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-15:0/i-21:0) 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-15:0/i-21:0) contains one chain of 10-methylundecanoic acid at the C1 position, one chain of 22-methyltricosanoic acid at the C2 position, one chain of 13-methyltetradecanoic acid at the C3 position, one chain of 19-methyleicosanoic 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 ...
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 ...
TY - JOUR. T1 - Lysocardiolipin acyltransferase regulates TGF-β mediated lung fibroblast differentiation. AU - Huang, Long Shuang. AU - Jiang, Peiyue. AU - Feghali-Bostwick, Carol. AU - Reddy, Sekhar P.. AU - Garcia, Joe G.N.. AU - Natarajan, Viswanathan. PY - 2017/11. Y1 - 2017/11. N2 - Lysocardiolipin acyltransferase (LYCAT), a cardiolipin remodeling enzyme, plays a key role in mitochondrial function and vascular development. We previously reported that reduced LYCAT mRNA levels in peripheral blood mononuclear cells correlated with poor pulmonary function outcomes and decreased survival in IPF patients. Further LYCAT overexpression reduced lung fibrosis, and LYCAT knockdown accentuated experimental pulmonary fibrosis. NADPH Oxidase 4 (NOX4) expression and oxidative stress are known to contribute to lung fibroblast differentiation and progression of fibrosis. In this study, we investigated the role of LYCAT in TGF-β mediated differentiation of human lung fibroblasts to myofibroblasts, and ...
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
CL(14:0/14:0/18:1(11Z)/22:0) is a cardiolipin (CL). Cardiolipins are sometimes called a double phospholipid because they have four fatty acid tails, instead of the usual two. CL(14:0/14:0/18:1(11Z)/22:0) contains two chains of tetradecanoic acid at the C1 and C2 positions, one chain of (11Z-octadecenoyl) at the C3 position, one chain of docosanoic acid at the C4 position. While the theoretical charge of cardiolipins is -2, under normal physiological conditions (pH near 7), the molecule may carry only one negative charge. In prokaryotes such as E. coli, the enzyme known as diphosphatidylglycerol synthase catalyses the transfer of the phosphatidyl moiety of one phosphatidylglycerol to the free 3-hydroxyl group of another, with the elimination of one molecule of glycerol. In E. coli, which acylates its glycerophospholipids with acyl chains ranging in length from 12 to 18 carbons and possibly containing an unsaturation, or a cyclopropane group more than 100 possible CL molecular species are ...
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 ...
All-trans-retinoic-acid (ATRA) is a promising agent in the prevention/treatment of breast-cancer. There is growing evidence that reprogramming of cellular lipid metabolism contributes to malignant transformation and progression. Lipid metabolism is implicated in cell differentiation and metastatic colonization and it is involved in the mechanisms of sensitivity/resistance to different anti-tumor agents. The role played by lipids in the anti-tumor activity of ATRA has never been studied. We used 16 breast cancer cell-lines whose degree of sensitivity to the anti-proliferative action of ATRA is known. We implemented a non-oriented mass-spectrometry based approach to define the lipidomic profiles of each cell-line grown under basal conditions and following treatment with ATRA. To complement the lipidomic data, untreated and retinoid treated cell-lines were also subjected to RNA-sequencing to define the perturbations afforded by ATRA on the whole-genome gene-expression profiles. The number and functional
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 ...
After obtaining a skin sample from a patient with Barth syndrome, Pu was able to make stem cells from the patients skin cells, which he then turned into heart muscle cells. What was once skin became a beating heart muscle.. Next, Pu worked with Kevin Kit Parkers bioengineering group at the Wyss Institute for Biologically Inspired Engineering to assemble the muscle cells on a tiny, flexible plastic film that instructs the cells to line up and form organized muscle fibers. When the cells are assembled into this "heart-on-a-chip" format, they are able to work together so their beating bends the plastic film. The amount of force the patients heart muscle generates can be measured by how much the sheet bends with each beat. Comparing a Barth syndrome patients sheet with one from a patient with a normally functioning heart further revealed that the Barth heart muscle cells were very weak compared to normal heart muscle cells. Thus, combining the stem cell and "heart-on-a-chip" technologies allowed ...
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 ...