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. ...

TY - JOUR. T1 - Class IV mammalian alcohol dehydrogenase. Structural data of the rat stomach enzyme reveal a new class well separated from those already characterized. AU - Parés, Xavier. AU - Moreno, Alberto. AU - Cederlund, Ella. AU - Höög, Jan Olov. AU - Jörnvall, Jans. PY - 1990/12/17. Y1 - 1990/12/17. N2 - The stomach form of alcohol dehydrogenase has been structurally evaluated by peptide analysis covering six separate regions of the rat enzyme. Overall, this new structure diners widely (32-40% residue differences) from the structures of three classes of alcohol dehydrogenase characterized before from the same species. Consequently, this novel enzyme constitutes a true fourth class of mammalian alcohol dehydrogenase. In particular, differences are extensive also towards class II, although enzymatic and physicochemical properties initially suggested overall similarities with class II. The new structure establishes the presence of one further alcohol dehydrogenase mammalian gene, extends ...

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 ...

definition of HLDH5, what does HLDH5 mean?, meaning of HLDH5, Human Lactate Dehydrogenase Isoenzyme 5, HLDH5 stands for Human Lactate Dehydrogenase Isoenzyme 5

TY - JOUR. T1 - Alcohol dehydrogenase activity in mouse brown adipose tissue. AU - Muralidhara, D. V.. AU - Desautels, M.. PY - 1996/4/1. Y1 - 1996/4/1. N2 - The present work provides evidence for the occurrence of the enzyme alcohol dehydrogenase (ADH) in very minute concentration in mice brown adipose tissue (BAT). Mice consuming 10% ethanol for 10 days showed significantly lowered enzyme activity in brown fat while liver ADH activity was increased but not significantly. Measurements of basal and norepinephrine stimulated oxygen consumption of isolated brown adipocytes indicated that the presence of ADH in BAT of mice is unlikely to play any role in ethanol oxidation.. AB - The present work provides evidence for the occurrence of the enzyme alcohol dehydrogenase (ADH) in very minute concentration in mice brown adipose tissue (BAT). Mice consuming 10% ethanol for 10 days showed significantly lowered enzyme activity in brown fat while liver ADH activity was increased but not significantly. ...

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. ...

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?

K00004 BDH; (R,R)-butanediol dehydrogenase / meso-butanediol dehydrogenase / diacetyl reductase [EC:1.1.1.4 1.1.1.- 1.1.1.303] K07535 badH; 2-hydroxycyclohexanecarboxyl-CoA dehydrogenase [EC:1.1.1.-] K08317 hcxA; hydroxycarboxylate dehydrogenase A [EC:1.1.1.-] K18369 adh2; alcohol dehydrogenase [EC:1.1.1.-] K19954 adh1; alcohol dehydrogenase [EC:1.1.1.-] K19955 adh2; alcohol dehydrogenase [EC:1.1.1.-] K21416 acoA; acetoin:2,6-dichlorophenolindophenol oxidoreductase subunit alpha [EC:1.1.1.-] K21416 acoA; acetoin:2,6-dichlorophenolindophenol oxidoreductase subunit alpha [EC:1.1.1.-] K21417 acoB; acetoin:2,6-dichlorophenolindophenol oxidoreductase subunit beta [EC:1.1.1.-] K21417 acoB; acetoin:2,6-dichlorophenolindophenol oxidoreductase subunit beta [EC:1.1.1.-] K22230 iolU; scyllo-inositol 2-dehydrogenase (NADP+) [EC:1.1.1.-] K23257 yvgN; methylglyoxal/glyoxal reductase [EC:1.1.1.283 1.1.1 ...

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 ...

Define glutamic oxaloacetic transaminase. glutamic oxaloacetic transaminase synonyms, glutamic oxaloacetic transaminase pronunciation, glutamic oxaloacetic transaminase translation, English dictionary definition of glutamic oxaloacetic transaminase. Noun 1. glutamic oxaloacetic transaminase - an enzyme involved in transamination glutamic oxalacetic transaminase aminopherase, aminotransferase,...

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?

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 ...

TY - JOUR. T1 - S-nitrosoglutathione reductase activity of human and yeast glutathione-dependent formaldehyde dehydrogenase and its nuclear and cytoplasmic localisation. AU - Fernández, M. R.. AU - Biosca, J. A.. AU - Parés, X.. PY - 2003/5/1. Y1 - 2003/5/1. N2 - S-nitrosoglutathione (GSNO) formation represents a mechanism for storage and transport of nitric oxide. Analysis of human liver and Saccharomyces cerevisiae extracts has revealed the presence of only one enzyme able to significantly reduce GSNO, identified as glutathione-dependent formaldehyde dehydrogenase (FALDH). GSNO is the best substrate known for the human and yeast enzymes (kcat/Km = 444,400 and 350,000 mM-1 min-1, respectively). Although NADH is the preferred cofactor, some activity with NADPH (Km = 460 μM) can be predicted in vivo. The subcellular localization demonstrates a cytosolic and nuclear distribution of FALDH in living yeast cells. This agrees with previous results in rat, and suggests a role in the regulation of ...

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. ...

One research study found that in the past, c-type cytochromes in Pelobacter species had not been detected even though close relatives in the Geobacteraceae family have many c-type cytochromes present. Careful study of the entire completed genome sequence of Pelobacter carbinolicus found 14 open reading frames that encode for c-type cytochromes. It was found that three c-type cytochrome genes were expressed during iron reduction, which suggests that these particular proteins may play a role in electron transfer to iron. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis gels of acetoin-fermenting Pelobacter carbinolicus protein cells showed three heme-staining bands. In addition, many of the c-type cytochromes that genetic studies have realized are required for optimal iron reduction in G. sulfurreducens were not present in the P. carbinolicus genome. The results from this study suggest more in depth studies of the functions of c-type cytochromes may possibly be beneficial for further ...

Our previous studies showed that glycerol fermentation by Hafnia alvei AD27 strain was accompanied by formation of high quantities of lactate. The ultimate aim of this work was the elimination of excessive lactate production in the 1,3-propanediol producer cultures. Group II intron-mediated deletion of ldh (lactate dehydrogenase) gene in an environmental isolate of H. alvei AD27 strain was conducted. The effect of the Δldh genotype in H. alvei AD27 strain varied depending on the culture medium applied. Under lower initial glycerol concentration (20 gL-1), lactate and 1,3-propanediol production was fully abolished, and the main carbon flux was directed to ethanol synthesis. On the other hand, at higher initial glycerol concentrations (40 gL-1), 1,3-propanediol and lactate production was recovered in the recombinant strain. The final titers of 1,3-propanediol and ethanol were similar for the recombinant and the WT strains, while the Δldh genotype displayed significantly decreased lactate titer. ...

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 ...

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.

The ability of Pelobacter carbinolicus to oxidize electron donors with electron transfer to the anodes of microbial fuel cells was evaluated because microorganisms closely related to Pelobacter species are generally abundant on the anodes of microbial fuel cells harvesting electricity from aquatic sediments. P. carbinolicus could not produce current in a microbial fuel cell with electron donors which support Fe(III) oxide reduction by this organism. Current was produced using a coculture of P. carbinolicus and Geobacter sulfurreducens with ethanol as the fuel. Ethanol consumption was associated with the transitory accumulation of acetate and hydrogen. G. sulfurreducens alone could not metabolize ethanol, suggesting that P. carbinolicus grew in the fuel cell by converting ethanol to hydrogen and acetate, which G. sulfurreducens oxidized with electron transfer to the anode. Up to 83% of the electrons available in ethanol were recovered as electricity and in the metabolic intermediate acetate. ...

1. Previous studies have not detected c-type cytochromes in Pelobacter species even though its other close relatives in the Geobacteraceae family, such as Geobacter and Desulfuromonas species, have many c-type cytochromes. Analysis of the recently completed genome sequence of Pelobacter carbinolicus showed 14 open reading frames that may encode for c-type cytochromes. Transcripts for all but one of the open reading frames were detected in acetoin-fermenting and/or Fe(III)-reducing cells. It was found that three putative c-type cytochrome genes were expressed during Fe(III) reduction, which suggests that the encoded proteins may play a role in electron transfer to Fe(III). One of these proteins was a periplasmic triheme cytochrome which was very similar to PpcA, which has a role in Fe(III) reduction in Geobacter sulfurreducens. Genes for heme biosynthesis and system II cytochrome c biogenesis were also identified in the genome and expressed. Sodium dodecyl sulfate-polyacrylamide gel ...

Effect of Elaeagnus Conferta Roxb (Elaeagnaceae) Dry Fruit on the Activities of Hepatic Alcohol Dehydrogenase and Aldehyde Dehydrogenase in Mice. Chongming Wu; Rongji Dai; Jingmiao Bai; Yan Chen; Yuhong Yu; Weiwei Meng; Yulin Deng // Tropical Journal of Pharmaceutical Research;Dec2011, Vol. 10 Issue 6, p761 Purposes: To determine the effect of Elaeagnus conferta Roxb dry fruit powder (ECR) on blood alcohol clearance and on the activities of hepatic alcohol dehydrogenase (ADH) and aldehyde dehydrogenase (ALDH). Methods: In a randomized controlled study, acute alcohol intoxication was induced in mice... ...

Previously we have developed a butanol tolerant mutant of Clostridium acetobutylicum Rh8, from the wild type strain DSM 1731. Strain Rh8 can tolerate up to 19 g/L butanol, with solvent titer improved accordingly, thus exhibiting industrial application potential. To test if strain Rh8 can be used for production of high level mixed alcohols, a single secondary alcohol dehydrogenase from Clostridium beijerinckii NRRL B593 was overexpressed in strain Rh8 under the control of thl promoter. The heterogenous gene sADH was functionally expressed in C. acetobutylicum Rh8. This simple, one-step engineering approach switched the traditional ABE (acetone-butanol-ethanol) fermentation to IBE (isopropanol-butanol-ethanol) fermentation. The total alcohol titer reached 23.88 g/l (7.6 g/l isopropanol, 15 g/l butanol, and 1.28 g/l ethanol) with a yield to glucose of 31.42%. The acid (butyrate and acetate) assimilation rate in isopropanol producing strain Rh8(psADH) was increased. The improved butanol tolerance and the

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] ...

BioAssay record AID 723630 submitted by ChEMBL: Antiplasmodial activity against erythrocyte form of chloroquine-resistant Plasmodium falciparum Dd2 by parasite lactate dehydrogenase assay.

Background. Lysosomal enzymuria is usually considered to be a non-specific marker of renal injury, but little is known about lysosomal enzyme excretion in renal proximal tubular cell disorders such as the renal Fanconi syndrome (FS). We examined excretion of two lysosomal enzymes and the cation-independent mannose-6-phosphate receptor (CI-MPR) in patients with inherited FS.. Methods. The lysosomal enzyme cathepsin D was measured by ELISA and isolated by pepstatin-agarose affinity chromatography; N-acetyl-β-d-glucosaminidase (NAG) was assayed colorimetrically, as was the cytosolic enzyme lactate dehydrogenase (LDH). Cathepsin D, procathepsin D and CI-MPR were also detected by western blotting. No patient had a serum creatinine concentration ,170 μmol/L. Soluble CI-MPR, isolated from fetal calf serum and bound to agarose, was used to probe cathepsin D for mannose-6-phosphate (M6P).. Results. Increased excretion of cathepsin D (mean = 44-fold) and NAG (mean = 12-fold) was found in FS patients: ...

The aerobic alkane conversion pathways for various lengths of alkanes from Gordonia sp. TF6 and Geobacillus thermodenitrificans were the basis for the alkane degradation parts. These pathways were implemented in E.coli using the BioBrick principle and characterized in detail with respect to single enzyme activities and affinity. Using these measurements the efficiency of different enzymes can easily be compared. We succesfully characterized the alkane hydroxylase system, which had an enzyme activity of 4.49x10-2 U/mg dry weight compared to 1.23x10-3 U/mg dry weight for the negative control. The enzyme activity of LadA was found to be 3.33x10-3U/mg total protein, compared to 5.49x10-4 U/mg total protein for the negative control strain. The characterization showed that the implemented alcohol dehydrogenase (ADH, for the conversion of long chain alkanols to alkanals) converted dodecanol 43% better than the standard E.coli. On the other hand, the expression of our ALDH system increased the dodecanal ...

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 ...

Candidate-gene-based association study which involves the identification of causative Single Nucleotide Polymorphisms (SNPs) for excellent traits has been proposed as a promising approach to dissect complex traits in forest trees. Hence, the goal of this study was to identify the genetic association among SNPs from Cinnamate 4-Hydroxylase (C4H) and Cinnamyl Alcohol Dehydrogenase (CAD) genes and an array of wood properties namely, specific gravity, wood density, fiber-length, cell wall thickness and microfibril angle from Acacia mangium Superbulk trees. Sequence variations within these two genes in 12 A. mangium Superbulk trees were examined and wood properties were measured. The data obtained was tested using General Linear Model (GLM) within TASSEL software. Two SNPs were identified in the exon of C4H, of which all the SNPs caused nonsynonymous mutations whereas five SNPs were identified in the CAD exons along with one deletion mutation. In addition, two SNPs were also identified in the CAD ...

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 acyl-CoA dehydrogenase family member 9, mitochondrial in the Medical Dictionary? acyl-CoA dehydrogenase family member 9, mitochondrial explanation free. What is acyl-CoA dehydrogenase family member 9, mitochondrial? Meaning of acyl-CoA dehydrogenase family member 9, mitochondrial medical term. What does acyl-CoA dehydrogenase family member 9, mitochondrial mean?

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 ...

Semin Gastrointest Dis ; Bacterial contamination of pancreatic necrosis: Is CT guided fine needle aspiration helpful in patients with infected necrosis. Acute pancreatitis dietary management MD, Mittal A, van den Heever M, et al, for the Pancreas Network of New Zealand. MRI may also identify early duct disruption that Acute pancreatitis dietary management not seen on CT. The serum amylase level is IU per liter, the serum lipase level is IU per liter, the serum alanine aminotransferase level is Acute pancreatitis dietary management per liter, and the serum lactate dehydrogenase level Acute pancreatitis dietary management IU per liter. For patients with mild acute pancreatitis, nasojejunal feedings can be avoided unless patients are unable to tolerate oral intake for over 1 week. Most of these collections resolve Acute pancreatitis dietary management. Evaluation of severity in patients with acute pancreatitis. Radiol Clin North Am. Generally, however, pancreatic stents are difficult to monitor, ...

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

Cellular location of ganglioside-sialidase activity was determined in confluent hamster embryo fibroblasts transformed with herpes simplex virus type 2. Approximately equal specific activities of ganglioside-sialidase activity were found to be associated with the crude lysosomal and crude plasma membrane fractions isolated from whole cell homogenates. Whole transformed cells hydrolyzed exogenous ganglioside substrate, suggesting a partial location of the cellular sialidase on the outer surface of the plasma membrane of these cells. Intact cells were treated with the diazonium salt of sulfanilic acid, a nonpenetrating reagent inhibitory to ecto-enzymes (DePierre, J.W., and M. L. Karnovsky. 1974. J. Biol. Chem. 249:7111-7120). Cytoplasmic lactate dehydrogenase activity was not inhibited by this treatment, and mitochondrial succinate dehydrogenase activity was inhibited only 10%, indicating that intracellular enzymes were not affected. 5'-Nucleotidase activity was diminished 90%, and sialidase very ...

pyruvate decarboxylase ^ CO2. a-acetolactate/acetaldehyde-TPP. ,CO2. CO2 diacetyl reductase diacetyl -^ ^ ► acetoin reductase / % '. NADH+H NAD. acetoin reductase c. NADH+H. NAD 2,3-butanediol catalyzed by a-acetolactate synthase and requiring high concentrations of pyruvate. The a-acetolactate is unstable in the presence of oxygen and is finally decarboxylated, nonenzy-matically, to form diacetyl. It is important to note that diacetyl is not necessarily the terminal end-product of the pathway. Further reduction of diacetyl can also occur, forming acetoin and 2,3-butanediol, compounds that contribute no flavor or aroma to the product.. Following the addition of the culture, the mix is incubated at 21°C to 22°C for twelve to sixteen hours. At the end of the fermentation, when the titratable acidity has reached 0.85% to 0.90% and the pH has decreased to about 4.5, the product is cooled to 2°C and agitated to break up the coagulum. Salt is usually added, and, if desired, butter granules or ...

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 ...

Deoxynivalenol (DON) frequently contaminates animal feed, including fish feed used in aquaculture. This study intends to further investigate the effects of DON on carp (Cyprinus carpio L.) at concentrations representative for commercial fish feeds. Experimental feeding with 352, 619 or 953 μg DON kg−1 feed resulted in unaltered growth performance of fish during six weeks of experimentation, but increased lipid peroxidation was observed in liver, head kidney and spleen after feeding of fish with the highest DON concentration. These effects of DON were mostly reversible by two weeks of feeding the uncontaminated control diet. Histopathological scoring revealed increased liver damage in DON-treated fish, which persisted even after the recovery phase. At the highest DON concentration, significantly more fat, and consequently, increased energy content, was found in whole fish body homogenates. This suggests that DON affects nutrient metabolism in carp. Changes of lactate dehydrogenase (LDH) activity in

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. ...

Following treatment with the mutagen N-methyl-N'-nitro-N-nitrosoguanidine, three mutants of Lactococcus lactis subsp. lactis biovar diacetylactis CNRZ 483 that produced diacetyl and acetoin from glucose were isolated. The lactate dehydrogenase activity of these mutants was strongly attenuated, and the mutants produced less lactate than the parental strain. The kinetic properties of lactate dehydrogenase of strain CNRZ 483 and the mutants revealed differences in the affinity of the enzyme for pyruvate, NADH, and fructose-1,6-diphosphate. When cultured aerobically, strain CNRZ 483 transformed 2.3% of glucose to acetoin and produced no diacetyl or 2,3-butanediol. Under the same conditions, mutants 483L1, 483L2, and 483L3 transformed 42.0, 78.9, and 75.8%, respectively, of glucose to C4 compounds (diacetyl, acetoin, and 2,3-butanediol). Anaerobically, strain CNRZ 483 produced no C4 compounds, while mutants 483L1, 483L2, and 483L3 transformed 2.0, 37.0, and 25.8% of glucose to acetoin and 2,3-butanediol. In

K04072 adhE; acetaldehyde dehydrogenase / alcohol dehydrogenase [EC:1.2.1.10 1.1.1.1] K00656 E2.3.1.54; formate C-acetyltransferase [EC:2.3.1.54] K01641 E2.3.3.10; hydroxymethylglutaryl-CoA synthase [EC:2.3.3.10] K01652 E2.2.1.6L; acetolactate synthase I/II/III large subunit [EC:2.2.1.6] K01652 E2.2.1.6L; acetolactate synthase I/II/III large subunit [EC:2.2.1.6] K01653 E2.2.1.6S; acetolactate synthase I/III small subunit [EC:2.2.1.6] K01575 alsD; acetolactate decarboxylase [EC:4.1.1.5] K00004 BDH; (R,R)-butanediol dehydrogenase / meso-butanediol dehydrogenase / diacetyl reductase [EC:1.1.1.4 1.1.1.- 1.1.1.303] K03366 butA; meso-butanediol dehydrogenase / (S,S)-butanediol dehydrogenase / diacetyl reductase [EC:1.1.1.- 1.1.1.76 1.1.1.304 ...

Hemorrhagic shock (HS) following acute alcohol intoxication can increase proinflammatory cytokine production and induce marked immunosuppression. We investigated the effects of ethanol on physiopathology and cytokine levels following HS in acutely alcohol-intoxicated rats. Rats received an intravenous injection of 5 g/kg ethanol over 3 h followed by HS induced by withdrawal of 40% of total blood volume from a femoral arterial catheter over 30 min. Mean arterial pressure (MAP) and heart rate (HR) were monitored continuously for 48 h after the start of blood withdrawal. Biochemical parameters, including hemoglobin, ethanol, glutamic oxaloacetic transaminase (GOT), glutamic pyruvic transaminase (GPT), blood urea nitrogen (BUN), creatinine (Cre), lactic dehydrogenase (LDH), and creatine phosphokinase (CPK), were measured at 30 min before induction of HS and 0, 1, 3, 6, 9, 12, 18, 24, and 48 h after HS. Serum tumor necrosis factor-|i|α|/i| (TNF-|i|α|

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 ...

Acetoin (AC) and 2,3-butanediol (2,3-BD) as highly promising bio-based platform chemicals have received more attentions due to their wide range of applications. However, the non-efficient substrate conversion and mutually transition between AC and 2,3-BD in their natural producing strains not only led to a low selectivity but also increase the difficulty of downstream purification. Therefore, synthetic engineering of more suitable strains should be a reliable strategy to selectively produce AC and 2,3-BD, respectively. In this study, the respective AC (alsS and alsD) and 2,3-BD biosynthesis pathway genes (alsS, alsD, and bdhA) derived from Bacillus subtilis 168 were successfully expressed in non-natural AC and 2,3-BD producing Corynebacterium crenatum, and generated recombinant strains, C. crenatum SD and C. crenatum SDA, were proved to produce 9.86 g L−1 of AC and 17.08 g L−1 of 2,3-BD, respectively. To further increase AC and 2,3-BD selectivity, the AC reducing gene (butA) and lactic acid

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

As long as your yeast is healthy and abundant and you're not cold-crashing the beer as soon as fermentation tails off, you'll be fine. I've fermented with a number of Saison and Belgian strains (though I can't remember which off the top of my head) and I've never had any problems with diacetyl. Also, basically any strain that's known to produce diacetyl in over-abundance will say so on its spec sheet.. The elevated temperature many Belgians ferment at is actually a boon to yeast's reduction of diacetyl to flavor-neutral compounds after primary. Also, most Belgians are medium to medium-low flocculators (highly flocculent yeast tends to leave more diacetyl around). More yeast in suspension = more power to reduce diacetyl post-fermentation. To be safe, there's really no reason not to just let your beers sit for a week at primary temperature after fermentation is done to make extra sure all the D is gone. A small, virtually risk-free step you can take to ensure good beer.. As far as I'm concerned ...

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