fetal ...
PURPOSE OF REVIEW: Laboratory criteria for the classification of antiphospholipid syndrome include the detection of a lupus anticoagulant and/or anticardiolipin and anti-β2-glycoprotein I antibodies. However, the majority of patients who test positive in these assays do not have thrombosis. Current risk-stratification tools are largely limited to the antiphospholipid antibody profile and traditional thrombotic risk factors. RECENT FINDINGS: Novel biomarkers that correlate with disease activity and potentially provide insight into future clinical events include domain 1 specific anti-β2GPI antibodies, antibodies to other phospholipids or phospholipid/protein antigens (such as anti-PS/PT), and functional/biological assays such as thrombin generation, complement activation, levels of circulating microparticles, and annexin A5 resistance ...
A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Barth syndrome
NorthShore encourages patients to utilize our medical library. Read our Antiphospholipid antibody syndrome encyclopedia resources online.
Hepatitis C, a worldwide viral infection, is an important health problem in Brazil. The virus causes chronic infection, provoking B lymphocyte dysfunction, as represented by cryoglobulinemia, non-organ-specific autoantibody production, and non-Hodgkins lymphoma. The aim of this research was to screen for the presence of antiphospholipid autoantibodies in 109 Brazilian hepatitis C virus carriers without clinical history of antiphospholipid syndrome. Forty healthy individuals were used as the control group. IgA, IgG, and IgM antibodies against cardiolipin and β2-glycoprotein I were measured with an enzyme-linked immunosorbent assay, using a cut-off point of either 20 UPL or 20 SBU. While 24 (22.0%) hepatitis C carriers had moderate titers of IgM anticardiolipin antibodies (median, 22.5 MPL; 95%CI: 21.5-25.4 MPL), only three carriers (,3%) had IgG anticardiolipin antibodies (median, 23 GPL; 95%CI: 20.5-25.5 GPL). Furthermore, IgA anticardiolipin antibodies were not detected in these individuals. ...
Antiphospholipid antibodies occur in various clinical states, including the primary antiphospholipid syndrome. Clinical features in these conditions appear to be caused by vasculopathy associated with the presence of these antibodies.. We report the case of a patient with primary antiphospholipid syndrome who experienced cardiac necrosis secondary to myocardial microvasculopathy in the absence of vasculitis. This case demonstrates unequivocally that noninflammatory myocardial microvasculopathy occurs in the primary antiphospholipid syndrome per se without any clinical or immunologic signs of systemic lupus erythematosus or other disease process. The histopathologic findings in the skin and myocardial biopsies showed a noninflammatory vasculopathy characterized by bland thrombi and lack of infiltration of the vessel wall by inflammatory cells. Ultrastructural examination of the myocardial biopsy confirmed the vascular thrombosis and endothelial activation and showed no deposits in basement ...
Outcome of primary antiphospholipid syndrome in childhood. Gattorno, M.; Falcini, F.; Ravelli, A.; Zulian, F.; Buoncompagni, A.; Martini, G.; Resti, M.; Picco, P.; Martini, A. // Lupus;2003, Vol. 12 Issue 6, p449 The objective of this paper is to investigate the long-term outcome of primary antiphospholipid syndrome (APS) in the paediatric age. The features of unselected patients with primary APS who had disease onset before the age of 16 years were retrospectively analysed in three Italian referral... ...
... since β2-glycoprotein-I antibodies are a subset of anti-cardiolipin antibodies, an anti-cardiolipin assay can be performed as a ... Anti-cardiolipin IgG and/or IgM measured by standardized, non-cofactor dependent ELISA on 2 or more occasions, not less than 12 ... Anti-cardiolipin antibodies can be detected using an enzyme-linked immunosorbent assay (ELISA) immunological test, which ... Anti-ApoH and a subset of anti-cardiolipin antibodies bind to ApoH, which in turn inhibits Protein C, a glycoprotein with ...
Anti-cardiolipin. Anti-cytoplasm antibody. *Anti-neutrophil cytoplasmic *C-ANCA. *P-ANCA ...
Anti-cardiolipin. Anti-cytoplasm antibody. *Anti-neutrophil cytoplasmic *C-ANCA. *P-ANCA ...
Nowicki M, Müller F, Frentzen M (April 2005). "Cardiolipin synthase of Arabidopsis thaliana". FEBS Letters. 579 (10): 2161-5. ... Cardiolipins are a subclass of glycerophospholipids containing four acyl chains and three glycerol groups that are particularly ... "Cardiolipins and biomembrane function" (PDF). Biochimica et Biophysica Acta. 1113 (1): 71-133. doi:10.1016/0304-4157(92)90035- ...
... and cardiolipin is oxidized by a peroxidase function of the cardiolipin-cytochrome c complex. The hemoprotein is then detached ... Orrenius S, Zhivotovsky B (Sep 2005). "Cardiolipin oxidation sets cytochrome c free". Nature Chemical Biology. 1 (4): 188-9. ... Cytochrome c binds to cardiolipin in the inner mitochondrial membrane, thus anchoring its presence and keeping it from ... While the initial attraction between cardiolipin and cytochrome c is electrostatic due to the extreme positive charge on ...
A membrane lipid cardiolipin is exclusively found in the inner mitochondrial membrane and bacterial cell membranes.[58] ... that cardiolipin is found only in the inner mitochondrial membrane and bacterial cell membranes; and that some mitochondria and ... "Cardiolipin membrane domains in prokaryotes and eukaryotes". Biochim Biophys Acta. 1788 (10): 2084-2091. doi:10.1016/j.bbamem. ...
M. Nowicki (2006). "Characterization of the Cardiolipin Synthase from Arabidopsis thaliana". Ph.D. thesis, RWTH-Aachen ... "Cardiolipin synthase of Arabidopsis thaliana". FEBS Letters. 579 (10): 2161-2165. doi:10.1016/j.febslet.2005.03.007. PMID ... of the inner mitochondrial membrane where it is thought to be involved in the synthesis of phosphatidylglycerol and cardiolipin ...
Other members of this family are able hydrolyze other phospholipid substrates, such as cardiolipin, or even the phosphodiester ... M. Nowicki; M. Frentzen (2005). "Cardiolipin synthase of Arabidopsis thaliana". FEBS Letters. 579 (10): 2161-2165. doi:10.1016/ ... M. Nowicki (2006). "Characterization of the Cardiolipin Synthase from Arabidopsis thaliana". Ph.D. thesis, RWTH-Aachen ... PLD5 PC-hydrolyzing PLD is a homologue of cardiolipin synthase, phosphatidylserine synthase, bacterial PLDs, and viral proteins ...
... and its overexpression in cultured cells resulted in increased cardiolipin synthase, activity. As cardiolipin is synthesized in ... The active t-Bid fragment is shown to localize to mitochondria through a positive interaction with cardiolipin. This activated ... M. Nowicki (2006). "Characterization of the Cardiolipin Synthase from Arabidopsis thaliana". Ph.D. thesis, RWTH-Aachen ... Recent findings suggest that PLSCR3 is involved in regulation of biosynthesis of cardiolipin in mitochondria, ...
2003). "Only one splice variant of the human TAZ gene encodes a functional protein with a role in cardiolipin metabolism". J. ... 2008). "The Cardiolipin Transacylase, Tafazzin, Associates with Two Distinct Respiratory Components Providing Insight into ... 2009). "The enigmatic role of tafazzin in cardiolipin metabolism". Biochim. Biophys. Acta. 1788 (10): 2003-14. doi:10.1016/j. ... It is involved in the metabolism of cardiolipin. Tafazzin functions as a phospholipid-lysophospholipid transacylase. The ...
A role for cardiolipin". The Journal of Biological Chemistry. 278 (34): 31861-70. doi:10.1074/jbc.m300190200. PMID 12805375.. ...
One of its functions is to bind cardiolipin. When bound the structure of cardiolipin and Apo-H both undergo large changes in ... Anti-cardiolipin antibodies are found in both infectious (syphilis) and autoimmune disease (sclerosis, lupus). The activity of ... "Interactions and molecular structure of cardiolipin and beta 2-glycoprotein 1 (beta 2-GP1)". Clin. Exp. Immunol. 102 (2): 373-8 ... anti-cardiolipin antibodies in autoimmune antiphospholipid syndrome requires apolipoprotein H. The subset of antibodies that ...
The main phospholipids are phosphatidylethanolamine, phosphatidylglycerol, and cardiolipin. Ectoine, glutamate, and, to a ...
Identify via agglutination with cardiolipin sensitive chicken erythrocytes. RCN infected Strain 143 human osteosarcoma cells ...
In addition it contains cardiolipin and phosphatidylcholine as the major phospholipids, while sterols are absent. Cardiolipin ...
Nowicki M, Müller F, Frentzen M (April 2005). "Cardiolipin synthase of Arabidopsis thaliana". 》FEBS Letters》 579 (10): 2161-5. ... "Cardiolipins and biomembrane function" (PDF). 》Biochimica et Biophysica Acta》 1113 (1): 71-133. doi:10.1016/0304-4157(92)90035 ...
Cardiolipin is intimately connected with the electron transport chain proteins and the membrane structure of the mitochondria ... Kulik found that all the BTHS individuals that he tested had abnormalities in their cardiolipin molecules, a lipid found inside ... Claypool SM, Boontheung P, McCaffery JM, Loo JA, Koehler CM (December 2008). "The cardiolipin transacylase, tafazzin, ... December 2000). "Defective remodeling of cardiolipin and phosphatidylglycerol in Barth syndrome". Biochemical and Biophysical ...
Phosphatidylcholine makes up about 26.5%, cardiolipin 25.4%, and phosphatidylinositol 4.5%. In S. cerevisiae mitochondria, ... cardiolipin 16.1%, phosphatidylserine 3.8%, and phosphatidic acid 1.5%. In the inner mitochondrial membrane, the protein-to- ...
Although MLCL AT-1 and MTP are different proteins, in 2012 the same lab discovered that MTP did indeed have cardiolipin ... This suggests a possible link between mitochondrial membrane cardiolipin content and beta oxidation. Disorders are associated ... "Human Trifunctional Protein Alpha Links Cardiolipin Remodeling to Beta-Oxidation". PLoS ONE. 7 (11): e48628. doi:10.1371/ ... linking MTP to the remodeling of cardiolipin from monolysocardiolipin. ...
... into cardiolipin. In 1990, biologists Michael Schlame and Bernd Rustow observed the deacylation of cardiolipin into MLCL, which ... producing cardiolipin. This lipid remodeling is separate from the cardiolipin synthesis pathway, and is essential to maintain ... Since cardiolipin content is significantly diminished in the inner mitochondrial membrane during apoptosis, the increase of ... "Cardiolipin biosynthesis and remodeling enzymes are altered during development of heart failure". The Journal of Lipid Research ...
... forms a complex with prohibitins to regulate cardiolipin remodeling". Cell Metabolism. 20 (1): 158-71. doi:10.1016/j.cmet. ... TIM14 helps regulate mitochondrial morphology by complexing with prohibitins to perform disphosphoglycerolipid cardiolipin (CL ...
... and in particular requires the presence of cardiolipin, a unique mitochondrial lipid. In yeast mitochondria lacking cardiolipin ... Zhang, M. (2005). "Cardiolipin Is Essential for Organization of Complexes III and IV into a Supercomplex in Intact Yeast ... 2009), cardiolipin stabilizes the supercomplex formation by neutralizing the charges of lysine residues in the interaction ... CARDIOLIPIN IS REQUIRED FOR SUPERCOMPLEX FORMATION IN THE INNER MITOCHONDRIAL MEMBRANE". Journal of Biological Chemistry. 277 ( ...
Two phosphatidylglycerols form cardiolipin, the constituent molecule of the mitochondrial inner membrane. Glycerol Cardiolipin ...
This is a family of proteins that acts as a mitochondrial phosphatase in cardiolipin biosynthesis. Cardiolipin is a unique ... "A mitochondrial phosphatase required for cardiolipin biosynthesis: the PGP phosphatase Gep4". The EMBO Journal. 29 (12): 1976- ...
The envolope contains a variety of lipids including cardiolipins, phosphatidylglycerols, phosphatidylglycerophosphate methyl ...
ALCAT1 is upregulated by oxidative stress and diet-induced obesity, and produces a species of cardiolipin that is highly ... Cao, J. (2004). "A Novel Cardiolipin-remodeling Pathway Revealed by a Gene Encoding an Endoplasmic Reticulum-associated Acyl- ... "Cardiolipin Remodeling by ALCAT1 Links Oxidative Stress and Mitochondrial Dysfunction to Obesity". Cell Metabolism. 12 (2): 154 ... of the endoplasmic reticulum which is primarily known for catalyzing the acylation of monolysocardiolipin back into cardiolipin ...
Cardiolipin Abnormalities. A failure of BTHS mitochondria to make adequate amounts of tetralinoleoyl-cardiolipin, an essential ...
Cardiolipin antibody tests help diagnose the cause of blood clots, recurrent miscarriages or antiphospholipid syndrome (APS). ... Cardiolipin (anticardiolipin) antibodies are autoantibodies that can affect blood clotting. ... Cardiolipin antibodies are autoantibodies produced by the immune system that mistakenly target the bodys own cardiolipins, ... Cardiolipin antibodies target cardiolipins and are associated with an increased risk of developing recurrent inappropriate ...
... has displayed the importance of cardiolipin in mitochondrial functionality. Cardiolipin effects mitochondrial bioenergetics by ... Changes in cardiolipin concentrations and alterations to its acyl groups have led to a variety of pathological conditions such ... Specifically, interaction of cardiolipin with cytochrome oxidase and adenine nucleotide translocase is required in order for ... a disease associated with abnormalities in the fatty acid composition of cardiolipin causing cardiomyopathy and neutropenia, ...
cardiolipin synonyms, cardiolipin pronunciation, cardiolipin translation, English dictionary definition of cardiolipin. n. A ... cardiolipin. Also found in: Medical, Encyclopedia, Wikipedia.. Related to cardiolipin: Lupus anticoagulant ... NeoPharm uses a proprietary synthetic form of cardiolipin. In its natural form, cardiolipin occurs as a negatively charged ... Cardiolipin - definition of cardiolipin by The Free Dictionary https://www.thefreedictionary.com/cardiolipin ...
Cardiolipin_synth_ClsA (MF_00190). Accession MF_00190 Integration. Cardiolipin synthase A (IPR030840) Member database. HAMAP. ...
... ,ARUP Laboratories is a national reference laboratory and a worldwide leader in innovative ... Anti-Cardiolipin Antibodies, IgG & IgM. 2. Anti-Cardiolipin Antibody, IgG. 3. Adenovirus Antibody, IgG. 4. Adenovirus Antibody ...
C. Ye, Z. Shen, and M. L. Greenberg, "Cardiolipin remodeling: a regulatory hub for modulating cardiolipin metabolism and ... The Role of Cardiolipin in Cardiovascular Health. Zheni Shen,1 Cunqi Ye,1,2 Keanna McCain,1 and Miriam L. Greenberg1 ... A. S. Joshi, J. Zhou, V. M. Gohil, S. Chen, and M. L. Greenberg, "Cellular functions of cardiolipin in yeast," Biochimica et ... Figure 1: Cardiolipin synthesis and remodeling pathway in humans and yeast. Phosphatidic acid (PA) is converted to CDP- ...
The ImmunoWELL Cardiolipin Antibody (IgM) Test is an enzyme immunoassay (EIA) for the quantitative detection of antibodies to ... cardiolipin antigen in serum and is used as an aid for assessing the risk of thrombosis in individuals with systemic lupus ... ImmunoWELL Cardiolipin Antibody (IgM) Test. Features. *ImmunoWELL products use the industry standard microtiter plate format. * ... ImmunoWELL Cardiolipin Antibody (IgG) Test. 2. ImmunoWELL TPO IgG Quantitative Test. 3. ImmunoWELL Thyroglobulin IgG ...
Cardiolipins have multiple vital functions within biological cell membranes, most notably in the energy metabolism associated ... Considering their essential role, peroxidation of cardiolipins may plaus... ... How cardiolipin peroxidation alters the properties of the inner mitochondrial membrane?. *Vähäheikkilä M ... Cardiolipins have multiple vital functions within biological cell membranes, most notably in the energy metabolism associated ...
Cardiolipin from a cow heart is used as an antigen in the Wassermann test for syphilis. Anti-cardiolipin antibodies can also be ... 2009;23(4):210-2. Cardiolipin at the US National Library of Medicine Medical Subject Headings (MeSH) Cardiolipin ( ... Phospholipase D (PLD) in the mitochondrion hydrolyses cardiolipin to phosphatidic acid. Because of cardiolipins unique ... The enzyme can operate in reverse under some physiological conditions to remove cardiolipin. Catabolism of cardiolipin may ...
Cardiolipins Are Biomarkers of Mitochondria-Rich Thyroid Oncocytic Tumors. Jialing Zhang, Wendong Yu, Seung Woo Ryu, John Lin, ... Cardiolipins Are Biomarkers of Mitochondria-Rich Thyroid Oncocytic Tumors. Jialing Zhang, Wendong Yu, Seung Woo Ryu, John Lin, ... Cardiolipins Are Biomarkers of Mitochondria-Rich Thyroid Oncocytic Tumors. Jialing Zhang, Wendong Yu, Seung Woo Ryu, John Lin, ... Cardiolipin fingerprinting of leukocytes by MALDI-TOF/MS as a screening tool for Barth syndrome. J Lipid Res 2015;56:1787-94. ...
Anti-cardiolipin antibodies (ACA) are antibodies often directed against cardiolipin and found in several diseases, including ... Anti-cardiolipin antibodies can be classified in two ways: As IgM, IgG or IgA As β2-glycoprotein dependent or independent In ... In SLE, anti-DNA antibodies and anti-cardiolipin antibodies may be present individually or together; the two types of ... Only a subset of autoimmune anti-cardiolipin antibodies bind Apo-H, these anti-apolipoprotein antibodies are associated with ...
Cardiolipin antibodies are associated with the lupus inhibitor, beta-2-glycoprotein1 antibodies, and with false positive ... Moderate to high levels of IgG cardiolipin antibody are seen in the Phospholipid antibody syndrome, but the antibody may also ...
We have utilized bovine heart cardiolipin as well as commercially available tetralinoleoyl cardiolipin oxidized with cytochrome ... 2D-LC-MS analysis of oxidized lipid species: application to the study of cardiolipin in apoptosis and damaged tissue.. ... We identified oxidized linoleic acyl chains (50%) as the preferred moieties for oxidation in cardiolipin, followed by ... to separate and identify cardiolipin oxidation products, critical to the cell death pathways in these tissues following ...
... cardiolipin (10). In light of these observations, we assayed 2F5 and 4E10 mAbs, along with other mAbs to HIV-1, for cardiolipin ... Unlike antibodies to cardiolipin found in SLE, "infectious" antibodies to cardiolipin, such as those found in HIV-1 infection, ... Cardiolipin HEp-2 cell reactivity Ro (SSA) dsDNA Centromere B Histones Membrane-proximal external region (2F5) 47 +Cytoplasmic ... expressing the 4E10 epitope inhibit binding of 4E10 mAb to cardiolipin. The IC50 of inhibition of 4E10 binding to cardiolipin ...
Cardiolipin antibodies (IgG, IgM, IgA), anticardiolipin What is this test?. A cardiolipin antibodies test looks for a certain ... When high levels of cardiolipins are found in people with these or other issues, it is known as cardiolipin antibody syndrome. ... The results of a cardiolipin antibodies test are easy to understand. If youre negative for cardiolipin antibodies, that is ... They are antibodies that form in response to cardiolipins. Cardiolipin is a phospholipid, or a kind of fat in the blood. The ...
The most commonly used phospholipid substrate is cardiolipin. The term phospholipid antibody is actually a misnomer. The ... These autoantibodies are called phospholipid or cardiolipin antibodies when they are detected by immunoassays that employ ... and most immunoassays for phospholipid antibodies employ a composite substrate consisting of cardiolipin plus beta-2 GP1. Beta- ... An IgA phospholipid (cardiolipin) antibody result above 15 APL with negative IgG and IgM phospholipids (cardiolipin) antibody ...
Binding of Cardiolipin to c-Rings.. Cardiolipin, in strong preference to phosphatidyl lipids, became bound to native c8-rings ... Role of Cardiolipin Molecules in ATP Synthase.. The sites where cardiolipins are bound permanently to complexes III and IV and ... 1). Cardiolipin remained longer in the inner annulus than phosphatidyl lipids. Once a cardiolipin entered the lipid annulus in ... Each gray line represents a cardiolipin, and colored bars along the line indicate when a cardiolipin phosphate bound to a c- ...
... a disorder of cardiolipin metabolism) induces mitochondrial dysfunction and cardiomyopathy, and since decreases in cardiolipin ... Shotgun lipidomics identifies cardiolipin depletion in diabetic myocardium linking altered substrate utilization with ... cardiolipin, in diabetic murine myocardium (from 7.2 +/- 0.3 nmol/mg of protein in control hearts to 3.1 +/- 0.1 nmol/mg of ... cardiolipin depletion, mitochondrial dysfunction, and resultant hemodynamic compromise. ...
Cardiolipin is an inner mitochondrial membrane phospholipid. We found that rotenone, staurosporine, 6-hydroxydopamine and other ... pro-mitophagy stimuli caused externalization of cardiolipin to the mitochondrial surface in primary cortical neurons and SH ... RNAi knockdown of cardiolipin synthase or of phospholipid scramblase-3, which transports cardiolipin to the outer mitochondrial ... Cardiolipin externalization to the outer mitochondrial membrane acts as an elimination signal for mitophagy in neuronal cells. ...
Anti-Cardiolipin Antikörper (IgG in GPL-U/ml und IgM in MPL-U/ml) ... PBL Fluoroenzyme-Immunoassay FOR ANTI Cardiolipin - ANTIBODIES IgM Phadia GmbH 250-5530-021. ... PBL Fluoroenzyme-Immunoassay FOR ANTI Cardiolipin - ANTIBODIES IgG Phadia GmbH 250-5529-021 ...
Catalyzes the phosphatidyl group transfer from one phosphatidylglycerol molecule to another to form cardiolipin (CL) ( ... Cardiolipin synthase BAdd BLAST. 413. Proteomic databases. PaxDb, a database of protein abundance averages across all three ... Cardiolipin synthase subfamily. ClsB sub-subfamily.UniRule annotation. Manual assertion according to rulesi ... 2 1,2-diacyl-sn-glycero-3-phospho-(1-sn-glycerol) = a cardiolipin + glycerol.UniRule annotation. Manual assertion according to ...
To determine whether cardiolipin is essential for yeast growth, we generated a cardiolipin synthase null mutant by disrupting ... Biochemical analysis of the mutant indicated that it had no cardiolipin synthase activity and no cardiolipin in its membranes. ... Cardiolipin is not essential for the growth of Saccharomyces cerevisiae on fermentable or non-fermentable carbon sources.. ... Cardiolipin is a unique dimeric phospholipid, which is present throughout the eukaryotic kingdom and is specifically localized ...
Cardiolipin is a mitochondria-specific phospholipid that is located mainly in the IM (Li et al., 2007). Cardiolipin contains ... Ups1p and Ups2p control the level of cardiolipin in mitochondria. A recent study has shown that cardiolipin is required for the ... In this study, we showed that Ups1p and Ups2p control the level of cardiolipin in mitochondria. Cardiolipin is a unique ... 8, the level of cardiolipin was considerably reduced in ups1Δ mitochondria. This decreased cardiolipin level was rescued by ...
Catalyzes the reversible phosphatidyl group transfer from one phosphatidylglycerol molecule to another to form cardiolipin (CL ... IPR022924 Cardiolipin_synthase. IPR030840 CL_synthase_A. IPR027379 CLS_N. IPR025202 PLD-like_dom. IPR001736 PLipase_D/ ... Cardiolipin synthase subfamily. ClsA sub-subfamily.UniRule annotation. Manual assertion according to rulesi ... 2 1,2-diacyl-sn-glycero-3-phospho-(1-sn-glycerol) = a cardiolipin + glycerol.UniRule annotation. Manual assertion according to ...
  • Interestingly, although all three compounds increased the generation of reactive oxygen species and decreased phosphorylation of extracellular signal-regulated kinase, only cis -guggulsterone induced a rapid depletion of reduced glutathione levels and oxidation of the mitochondrial phospholipid cardiolipin. (aacrjournals.org)
  • In addition, the analysts of the report have served the qualitative and quantitative scrutinizing of different micro- and macro-economic factors influencing the global Cardiolipin Antibodies Testmarket. (pharmiweb.com)
  • ELISA method for quantitative determination of Anti-Cardiolipin / Anti-Phospholipid IgG, IgM, IgA isotypes. (aniara.com)
  • The amount of bound Mouse ACA-IgM (Anti-Cardiolipin IgM) is proportional to the signal generated by the reaction meaning the kit assay gives you a quantitative measurement of the analyte in your samples. (elisagenie.com)
  • Barth Syndrome is the only known Mendelian disorder of cardiolipin remodeling, with characteristic clinical features of cardiomyopathy, skeletal myopathy, and neutropenia. (kennedykrieger.org)
  • A full screen for hyperviscosity conditions including plasma homocysteine levels, anti-DNA antibodies, cardiolipin antibodies, protein C and protein S, factor V Leiden and prothrombin 20210A was normal, as were the blood glucose level and a lipogram. (thefreedictionary.com)
  • As there are four distinct alkyl chains in cardiolipin, the potential for complexity of this molecule species is enormous. (wikipedia.org)
  • However, in most animal tissues, cardiolipin contains 18-carbon fatty alkyl chains with 2 unsaturated bonds on each of them. (wikipedia.org)
  • To this end, we explored what happens when varying fractions of fatty acid chains of cardiolipin are replaced by its four different oxidized products in systems modeling the inner mitochondrial membrane. (mendeley.com)
  • Cardiolipin consists of two 3-phosphatidyl groups linked by a glycerol bridge, and in bovine mitochondria, the four acyl chains have 18 carbon atoms with one or two unsaturated linkages ( 4 ). (pnas.org)
  • Cardiolipin, a unique phospholipid composed of four fatty acid chains, is located mainly in the mitochondrial inner membrane (IM). (rupress.org)
  • Although the total amount of cardiolipin was unchanged, we disclosed in retinal samples increased amount of the major specie of cardiolipins, i.e. cardiolipins containing 18-carbon fatty alkyl chains with two unsaturated bonds on each of them (0.213±0.015 vs 0.405±0.13). (arvojournals.org)
  • Biochemical abnormalities include decreased levels of the mitochondrial phospholipid cardiolipin, increased levels of monolysocardiolipin, and a lower degree of unsaturation of the (monolyso)cardiolipin acyl chains. (ru.nl)
  • BioVision's Cardiolipin Assay Kit (K944-100) is a fluorometric assay that makes use of a proprietary probe that binds specifically to Cardiolipin but not with any other lipids such as to Phosphatidylcholine and Sphingomyelin, thus making it highly specific. (eprnews.com)
  • Expression of monolysocardiolipin acyltransferase activity is regulated in concert with the level of cardiolipin and cardiolipin biosynthesis in the mammalian heart. (sigmaaldrich.com)
  • Monolysocardiolipin acyltransferase (MLCL AT) catalyzes the acylation of monolysocardiolipin to cardiolipin in mammalian tissues. (sigmaaldrich.com)
  • While the primary biochemical defects of reduced mature cardiolipin and increased monolysocardiolipin are well-described, much of the downstream biochemical dysregulation has not been uncovered, and biomarkers are limited. (kennedykrieger.org)
  • Cardiolipin and monolysocardiolipin analysis in fibroblasts, lymphocytes, and tissues using high-performance liquid chromatography-mass spectrometry as a diagnostic test for Barth syndrome. (ru.nl)
  • Because a validated confirmatory method is not yet available, we set up and validated a high-performance liquid chromatography-mass spectrometry (HPLC-MS) method for BTHS in cultured fibroblasts, lymphocytes, and skeletal muscle based on cardiolipin, monolysocardiolipin, and the monolysocardiolipin/cardiolipin ratio. (ru.nl)
  • To determine whether cardiolipin is essential for yeast growth, we generated a cardiolipin synthase null mutant by disrupting the CLS1 gene (open reading frame YDL142c on chromosome IV) of Saccharomyces cerevisiae. (nih.gov)
  • In silico analysis of the M. hyorhinis genome revealed the absence of the pld gene but the presence of the cls gene encoding cardiolipin synthetase, which contains two PLD active domains. (asm.org)
  • The transformation of Mycoplasma gallisepticum that has neither the cls gene nor the reverse CAMP phenomenon with the cls gene from M. hyorhinis resulted in the reverse CAMP phenomenon, suggesting for the first time that reverse CAMP can be induced by cardiolipin synthetase. (asm.org)
  • The grant proposal entitled A mouse model of Barth syndrome, a mitochondrial cardiolipin disorder is designed to study molecular mechanisms of pathogenesis of Barth syndrome, a rare genetic pediatric disorder, caused by mutations in the tafazzin gene. (grantome.com)
  • The mAbs 2F5 and 4E10, two additional rare broadly reactive neutralizing mAbs (2G12 and IgG1b12) ( 11 - 13 ), and 31 common human mAbs to HIV-1 Env were tested for reactivity with cardiolipin ( 14 ) ( Table 1 ). (sciencemag.org)
  • The identification of Barth′s syndrome, a disease associated with abnormalities in the fatty acid composition of cardiolipin causing cardiomyopathy and neutropenia, has displayed the importance of cardiolipin in mitochondrial functionality. (sigmaaldrich.com)
  • In addition, we performed retrospective analysis of 121 muscle samples of patients with myopathy of which mitochondrial origin was presumed, and we identified one patient with cardiolipin abnormalities similar to BTHS patients. (ru.nl)
  • Specifically, interaction of cardiolipin with cytochrome oxidase and adenine nucleotide translocase is required in order for both of these mitochondrial enzymes to maintain activity. (sigmaaldrich.com)
  • Mutation of LC3 residues predicted as cardiolipin-interaction sites by computational modelling inhibited its participation in mitophagy. (cdc.gov)
  • Our findings indicate that the Mic10 core machinery of MICOS is regulated by several mechanisms, including interaction with cardiolipin and antagonistic actions of Mic26 and Mic27. (rug.nl)
  • We found that the oxidation of cardiolipin leads to a conformational change both in the backbone/head group and in chain regions of oxidized cardiolipin molecules. (mendeley.com)
  • The acyl chain conformational order of unoxidized lipids exposed to interactions with oxidized cardiolipins was increased in carbons 3-5 and decreased in carbons 13-17 due to the structural reorganization of the cardiolipin molecules. (mendeley.com)
  • In computer simulations of hydrated membranes containing trimethylated or unmethylated bovine c 8 -rings and bacterial c 10 - or c 11 -rings, the head-groups of cardiolipin molecules became associated selectively with these modified and unmodified lysine residues and with adjacent polar amino acids and with a second conserved lysine on the opposite side of the membrane, whereas phosphatidyl lipids were attracted little to these sites. (pnas.org)
  • In mammalian cells, but also in plant cells, cardiolipin (CL) is found almost exclusively in the inner mitochondrial membrane where it is essential for the optimal function of numerous enzymes that are involved in mitochondrial energy metabolism. (wikipedia.org)
  • Cardiolipins have multiple vital functions within biological cell membranes, most notably in the energy metabolism associated with the inner mitochondrial membrane. (mendeley.com)
  • In this study, we report that two homologous intermembrane space proteins, Ups1p and Ups2p, control cardiolipin metabolism and affect the assembly state of TIM23 and its association with PAM in an opposing manner. (rupress.org)
  • Our findings provide a novel molecular mechanism for the regulation of cardiolipin metabolism. (rupress.org)
  • Retinal cardiolipin metabolism is altered in a mouse model of Dominant Optic Atrophy. (arvojournals.org)
  • Thus, here we tested the hypothesis of retinal cardiolipin metabolism modification in mitochondria responsible for these respiratory chain alterations. (arvojournals.org)
  • Thus, our results showed that Opa1 haploinsufficiency impacts cardiolipin metabolism in the retina. (arvojournals.org)
  • Growing evidence links phospholipid alterations especially cardiolipins (CLs) to defects in mitochondrial function and energy metabolism in heart failure. (biomedcentral.com)
  • We used atomistic molecular dynamics simulations to study how peroxidation of cardiolipin affects the properties of the inner mitochondrial membrane. (mendeley.com)
  • We identified a novel molecular signature of oncocytic tumors characterized by an abnormally high abundance and chemical diversity of cardiolipins (CL), including many oxidized species. (aacrjournals.org)
  • The inner mitochondrial membrane of eukaryotic cells has a complex structure and molecular composition, recognized by the presence of cardiolipins (CL), a unique class of anionic glycerophospholipid located predominantly (if not exclusively) in mitochondria. (aacrjournals.org)
  • Cardiolipin externalization to the outer mitochondrial membrane acts as an elimination signal for mitophagy in neuronal cells. (cdc.gov)
  • We found that rotenone, staurosporine, 6-hydroxydopamine and other pro-mitophagy stimuli caused externalization of cardiolipin to the mitochondrial surface in primary cortical neurons and SH-SY5Y cells. (cdc.gov)
  • It has been proposed that under physiological conditions, the central hydroxyl and the two phosphates trap a proton in a resonance structure and that cardiolipin carries one net negative charge ( 5 ). (pnas.org)
  • However, re-evaluations of the pK a values of the phosphates indicate that under physiological conditions, the head-group of cardiolipin bears two negative charges ( 6 , 7 ). (pnas.org)
  • Changes in cardiolipin concentrations and alterations to its acyl groups have led to a variety of pathological conditions such as heart failure. (sigmaaldrich.com)