Our study has confirmed that the most important criterion for the detection of MCAD deficiency is the presence in the blood spot of octanoylcarnitine at a concentration , 0.3 μM (in this study , 0.38 μM). However, we have also shown that blood spot octanoylcarnitine concentrations are higher in neonates with MCAD deficiency and that there is an association between low octanoylcarnitine and low free carnitine. There are two possible explanations for the latter association. The first is that the volume of blood in the 6 mm disc was substantially less than 10 μl or that the elution was much less efficient for this group of blood spots. We consider this to be very unlikely; all the Guthrie cards that were received were made from approved brands of filter paper, all blood spots were inspected visually to make sure that the 6 mm disc was completely filled, and a standardised procedure was adopted for the elution step. The second and more likely explanation for the association is that these patients ...
Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare genetic condition resulting from a mutation (change) in a person's DNA. Due to this change, people with VLCAD have problems breaking down certain fats properly. VLCAD occurs when the body either does not make enough or makes non-working enzyme called very long-chain acyl-CoA dehydrogenase. Enzymes are proteins that help break down the food we eat into the pieces our body can use for energy. In this case, the job of the VLCAD enzyme is to break down the very long-chain fatty acids, which are parts of the fat from our food. These fatty acids are important energy sources when there are not enough sugars in the body, such as in between meals. A person with VLCAD cannot use this type of fatty acid for energy because it can't break it down. This also causes a build-up of too many unused very long-chain fatty acids, which can be harmful to the body.. Those affected by VLCAD can show symptoms any time between infancy and adulthood. ...
Amino Acid Disorders. Phenylketonuria (PKU). Maple Syrup Urine Disease (MSUD). Homocystinuria Tyrosinemia Type I. Tyrosinemia Type II. Tyrosinemia Type III. Argininosuccinic Aciduria Citrullinemia (Argininosuccinic Synthetase Deficiency). Argininemia. Histidinemia. Hyperornithinemia. Hyper/Hypomethioninemia. 5-Oxoprolinuria (Pyroglutamic Aciduria). Fatty Acid Disorders. Carnitine Palmytoyltransferase Deficiency Type I. Carnitine Palmytoyltransferase Deficiency Type II. Carnitine / Acylcarnitine Translocase Deficiency (CACT). Carnitine Uptake Deficiency (CUD). Trifunctional Protein Deficiency. Very Long Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD). Long Chain 3-Hydroxy Acyl -CoA Dehydrogenase Deficiency (LCHADD). Medium Chain Acyl - CoA Dehydrogenase Deficiency (MCADD). 3-Hydroxy Acyl -CoA Dehydrogenase Deficiency (M/SCHADD). Short Chain Acyl-CoA Dehydrogenase Deficiency (SCADD). Glutamic Aciduria Type II (MADD). Ethylmalonic Aciduria 2,4 Dienoyl - CoA Reductase Deficiency Organic Acid ...
Pig kidney general acyl-CoA dehydrogenase is markedly stabilized against loss of flavin and activity in 7.3 M-urea or at 60 degrees C upon reduction with sodium dithionite or octanoyl-CoA. Electron transferring flavoprotein is similarly stabilized, whereas egg white riboflavin-binding protein loses flavin more readily on reduction. These and other data support the anticipated correlation between the kinetic stability of the holoproteins and the oxidation-reduction potential of their bound flavins. ...
Glutathione S-transferase (GST) isoenzymes of human pancreas were purified, characterized and evaluated for their possible role in the metabolism of ethanol. Human pancreas has at least two GST isoenzymes belonging to the Alpha class (pI 8.8 and 8.1), one belonging to the Mu class (pI 6.4) and one belonging to the Pi class (pI 4.9). During the purification of GSTs from pancreas as well as from heart, liver, lung, brain and muscle, the fatty acid ethyl ester synthase (FAEES) activity was monitored in order to evaluate the role of GSTs in metabolism of ethanol, as suggested in earlier studies. Both t.l.c. and h.p.l.c. were used to identify ethyl oleate in reaction mixtures to monitor FAEES activity. During the purification of GSTs with the use of affinity chromatography on GSH linked to epoxy-activated Sepharose 6B, FAEES and GST activities from each of these tissues segregated independently. Purified GST isoenzymes from these tissues did not exhibit any FAEES activity. Antibodies raised against ...
Looking for online definition of 3-hydroxyacyl-CoA dehydrogenase in the Medical Dictionary? 3-hydroxyacyl-CoA dehydrogenase explanation free. What is 3-hydroxyacyl-CoA dehydrogenase? Meaning of 3-hydroxyacyl-CoA dehydrogenase medical term. What does 3-hydroxyacyl-CoA dehydrogenase mean?
Short chain L-3-hydroxyacyl CoA dehydrogenase (SCHAD) is a mitochondrial enzyme involved in the metabolism of fatty acids. It catalyzes the oxidation of the hydroxyl group of L-3-hydroxyacyl CoA to a keto group, concomitant with the reduction of NAD+ to NADH. Deficiencies in this enzyme result in various disease states, including hypertrophic cardiomyopathy, skeletal myopathy, hypoketotic hypoglycemia, and liver dysfunction. The proposed research utilizes a multi-faceted approach to understanding the structure-function relationships present in human SCHAD. SCHAD will be purified to homogeneity from an appropriate expression system and its kinetic properties characterized. Crystals of human SCHAD suitable for x-ray diffraction studies will then be prepared in order to resolve the three-dimensional structure of the apoenzyme, the NAD+-complexed enzyme, and the acyl-CoA-complexed enzyme. Site directed mutagenesis and rational inhibitor design studies will be initiated to confirm or further develop ...
Definition of plasma very long-chain fatty acid assay in the Legal Dictionary - by Free online English dictionary and encyclopedia. What is plasma very long-chain fatty acid assay? Meaning of plasma very long-chain fatty acid assay as a legal term. What does plasma very long-chain fatty acid assay mean in law?
Author Summary Vaccinia virus, the prototypic poxvirus, is closely related to variola virus, the etiological agent of smallpox. A full understanding of the poxviral life cycle is imperative for the development of novel antiviral therapies, the design of new vaccines, and the effective and safe use of these viruses as oncolytic agents. Metabolomic studies have shed light on the novel mechanisms used by viruses to replicate efficiently within their hosts. de novo fatty acid biosynthesis has been shown to be of relevance for numerous viral infections as well as for the development of cancer. Here we describe an important role for de novo fatty acid biosynthesis during vaccinia infection. Ongoing synthesis of palmitate is needed to fuel the production of energy within mitochondria. The biochemical events of viral DNA replication and protein synthesis are minimally affected by inhibition of this pathway, but viral assembly is disrupted more dramatically. Further exploration of this pathway will provide
TY - JOUR. T1 - Human fatty acid transport protein 2a/very long chain Acyl-CoA synthetase 1 (FATP2a/Acsvl1) has a preference in mediating the channeling of exogenous n-3 fatty acids into phosphatidylinositol. AU - Melton, Elaina M.. AU - Cerny, Ronald L.. AU - Watkins, Paul A.. AU - DiRusso, Concetta C.. AU - Black, Paul N.. PY - 2011/9/2. Y1 - 2011/9/2. N2 - The trafficking of fatty acids across the membrane and into downstream metabolic pathways requires their activation to CoA thioesters. Members of the fatty acid transport protein/ very long chain acyl-CoA synthetase (FATP/Acsvl) family are emerging as key players in the trafficking of exogenous fatty acids into the cell and in intracellular fatty acid homeostasis.We have expressed two naturally occurring splice variants of human FATP2 (Acsvl1) in yeast and 293T-REx cells and addressed their roles in fatty acid transport, activation, and intracellular trafficking. Although both forms (FATP2a (M r 70,000) and FATP2b (M r 65,000 and lacking ...
Looking for online definition of 1-acyl-sn-glycerol-3-phosphate acyltransferase alpha in the Medical Dictionary? 1-acyl-sn-glycerol-3-phosphate acyltransferase alpha explanation free. What is 1-acyl-sn-glycerol-3-phosphate acyltransferase alpha? Meaning of 1-acyl-sn-glycerol-3-phosphate acyltransferase alpha medical term. What does 1-acyl-sn-glycerol-3-phosphate acyltransferase alpha mean?
The lipid A component of lipopolysaccharides from Fusobacterium nucleatum Fev 1 consists of beta-1',6-linked D-glucosamine disaccharides, which carry two phosphate groups: one in glycosidic and one in ester linkage. The amino groups of the glucosamine disaccharides are substituted by D-3-hydroxyhexadecanoic acid. The hydroxyl groups of the disaccharide backbone are acylated by tetradecanoic, hexadecanoic, and D-3-hydroxytetradecanoic acids. Part of the ester-bound D-3-hydroxytetradecanoic acid is 3-O-substituted by tetradecanoic acid. Whereas a similar pattern of fatty acids was detected in lipopolysaccharides from two other F. nucleatum strains, the amide-bound fatty acid in F. varium and F. mortiferum was D-3-hydroxytetradecanoic acid. The chemical relationships of lipid A from Fusobacteria and other gram-negative bacteria are discussed. ...
Synonyms for acyl CoA dehydrogenase deficiency in Free Thesaurus. Antonyms for acyl CoA dehydrogenase deficiency. 1 synonym for acyl: acyl group. What are synonyms for acyl CoA dehydrogenase deficiency?
1. GPAT (glycerol phosphate acyltransferase) and DHAPAT (dihydroxyacetone phosphate acyltransferase) activities were measured both in subcellular fractions prepared from fed rat liver and in whole homogenates prepared from freeze-stopped pieces of liver. 2. GPAT activity in mitochondria differed from the microsomal activity in that it was insensitive to N-ethylmaleimide, had a higher affinity towards the palmitoyl-CoA substrate and showed a different response to changes in hormonal and dietary status. 3. Starvation (48 h) significantly decreased mitochondrial GPAT activity. The ratio of mitochondrial to microsomal activities was also significantly decreased. The microsomal activity was unaffected by starvation, except after adrenalectomy, when it was significantly decreased. Mitochondrial GPAT activity was decreased by adrenalectomy in both fed and starved animals. 4. Acute administration of anti-insulin serum significantly decreased mitochondrial GPAT activity after 60 min without affecting the ...
We have measured the carbon isotopic compositions of individual fatty acids isolated from the adipose tissue of arctic foxes (Alopex lagopus) from the island of Svalbard on the Spitsbergen archipelago to examine whether dietary sources can be distinguished in the fatty acid isotopic signature. The diets of Svalbard arctic foxes are unusual in that some members of the population are believed to feed largely in the terrestrial food chain while others feed mainly from the marine food chain. Isotopic data were obtained for the five major fatty acids present (16:0, 16:1, 18:0, 18:1, and 20:1). A wide range in δ13C values for specific fatty acids in Arctic foxes is observed and the mixing lines produced by the correlation in δ13C of the 16- and 18-carbon fatty acids indicate that both marine and terrestrial dietary sources determine fatty acid isotope composition. The differences in δ13C values between marine and terrestrial organisms appear to be passed on to individual fatty acids. The abundance ...
TY - JOUR. T1 - Adipose tissue fatty acid composition in humans with lipoprotein lipase deficiency. AU - Ullrich, Nathan F.E.. AU - Purnell, Jonathan Q.. AU - Brunzell, John D.. PY - 2001/5. Y1 - 2001/5. N2 - Background: Lipid stores in human adipose tissue are maintained primarily by incorporating lipid from circulating chylomicrons and very low density lipoproteins. Adipose tissue lipoprotein lipase (LPL) hydrolyzes triglyceride from these lipoprotein particles to facilitate their entry into adipocytes for storage. Subjects deficient in LPL still have normal adiposity, and this may result from increased adipocyte lipogenesis or from uptake of circulating lipid through alternate mechanisms. The objective of this study was to determine whether fatty acid composition of adipose tissue from LPL-deficient subjects reflects maintenance of lipid stores through increased lipogenesis or through alternate mechanisms of lipoprotein uptake. Methods: Adipose tissue samples from LPL-deficient subjects who ...
A role for mitochondrial fatty acid oxidation in the peripheral signaling cascade of leptin, adiponectin and insulin has recently been proposed from animal studies but has not been investigated in humans. Children with trifunctional protein (TFP, including deficiency of long-chain hydroxyacyl-CoA dehydrogenase) and very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, inherited disorders of long-chain fatty acid ß-oxidation, lack an ability to oxidize fatty acids for energy. They have increased levels of body fat and circulating leptin and a high incidence of obesity. Current therapy for children with these disorders is based on frequent meals and consuming a low fat, very high carbohydrate diet. Despite treatment, exercise induced rhabdomyolysis is a common complication of TFP and VLCAD deficiency that frequently leads to exercise avoidance. The effects of these genetic defects on body composition and weight regulation have not been investigated. The contribution of fatty-acid oxidation ...
The safety and immunogenicity of the vaccine with the antigen NY-ESO-1 in combination with the MPLA will be evaluated in a phase 1 clinical trial conducted in patients with malignancies that express the antigen (lung, ovarian and melanoma). This study will involve 15 patients, who will receive 250 mcg of NY-ESO-1 and 100 mcg of MPLA ...
(R)-Lisofylline ((R)-Lisophylline) is a (R)-enantiomer of the metabolite of Pentoxifylline with anti-inflammatory properties. (R)-Lisofylline is a lysophosphatidic acid acyltransferase inhibitor with an IC50 of 0.6 µM and interrupts IL-12 signaling-mediated STAT4 activation. (R)-Lisofylline has the potential for type 1 diabetes, autoimmune disorders research. - Mechanism of Action & Protocol.
This study will treat children and adults who have documented deficiencies of mitochondrial fatty acid oxidation including disorders of the following enzymes: Carnitine-Acylcarnitine Translocase (CATR), Carnitine Palmitoyltransferase I and II (CPT I, CPT II), Very-Long Chain Acyl-CoA dehydrogenase (VLCAD), L-3-Hydroxy-Acyl-CoA Dehydrogenase (LCHAD), Acyl-CoA Dehydrogenase type 9 (ACAD9) and Mitochondrial Trifunctional Protein (TFP) with triheptanoin oil. This study is also open to patients with any type of glycogen storage disease, pyruvate carboxylase deficiency, type B, or Barth Syndrome.. Symptoms often persist with standard diet including supplementation with medium chain triglyceride oil. Preliminary data shows triheptanoin to reverse many of the clinical symptoms not well controlled by standard diet.. On study entry, clinical and laboratory assessments will be carried out with the subject on their usual home diet. A complete history and physical exam will be performed. An echocardiogram ...
2019 Elsevier Ltd A statement on measuring blood omega-3 long chain polyunsaturated fatty acid levels was developed and edited based on input from ISSFAL members and accepted by vote of the ISSFAL Board of Directors. Summary of Statement: Omega-3 long chain polyunsaturated fatty acid (n-3 LCPUFA) levels at baseline and post-intervention should be assessed and reported in future research to evaluate the efficacy of n-3 LCPUFA supplementation: b ecause; 1. there are numerous factors that affect n-3 LCPUFA levels in humans as described in the systematic literature review [1]; 2. assessing intake of n-3 LCPUFA from the diet and/or supplements is not sufficient to accurately determine n-3 LCPUFA levels in humans; 3. some studies do not provide sufficient doses of n-3 LCPUFA to produce a significant impact on bloodstream/organ content and there is substantial variability in the uptake of n-3 LPCUFA into tissues between individuals. In secondary analyses, clinical trials should consider the influence ...
Need some antibodies - posted in Immunology: I need antibody against LCHAD, MTP/TFP, VLCAD, SCHAD, MCAD, and don't know which company sells those antibodies. long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) mitochondrial trifunctional protein (MTP/TFP) very long-chain acyl-CoA dehydrogenase (VLCAD) short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) medium-chain 3-hydroxyacyl-CoA dehydrogenase (MCAD) Does anybody know? THANKS!!!
Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3-ketoacyl-CoA thiolase activity.
Lipid A, the hydrophobic anchor of lipopolysaccharide, is an essential component in the outer membrane of most Gram-negative bacteria. Food-borne pathogen Cronobacter sakazakii synthesizes two lipid A species, differing by the length of the secondary acyl chain. In this work, we identified three genes ESA02293, ESA02951 and ESA01386 encoding for the late acyltransferases of lipid A biosynthesis pathway in C. sakazakii. Based on the sequence alignment, proteins YP_001438378.1 encoded by ESA02293, YP_001439016.1 encoded by ESA02951, and YP_001437482.1 encoded by ESA01386 are homologous to E. coli LpxL, LpxP and LpxM, respectively. Functions of the three acyltransferases were confirmed by overexpressing the genes in E. coli, isolating lipid As and analyzing their structures using an ESI/MS. C. sakazakii LpxL and LpxM transfer a C14:0 secondary acyl chain to the 2′- and 3′-position of lipid A, respectively. C. sakazakii LpxP can transfer either a C16:1 or a C14:0 secondary acyl chains to the 2′
The signs and symptom information on this page attempts to provide a list of some possible signs and symptoms of Lactate dehydrogenase deficiency type C. This medical information about signs and symptoms for Lactate dehydrogenase deficiency type C has been gathered from various sources, may not be fully accurate, and may not be the full list of Lactate dehydrogenase deficiency type C signs or Lactate dehydrogenase deficiency type C symptoms. Furthermore, signs and symptoms of Lactate dehydrogenase deficiency type C may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they are indeed Lactate dehydrogenase deficiency type C symptoms. ...
K00826 E2.6.1.42; branched-chain amino acid aminotransferase [EC:2.6.1.42] K00382 DLD; dihydrolipoamide dehydrogenase [EC:1.8.1.4] K00382 DLD; dihydrolipoamide dehydrogenase [EC:1.8.1.4] K00382 DLD; dihydrolipoamide dehydrogenase [EC:1.8.1.4] K00249 ACADM; acyl-CoA dehydrogenase [EC:1.3.8.7] K00249 ACADM; acyl-CoA dehydrogenase [EC:1.3.8.7] K00249 ACADM; acyl-CoA dehydrogenase [EC:1.3.8.7] K00249 ACADM; acyl-CoA dehydrogenase [EC:1.3.8.7] K00249 ACADM; acyl-CoA dehydrogenase [EC:1.3.8.7] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] K01692 paaF; enoyl-CoA hydratase [EC:4.2.1.17] ...
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity).
Background Long-chain polyunsaturated fatty acids (LCPUFAs) reduce T-cell activation and dampen inflammation. They might thereby counteract the neonatal immune activation and hamper normal tolerance development to harmless environmental antigens. We investigated whether fatty acid composition of cord serum phospholipids affects allergy development up to age 13 years. Methods From a population-based birth-cohort born in 1996/7 and followed until 13 years of age (n = 794), we selected cases with atopic eczema (n = 37) or respiratory allergy (n = 44), as well as non-allergic non-sensitized controls (n = 48) based on diagnosis at 13 years of age. Cord and maternal sera obtained at delivery from cases and controls were analysed for proportions of saturated, monounsaturated and polyunsaturated fatty acids among serum phospholipids. Results The cord serum phospholipids from subject who later developed either respiratory allergy or atopic eczema had significantly higher proportions of 5/8 LCPUFA species, as
A common active site of polyhydroxyalkanoate synthase from Bacillus cereus YB-4 is involved in polymerization and alcoholysis reactionsA common active site of polyhydroxyalkanoate synthase from Bacillus cereus YB-4 is involved in polymerization and alcoholysis reactions ...
In enzymology, an acyloxyacyl hydrolase (EC 3.1.1.77) is an enzyme that catalyzes the chemical reaction 3-(acyloxy)acyl group of bacterial lipopolysaccharide (lipid A moiety) ⇌ {\displaystyle \rightleftharpoons } 3-hydroxyacyl group of bacterial lipopolysaccharide + a fatty acid Hence, this enzyme has one substrate, the 3-(acyloxy)acyl groups of bacterial lipopolysaccharides, and two products, [partially deacylated lipopolysaccharide] and fatty acid. The enzyme removes from lipid A the secondary acyl chains that are needed for lipopolysaccharides to be recognized by the MD-2--TLR4 receptor on animal cells. This reaction inactivates the lipopolysaccharide (endotoxin). Acyloxyacyl hydrolase is produced by monocyte-macrophages, neutrophils, dendritic cells, and renal cortical epithelial cells. It is a protein of Mr = ~60,000 that has two disulfide-linked subunits. The smaller subunit, of Mr = ~14,000 (including glycosylation), is a member of the SAPLIP (saposin-like protein) family along with ...
Global Medium Chain Triglycerides (MCT) Market 2019 is expected to demonstrate an enormous growth in the upcoming years. The analysts also have analyzed drawbacks with on-going Medium Chain Triglycerides (MCT) trends and the opportunities which are devoting to the increased growth of the market. International Medium Chain Triglycerides (MCT) market research report provides the perspective of this competitive landscape of worldwide markets. The report offers particulars that originated from the analysis of the focused market. Also, it targets innovative, trends, shares and cost by Medium Chain Triglycerides (MCT) industry experts to maintain a consistent investigation.. The Medium Chain Triglycerides (MCT) report presents an estimation of the forecast from 2019 to 2025 and market history from 2014 to 2018. The information provided in the form of earnings likely to be produced in (USD million) year to year by Medium Chain Triglycerides (MCT) growth rate (CAGR). The report explains market ...
Electron-transferring-flavoprotein dehydrogenase (ETF dehydrogenase or electron transfer flavoprotein-ubiquinone oxidoreductase, EC 1.5.5.1) is an enzyme that transfers electrons from electron-transferring flavoprotein in the mitochondrial matrix, to the ubiquinone pool in the inner mitochondrial membrane. It is part of the electron transport chain. The enzyme is found in both prokaryotes and eukaryotes and contains a flavin and FE-S cluster. In humans, it is encoded by the ETFDH gene. Deficiency in ETF dehydrogenase causes the human genetic disease multiple acyl-CoA dehydrogenase deficiency. ETQ-QO links the oxidation of fatty acids and some amino acids to oxidative phosphorylation in the mitochondria. Specifically, it catalyzes the transfer of electrons from electron transferring flavoprotein (ETF) to ubiquinone, reducing it to ubiquinol. The entire sequence of transfer reactions is as follows: Acyl-CoA → Acyl-CoA dehydrogenase → ETF → ETF-QO → UQ → Complex III. The overall reaction ...
Triacylglycerols (TAGs) and wax esters (WEs) are important neutral lipids which serve as energy reservoir in some plants and microorganisms. In recent years, these biologically produced neutral lipids have been regarded as potential alternative energy sources for biofuel production because of the increased interest on developing renewable and environmentally benign alternatives for fossil fuels. In bacteria, the final step in TAG and WE biosynthetic pathway is catalyzed by wax ester synthase/acyl coenzyme A (acyl-CoA):diacylglycerol acyltransferase (WS/DGAT). This bifunctional WS/DGAT enzyme is also a key enzyme in biotechnological production of liquid WE via engineering of plants and microorganisms. To date, knowledge about this class of biologically and biotechnologically important enzymes is mainly from biochemical characterization of WS/DGATs from Arabidopsis, jojoba and some bacteria that can synthesize both TAGs and WEs intracellularly, whereas little is known about WS/DGATs from eukaryotic
Ruthenium Oxidation Complexes explores ruthenium complexes, particularly those in higher oxidation states, which function as useful and selective organic oxidation catalysts. Particular emphasis is placed on those systems which are of industrial significance. The preparation, properties and applications of the ruthenium complexes are described, followed by a presentation of their oxidative properties and summary of the different mechanisms involved in the organic oxidations (e.g. oxidations of alcohols, alkenes, arenes and alkynes, alkanes, amines, ethers, phopshines and miscellaneous substrates). Moreover, future trends and developments in the area are discussed ...
Looking for online definition of 3-Deoxy-D-Manno-Octulosonate or what 3-Deoxy-D-Manno-Octulosonate stands for? 3-Deoxy-D-Manno-Octulosonate is listed in the World's largest and most authoritative dictionary database of abbreviations and acronyms
The mitochondrial beta-oxidation system is one of the central metabolic pathways of energy metabolism in mammals. Enzyme defects in this pathway cause fatty acid oxidation disorders. To elucidate the role of 2,4-dienoyl-CoA reductase (DECR) as an auxiliary enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids, we created a DECR-deficient mouse line. In Decr(-/-) mice, the mitochondrial beta-oxidation of unsaturated fatty acids with double bonds is expected to halt at the level of trans-2, cis/trans-4-dienoyl-CoA intermediates. In line with this expectation, fasted Decr(-/-) mice displayed increased serum acylcarnitines, especially decadienoylcarnitine, a product of the incomplete oxidation of linoleic acid (C-18:2), urinary excretion of unsaturated dicarboxylic acids, and hepatic steatosis, wherein unsaturated fatty acids accumulate in liver triacylglycerols. Metabolically challenged Decr(-/-) mice turned on ketogenesis, but unexpectedly developed hypoglycemia. Induced expression ...
Contents: Occurrence and importance of ether lipids in brain.- Introduction.- Classification of ether lipids found in brain.- Physicochemical properties of ether lipids.- Fecapentaenes, the novel plasmalogens.- Other ether lipid found in mammalian tissues.- Lipid metabolism in ether lipid-deficient mice.- Conclusion.- Biosynthesis of plasmalogens in brain.- General considerations and distribution of plasmalogens in brain.- Biosynthesis of plasmalogens.- Dihydroxyacetone phosphate acyltransferase.- Alkyl-dihydroxyacetone phosphate synthase.- Acyl/alkyl dihydroxyacetone phosphate reductase 2.2.4. Alkyl-GP acyltransferase.- Alkylacyl-GP phosphohydrolase.- CDP-ethanomamine: diacylglycerol ethanolaminephosphotransferase.- Plasmalogen synthesizing enzymes during brain development.- Topology and distribution of plasmalogen and plasmalogen synthesizing enzymes.- Plasmalogens in lipid rafts.- Plasmalogens in the nucleus.- Factors affecting plasmalogen biosynthesis in brain.- Conclusion.- Catabolism of ...
Francisella tularensis is a Gram-negative bacterium that infects hundreds of species including humans, and has evolved to grow efficiently within a plethora of cell types. RipA is a conserved membrane protein of F. tularensis, which is required for growth inside host cells. As a means to determine RipA function we isolated and mapped independent extragenic suppressor mutants in ∆ripA that restored growth in host cells. Each suppressor mutation mapped to one of two essential genes, lpxA or glmU, which are involved in lipid A synthesis. We repaired the suppressor mutation in lpxA (S102, LpxA T36N) and the mutation in glmU (S103, GlmU E57D), and demonstrated that each mutation was responsible for the suppressor phenotype in their respective strains. We hypothesize that the mutation in S102 altered the stability of LpxA, which can provide a clue to RipA function. LpxA is an UDP-N-acetylglucosamine acyltransferase that catalyzes the transfer of an acyl chain from acyl carrier protein (ACP) to UDP-N
Acute fatty liver of pregnancy is a rare life-threatening complication of pregnancy that occurs in the third trimester or the immediate period after delivery. It is thought to be caused by a disordered metabolism of fatty acids by mitochondria in the mother, caused by long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. The condition was previously thought to be universally fatal, but aggressive treatment by stabilizing the mother with intravenous fluids and blood products in anticipation of early delivery has improved prognosis. Acute fatty liver of pregnancy (or hepatic lipidosis of pregnancy) usually manifests in the third trimester of pregnancy, but may occur any time in the second half of pregnancy, or in the puerperium, the period immediately after delivery. On average, the disease presents during the 35th or 36th week of pregnancy. The usual symptoms in the mother are non-specific including nausea, vomiting, anorexia (or lack of desire to eat) and abdominal pain; excessive thirst ...
Essential gene studies often reveal novel essential functions for genes with dispensable homologues in other species. This is the case with the widespread family of electron transfer flavoproteins (ETFs), which are required for the metabolism of specific substrates or for symbiotic nitrogen fixation in some bacteria. Despite these non-essential functions high-throughput screens have identified ETFs as putatively essential in several species. In this study, we constructed a conditional expression mutant of one of the ETFs in Burkholderia cenocepacia, and demonstrated that its expression is essential for growth on both complex media and a variety of single-carbon sources. We further demonstrated that the two subunits EtfA and EtfB interact with each other, and that cells depleted of ETF are non-viable and lack redox potential. These cells also transition from the short rods characteristic of Burkholderia cenocepacia to small spheres independently of MreB. The putative membrane partner ETF dehydrogenase
Chlamydia trachomatis is a major etiologic agent of sexually transmitted diseases. Although C. trachomatis is a gram-negative pathogen, chlamydial infections are not generally thought of as endotoxin-mediated diseases. A molecular characterization of the acute immune response to chlamydia, especially with regard to the role of its lipopolysaccharide (LPS), remains to be undertaken. We extracted 15 mg of LPS from 5 x 10(12) C. trachomatis elementary bodies (EB) for analysis of structure and biological activity. When methylated lipid A was subjected to high-pressure liquid chromatography followed by mass spectrometry, the majority of the lipid A was found to be pentaacyl. The endotoxin activities of whole C. trachomatis EB and purified LPS were characterized in comparison with whole Salmonella minnesota R595 and with S. minnesota R595 LPS and lipooligosaccharide from Neisseria gonorrhoeae. Both C. trachomatis LPS and whole EB induced the release of tumor necrosis factor alpha from whole blood ex ...
Patient agrees to personally assume all risks associated with Patient's use of semen samples donated by a Donor that has tested positive as a carrier of D-bifunctional Protein Deficiency. Patient hereby releases Seattle Sperm Bank and its current and former officers, directors, employees, attorneys, insurers, agents and representatives of any liability or responsibility whatsoever for any and all outcomes, whether currently known, suspected, unknown or unsuspected, arising out of Patient's use of donor semen donated by Donor that has tested positive as a carrier of D-bifunctional Protein Deficiency ...
Our studies demonstrate that H. pylori LPS stimulates the release of both neutrophil-activating, C-X-C chemokines (IL-8 and ENA-78) and the monocyte-activating C-C chemokine MCP-1 from human monocytes. These chemokines are potent leukocyte chemoattractants and may play an important role in regulating inflammatory cell infiltration of H. pylori-infected gastric mucosa (7, 11, 14, 15,20, 21, 23, 27, 37, 40, 48). We found that H. pyloriLPS is less potent than Salmonella lipid A in inducing monocyte chemokine production. This finding agrees with previous studies showing low potency for H. pylori LPS in the induction of a wide variety of host inflammatory responses (9, 18,19, 34, 36, 38, 39, 42). However, when the actions of H. pylori LPS were specifically inhibited by using either an LPS antagonist or CD14 receptor blockade, the monocyte-activating potential of H. pylori water extract was almost completely abolished. These findings suggest that H. pylori LPS may be the primary monocyte-activating ...
Mito New England (MNE) is sponsoring a meeting April 30 to discuss new initiatives at Mass. General Hospital to assist in diagnosis and management of complex medical problems. Dr. Amel Karaa, Dr. Mark Korson, and Tim Boyd from NORD will share their perspectives. This event is open to all affected by complex disease, including but not limited to: Fabry disease, Gaucher disease, Pompe disease, Mucopolysaccahridosis, Mitochondrial disease, Niemann Pick, Fatty acid oxidation disorders, Glycogen storage diseases, Ehler Danlos Syndrome, and other random genetic conditions. Read more. ...
Limulus amebocyte lysate (LAL) is an aqueous extract of blood cells (amoebocytes) from the Atlantic horseshoe crab, Limulus polyphemus. LAL reacts with bacterial endotoxin lipopolysaccharide (LPS), which is a membrane component of gram-negative bacteria. This reaction is the basis of the LAL test, which is then used for the detection and quantification of bacterial endotoxins. Fred Bang reported in 1956 that gram-negative bacteria, even if killed, will cause the blood of the horseshoe crab to turn into a semi-solid mass. It was later recognized that the animal's blood cells, mobile cells called amoebocytes, contain granules with a clotting factor known as coagulogen; this is released outside the cell when bacterial endotoxin is encountered. The resulting coagulation is thought to contain bacterial infections in the animal's semi-closed circulatory system. In 1970 the U.S. Food and Drug Administration (FDA) approved LAL for testing drugs, products and devices that come in contact with the blood. ...
TY - JOUR. T1 - Interaction of malonyl-CoA and related compounds with mitochondria from different rat tissues. Relationship between ligand binding and inhibition of carnitine palmitoyltransferase I.. AU - Mills, S. E.. AU - Foster, D. W.. AU - McGarry, J. D.. PY - 1983/7/15. Y1 - 1983/7/15. N2 - The sensitivity of carnitine palmitoyltransferase I (CPT I; EC 2.3.1.21) to inhibition by malonyl-CoA and related compounds was examined in isolated mitochondria from liver, heart and skeletal muscle of the rat. In all three tissues the same order of inhibitory potency emerged: malonyl-CoA much greater than succinyl-CoA greater than methylmalonyl-CoA much greater than propionyl-CoA greater than acetyl-CoA. For any given agent, suppression of CPT I activity was much greater in skeletal muscle than in liver, with the heart enzyme having intermediate sensitivity. With skeletal-muscle mitochondria a high-affinity binding site for [14C]malonyl-CoA was readily demonstrable (Kd approx. 25 nM). The ability of ...
Dodecanedioic acid is a dicarboxylic acid which is water soluble and involves in a metabolic pathway intermediate to those of lipids and carbohydrates. (PMID 9591306). Dodecanedioid acid is an indicator of hepatic carnitine palmitoyltransferase I (CPT IA) deficiency. CPT IA deficiency is characterized by hypoketotic dicarboxylic aciduria with high urinary levels of dodecanedioic acid. This C12 dicarboxylic aciduria suggests that carnitine palmitoyltransferase I may play a role in the uptake of long-chain dicarboxylic acids by mitochondria after their initial shortening by beta-oxidation in peroxisomes. (PMID: 16146704 ...
Pseudomonas aeruginosa remains one of the major pathogens affecting immunocompromised patients. LPS-based monovalent (MV) and polyvalent (PV) conjugate vaccines were prepared from the most prevalent strains of P. aeruginosa International Antigenic Typing Scheme (IATS) 6, 10, 11 and 20 to evaluate their immunogenicity and protective capacities from infection by the pathogens. Conjugation of the O-polysaccharide (O-PS) antigens of P. aeruginosa strains to the common immunogenic recombinant Exotoxin A (rEPA) supports the multi-antigenic approach for the development of a vaccine that provides cross protection against various strains of the pathogen. The O-PSs were indirectly conjugated through adipic acid dihydrazide (ADH) to rEPA by carbodiimidemediated condensation reaction. Mice were immunized with the conjugates emulsified with monophosphoryl lipid A (MPL) or Freund's adjuvant compared with conjugates without adjuvant, unconjugated mixture of rEPA and O-PS emulsified with MPL, and sterile saline. The MV
In one family, genetic lightning struck twice. Two sisters were diagnosed with mitochondrial trifunctional protein (MTP) deficiency. This is a rare condition that stops the body from converting fats to energy, which can lead to lactic acidosis, recurrent breakdown of muscle tissue and release into the bloodstream (rhabdomyolysis), enlarged heart (cardiomyopathy) and liver failure.. Mitochondria are the cell's powerplants and inside them the MTP enzymatic complex catalyzes three steps in beta-oxidation of long-chain fatty acids. MTP deficiency is so rare that fewer than 100 cases have been reported in the literature says Hostensia Beng, M.D., who presented an MTP case study during the American Society of Nephrology's Kidney Week.. The 7-month-old girl with known MTP deficiency arrived at Children's National lethargic with poor appetite. Her laboratory results showed a low corrected serum calcium level, elevated CK level and protein in the urine (proteinuria) at a nephrotic range. The infant was ...
Initial step of β-oxidation is catalyzed by acyl-CoA dehydrogenase in prokaryotes and mitochondria, while acyl-CoA oxidase primarily functions in the peroxisomes of eukaryotes. Oxidase reaction accompanies emission of toxic by-product reactive oxygen molecules including superoxide anion, and superoxide dismutase and catalase activities are essential to detoxify them in the peroxisomes. Although there is an argument about whether primitive life was born and evolved under high temperature conditions, thermophilic archaea apparently share living systems with both bacteria and eukaryotes. We hypothesized that alkane degradation pathways in thermophilic microorganisms could be premature and useful to understand their evolution. An extremely thermophilic and alkane degrading Geobacillus thermoleovorans B23 was previously isolated from a deep subsurface oil reservoir in Japan. In the present study, we identified novel membrane proteins (P16, P21) and superoxide dismutase (P24) whose production levels were