3-Hydroxyacyl CoA Dehydrogenases: Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in the presence of NAD. They are key enzymes in the oxidation of fatty acids and in mitochondrial fatty acid synthesis.Acyl-CoA Dehydrogenases: Enzymes that catalyze the first step in the beta-oxidation of FATTY ACIDS.Acyl-CoA Dehydrogenase: A flavoprotein oxidoreductase that has specificity for medium-chain fatty acids. It forms a complex with ELECTRON TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.Lipid A: Lipid A is the biologically active component of lipopolysaccharides. It shows strong endotoxic activity and exhibits immunogenic properties.Acyltransferases: Enzymes from the transferase class that catalyze the transfer of acyl groups from donor to acceptor, forming either esters or amides. (From Enzyme Nomenclature 1992) EC 2.3.Fatty Acids: Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)Dictionaries, MedicalEnoyl-CoA Hydratase: An enzyme that catalyzes reversibly the hydration of unsaturated fatty acyl-CoA to yield beta-hydroxyacyl-CoA. It plays a role in the oxidation of fatty acids and in mitochondrial fatty acid synthesis, has broad specificity, and is most active with crotonyl-CoA. EC 4.2.1.17.Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase: An NAD-dependent 3-hydroxyacyl CoA dehydrogenase that has specificity for acyl chains containing 8 and 10 carbons.Mitochondrial Trifunctional Protein: A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. It contains enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, and acetyl-CoA C-acyltransferase activities and plays an important role in the metabolism of long chain FATTY ACIDS.Peroxisomal Bifunctional Enzyme: A monomeric protein found in liver peroxisomes that contains two enzymatically active domains; an enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase domain, and an (S)-3-hydroxyacyl-CoA dehydrogenase domain. The enzyme is stereospecific with regards to how cis and trans double bonds are metabolized. It is complemented by PEROXISOMAL MULTIFUNCTIONAL PROTEIN-2, which has the opposite stereospecificity.Peroxisomal Multifunctional Protein-2: A dimeric protein found in liver peroxisomes that plays an important role in FATTY ACID metabolism and steroid metabolism. The dimer is formed by cleavage of a single protein precursor and contains an enoyl-CoA hydratase-2 domain and a second domain that displays (S)-3-hydroxyacyl-CoA dehydrogenase and 17-beta-estradiol dehydrogenase activities. The enzyme is stereospecific with regards to arrangement of the substrate double bonds and position of the 3-hydroxy group of the reaction intermediate. It is complemented by PEROXISOMAL BIFUNCTIONAL ENZYME, which has the opposite reaction stereospecificity.Metabolome: The dynamic collection of metabolites which represent a cell's or organism's net metabolic response to current conditions.Metabolomics: The systematic identification and quantitation of all the metabolic products of a cell, tissue, organ, or organism under varying conditions. The METABOLOME of a cell or organism is a dynamic collection of metabolites which represent its net response to current conditions.Visual Acuity: Clarity or sharpness of OCULAR VISION or the ability of the eye to see fine details. Visual acuity depends on the functions of RETINA, neuronal transmission, and the interpretative ability of the brain. Normal visual acuity is expressed as 20/20 indicating that one can see at 20 feet what should normally be seen at that distance. Visual acuity can also be influenced by brightness, color, and contrast.Central Serous Chorioretinopathy: A visual impairment characterized by the accumulation of fluid under the retina through a defect in the retinal pigment epithelium.Lipid Metabolism, Inborn Errors: Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.Glucosephosphate Dehydrogenase Deficiency: A disease-producing enzyme deficiency subject to many variants, some of which cause a deficiency of GLUCOSE-6-PHOSPHATE DEHYDROGENASE activity in erythrocytes, leading to hemolytic anemia.Nigella sativa: A plant genus of the family RANUNCULACEAE that contains alpha-hederin, a triterpene saponin in the seeds, and is the source of black seed oil.Phenylhydrazines: Diazo derivatives of aniline, used as a reagent for sugars, ketones, and aldehydes. (Dorland, 28th ed)Acclimatization: Adaptation to a new environment or to a change in the old.Cold Temperature: An absence of warmth or heat or a temperature notably below an accustomed norm.Myocardium: The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.Heinz Bodies: Abnormal intracellular inclusions, composed of denatured hemoglobin, found on the membrane of red blood cells. They are seen in thalassemias, enzymopathies, hemoglobinopathies, and after splenectomy.Anemia, Hemolytic: A condition of inadequate circulating red blood cells (ANEMIA) or insufficient HEMOGLOBIN due to premature destruction of red blood cells (ERYTHROCYTES).Anemia: A reduction in the number of circulating ERYTHROCYTES or in the quantity of HEMOGLOBIN.Fatigue: The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli.Databases, Protein: Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.Oxidation-Reduction: A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).Sequence Analysis, Protein: A process that includes the determination of AMINO ACID SEQUENCE of a protein (or peptide, oligopeptide or peptide fragment) and the information analysis of the sequence.Antibodies: Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).Hydroxysteroid Dehydrogenases: Enzymes of the oxidoreductase class that catalyze the dehydrogenation of hydroxysteroids. (From Enzyme Nomenclature, 1992) EC 1.1.-.Antibody Specificity: The property of antibodies which enables them to react with some ANTIGENIC DETERMINANTS and not with others. Specificity is dependent on chemical composition, physical forces, and molecular structure at the binding site.Antibodies, Monoclonal: Antibodies produced by a single clone of cells.L-Lactate Dehydrogenase: A tetrameric enzyme that, along with the coenzyme NAD+, catalyzes the interconversion of LACTATE and PYRUVATE. In vertebrates, genes for three different subunits (LDH-A, LDH-B and LDH-C) exist.Recombinant Proteins: Proteins prepared by recombinant DNA technology.3-Hydroxysteroid Dehydrogenases: Catalyze the oxidation of 3-hydroxysteroids to 3-ketosteroids.Burning Mouth Syndrome: A group of painful oral symptoms associated with a burning or similar sensation. There is usually a significant organic component with a degree of functional overlay; it is not limited to the psychophysiologic group of disorders.Pharyngeal Diseases: Pathological processes involving the PHARYNX.Pruritus Vulvae: Intense itching of the external female genitals.Diagnostic Errors: Incorrect diagnoses after clinical examination or technical diagnostic procedures.Sensation: The process in which specialized SENSORY RECEPTOR CELLS transduce peripheral stimuli (physical or chemical) into NERVE IMPULSES which are then transmitted to the various sensory centers in the CENTRAL NERVOUS SYSTEM.Cellulitis: An acute, diffuse, and suppurative inflammation of loose connective tissue, particularly the deep subcutaneous tissues, and sometimes muscle, which is most commonly seen as a result of infection of a wound, ulcer, or other skin lesions.Ear: The hearing and equilibrium system of the body. It consists of three parts: the EXTERNAL EAR, the MIDDLE EAR, and the INNER EAR. Sound waves are transmitted through this organ where vibration is transduced to nerve signals that pass through the ACOUSTIC NERVE to the CENTRAL NERVOUS SYSTEM. The inner ear also contains the vestibular organ that maintains equilibrium by transducing signals to the VESTIBULAR NERVE.alpha-Linolenic Acid: A fatty acid that is found in plants and involved in the formation of prostaglandins.Plant Oils: Oils derived from plants or plant products.Fatty Liver: Lipid infiltration of the hepatic parenchymal cells resulting in a yellow-colored liver. The abnormal lipid accumulation is usually in the form of TRIGLYCERIDES, either as a single large droplet or multiple small droplets. Fatty liver is caused by an imbalance in the metabolism of FATTY ACIDS.DiglyceridesLiver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.Lipid Metabolism: Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS.Diacylglycerol Kinase: An enzyme of the transferase class that uses ATP to catalyze the phosphorylation of diacylglycerol to a phosphatidate. EC 2.7.1.107.

Identity of heart and liver L-3-hydroxyacyl coenzyme A dehydrogenase. (1/341)

Rat heart and liver cDNAs for precursor of L-3-hydroxyacyl-CoA dehydrogenase have been cloned and sequenced. The results indicate that these different rat organs express identical dehydrogenases. Furthermore, pig heart mRNA for L-3-hydroxyacyl-CoA dehydrogenase precursor was amplified by reverse transcription-polymerase chain reaction, and all the cDNA clones were found to encode a precursor of liver L-3-hydroxyacyl-CoA dehydrogenase (X.-Y. He, S.-Y. Yang, Biochim. Biophys. Acta 1392 (1998) 119-126) but not the well-documented heart form of the dehydrogenase (K.G. Bitar et al., FEBS Lett. 116 (1980) 196-198). Sequencing data and other evidence establish that the pig, like the rat, has the same dehydrogenase in heart and liver. Since the size and structure of pig heart L-3-hydroxyacyl-CoA dehydrogenase are identical to the pig liver dehydrogenase, reports that relied on the published sequence of the pig heart dehydrogenase need to be re-evaluated. For example, the signature pattern of the L-3-hydroxyacyl-CoA dehydrogenase family is HXFXPX3MXLXE. Furthermore, the published crystal structure of the pig heart dehydrogenase that substantiated each subunit comprising 307 residues with a mercury-binding residue at position 204 (J.J. Birktoft et al., Proc. Natl. Acad. Sci. U.S.A. 84 (1987) 8262-8266) must be re-examined in accordance with this revelation.  (+info)

High aerobic capacities in the skeletal muscles of pinnipeds: adaptations to diving hypoxia. (2/341)

The objective was to assess the aerobic capacity of skeletal muscles in pinnipeds. Samples of swimming and nonswimming muscles were collected from Steller sea lions (Eumetopias jubatus, n = 27), Northern fur seals (Callorhinus ursinus, n = 5), and harbor seals (Phoca vitulina, n = 37) by using a needle biopsy technique. Samples were either immediately fixed in 2% glutaraldehyde or frozen in liquid nitrogen. The volume density of mitochondria, myoglobin concentration, citrate synthase activity, and beta-hydroxyacyl-CoA dehydrogenase was determined for all samples. The swimming muscles of seals had an average total mitochondrial volume density per volume of fiber of 9.7%. The swimming muscles of sea lions and fur seals had average mitochondrial volume densities of 6.2 and 8.8%, respectively. These values were 1.7- to 2.0-fold greater than in the nonswimming muscles. Myoglobin concentration, citrate synthase activity, and beta-hydroxyacyl-CoA dehydrogenase were 1.1- to 2. 3-fold greater in the swimming vs. nonswimming muscles. The swimming muscles of pinnipeds appear to be adapted for aerobic lipid metabolism under the hypoxic conditions that occur during diving.  (+info)

Oxidation of medium-chain acyl-CoA esters by extracts of Aspergillus niger: enzymology and characterization of intermediates by HPLC. (3/341)

The activities of beta-oxidation enzymes were measured in extracts of glucose- and triolein-grown cells of Aspergillus niger. Growth on triolein stimulated increased enzyme activity, especially for acyl-CoA dehydrogenase. No acyl-CoA oxidase activity was detected. HPLC analysis after incubation of triolein-grown cell extracts with decanoyl-CoA showed that beta-oxidation was limited to one cycle. Octanoyl-CoA accumulated as the decanoyl-CoA was oxidized. Beta-oxidation enzymes in isolated mitochondrial fractions were also studied. The results are discussed in the context of methyl ketone production by fungi.  (+info)

Dietary management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey. (4/341)

Current dietary management of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD; long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxido-reductase, EC 1.1.1.211) deficiency (LCHADD) is based on avoiding fasting, and minimizing energy production from long-chain fatty acids. We report the effects of various dietary manipulations on plasma and urinary laboratory values in a child with LCHADD. In our patient, a diet restricted to 9% of total energy from long-chain fatty acids and administration of 1.5 g medium-chain triglyceride oil per kg body weight normalized plasma acylcarnitine and lactate levels, but dicarboxylic acid excretion remained approximately ten times normal. Plasma docosahexaenoic acid (DHA, 22:6n-3) was consistently low over a 2-year period; DHA deficiency may be related to the development of pigmentary retinopathy seen in this patient population. We also conducted a survey of metabolic physicians who treat children with LCHADD to determine current dietary interventions employed and the effects of these interventions on symptoms of this disease. Survey results indicate that a diet low in long-chain fatty acids, supplemented with medium-chain triclyceride oil, decreased the incidence of hypoketotic hypoglycaemia, and improved hypotonia, hepatomegaly, cardiomyopathy, and lactic acidosis. However, dietary treatment did not appear to effect peripheral neuropathy, pigmentary retinopathy or myoglobinuria.  (+info)

Human brain short chain L-3-hydroxyacyl coenzyme A dehydrogenase is a single-domain multifunctional enzyme. Characterization of a novel 17beta-hydroxysteroid dehydrogenase. (5/341)

Human brain short chain L-3-hydroxyacyl-CoA dehydrogenase (SCHAD) was found to catalyze the oxidation of 17beta-estradiol and dihydroandrosterone as well as alcohols. Mitochondria have been demonstrated to be the proper location of this NAD+-dependent dehydrogenase in cells, although its primary structure is identical to an amyloid beta-peptide binding protein reportedly associated with the endoplasmic reticulum (ERAB). This fatty acid beta-oxidation enzyme was identified as a novel 17beta-hydroxysteroid dehydrogenase responsible for the inactivation of sex steroid hormones. The catalytic rate constant of the purified enzyme was estimated to be 0.66 min-1 with apparent Km values of 43 and 50 microM for 17beta-estradiol and NAD+, respectively. The catalytic efficiency of this enzyme for the oxidation of 17beta-estradiol was comparable with that of peroxisomal 17beta-hydroxysteroid dehydrogenase type 4. As a result, the human SCHAD gene product, a single-domain multifunctional enzyme, appears to function in two different pathways of lipid metabolism. Because the catalytic functions of human brain short chain L-3-hydroxyacyl-CoA dehydrogenase could weaken the protective effects of estrogen and generate aldehydes in neurons, it is proposed that a high concentration of this enzyme in brain is a potential risk factor for Alzheimer's disease.  (+info)

Absence of spontaneous peroxisome proliferation in enoyl-CoA Hydratase/L-3-hydroxyacyl-CoA dehydrogenase-deficient mouse liver. Further support for the role of fatty acyl CoA oxidase in PPARalpha ligand metabolism. (6/341)

Peroxisomes contain a classical L-hydroxy-specific peroxisome proliferator-inducible beta-oxidation system and also a second noninducible D-hydroxy-specific beta-oxidation system. We previously generated mice lacking fatty acyl-CoA oxidase (AOX), the first enzyme of the L-hydroxy-specific classical beta-oxidation system; these AOX-/- mice exhibited sustained activation of peroxisome proliferator-activated receptor alpha (PPARalpha), resulting in profound spontaneous peroxisome proliferation in liver cells. These observations implied that AOX is responsible for the metabolic degradation of PPARalpha ligands. In this study, the function of enoyl-CoA hydratase/L-3-hydroxyacyl-CoA dehydrogenase (L-PBE), the second enzyme of this peroxisomal beta-oxidation system, was investigated by disrupting its gene. Mutant mice (L-PBE-/-) were viable and fertile and exhibited no detectable gross phenotypic defects. L-PBE-/- mice showed no hepatic steatosis and manifested no spontaneous peroxisome proliferation, unlike that encountered in livers of mice deficient in AOX. These results indicate that disruption of classical peroxisomal fatty acid beta-oxidation system distal to AOX step does not interfere with the inactivation of endogenous ligands of PPARalpha, further confirming that the AOX gene is indispensable for the physiological regulation of this receptor. The absence of appreciable changes in lipid metabolism also indicates that enoyl-CoAs, generated in the classical system in L-PBE-/- mice are diverted to D-hydroxy-specific system for metabolism by D-PBE. When challenged with a peroxisome proliferator, L-PBE-/- mice showed increases in the levels of hepatic mRNAs and proteins that are regulated by PPARalpha except for appreciable blunting of peroxisome proliferative response as compared with that observed in hepatocytes of wild type mice similarly treated. This blunting of peroxisome proliferative response is attributed to the absence of L-PBE protein in L-PBE-/- mouse liver, because all other proteins are induced essentially to the same extent in both wild type and L-PBE-/- mice.  (+info)

Unique multifunctional HSD17B4 gene product: 17beta-hydroxysteroid dehydrogenase 4 and D-3-hydroxyacyl-coenzyme A dehydrogenase/hydratase involved in Zellweger syndrome. (7/341)

Six types of human 17beta-hydroxysteroid dehydrogenases catalyzing the conversion of estrogens and androgens at position C17 have been identified so far. The peroxisomal 17beta-hydroxysteroid dehydrogenase type 4 (17beta-HSD 4, gene name HSD17B4) catalyzes the oxidation of estradiol with high preference over the reduction of estrone. The highest levels of 17beta-HSD 4 mRNA transcription and specific activity are found in liver and kidney followed by ovary and testes. A 3 kb mRNA codes for an 80 kDa (737 amino acids) protein featuring domains which are not present in the other 17beta-HSDs. The N-terminal domain of 17beta-HSD 4 reveals only 25% amino acid similarity with the other types of 17beta-HSDs. The 80 kDa protein is N-terminally cleaved to a 32 kDa enzymatically active fragment. Both the 80 kDa and the N-terminal 32 kDa (amino acids 1-323) protein are able to perform the dehydrogenase reaction not only with steroids at the C17 position but also with D-3-hydroxyacyl-coenzyme A (CoA). The enzyme is not active with L-stereoisomers. The central part of the 80 kDa protein (amino acids 324-596) catalyzes the 2-enoyl-acyl-CoA hydratase reaction with high efficiency. The C-terminal part of the 80 kDa protein (amino acids 597-737) facilitates the transfer of 7-dehydrocholesterol and phosphatidylcholine between membranes in vitro. The HSD17B4 gene is stimulated by progesterone, and ligands of PPARalpha (peroxisomal proliferator activated receptor alpha) such as clofibrate, and is down-regulated by phorbol esters. Mutations in the HSD17B4 lead to a fatal form of Zellweger syndrome.  (+info)

A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. (8/341)

BACKGROUND: Acute fatty liver of pregnancy and the HELLP syndrome (hemolysis, elevated liver-enzyme levels, and a low platelet count) are serious hepatic disorders that may occur during pregnancy in women whose fetuses are later found to have a deficiency of long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase. This enzyme resides in the mitochondrial trifunctional protein, which also contains the active site of long-chain 2,3-enoyl-CoA hydratase and long-chain 3-ketoacyl-CoA thiolase. We undertook this study to determine the relation between mutations in the trifunctional protein in infants with defects in fatty-acid oxidation and acute liver disease during pregnancy in their mothers. METHODS: In 24 children with 3-hydroxyacyl-CoA dehydrogenase deficiency, we used DNA amplification and nucleotide-sequence analyses to identify mutations in the alpha subunit of the trifunctional protein. We then correlated the results with the presence of liver disease during pregnancy in the mothers. RESULTS: Nineteen children had a deficiency only of long-chain 3-hydroxyacyl-CoA dehydrogenase and presented with hypoketotic hypoglycemia and fatty liver. In eight children, we identified a homozygous mutation in which glutamic acid at residue 474 was changed to glutamine. Eleven other children were compound heterozygotes, with this mutation in one allele of the alpha-subunit gene and a different mutation in the other allele. While carrying fetuses with the Glu474Gln mutation, 79 percent of the heterozygous mothers had fatty liver of pregnancy or the HELLP syndrome. Five other children, who presented with neonatal dilated cardiomyopathy or progressive neuromyopathy, had complete deficiency of the trifunctional protein (loss of activity of all three enzymes). None had the Glu474Gln mutation, and none of their mothers had liver disease during pregnancy. CONCLUSIONS: Women with acute liver disease during pregnancy may have a Glu474Gln mutation in long-chain hydroxyacyl-CoA dehydrogenase. Their infants are at risk for hypoketotic hypoglycemia and fatty liver.  (+info)

Purpose: : Long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) participates in the mitochondrial fatty acid oxidation. Genetic fatty acid oxidation defects induce cellular energetic deficiency, and thus early life threatening manifestations. An appropriate diet prevents those severe manifestations in organs that consume much fatty acid for their energetic needs. LCHAD deficiency is the only mitochondrial fatty acid oxidation deficiency to induce moreover a chorioretinopathy. The attempt consists in an atrophic degeneration predominating at the posterior pole and begins histologically at the level of retinal pigment epithelium. What is the pathogenesis of this specific chorioretinal degeneration ? Methods: : Reviewing of literature and biochemical mechanisms analysis were combined. Results: : LCHAD deficiency chorioretinopathy appears linked to toxic accumulation of 3-hydroxyacyl-carnitine rather than mitochondrial energetic defect. 3-hydroxyacyl, carried by l-carnitine, is the only hydroxylated ...
This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene of this gene on chromosome 15. [provided by RefSeq, May 2010 ...
Metabolic & Genetic Information Center Inborn erros of metabolism 3-HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY (SCHAD) HADH DEFICIENCY, SCHAD DEFICIENCY, FORMERLY
The focus of this thesis is to investigate the intracellular protein-peptide complex 3-hydroxyacyl-CoA dehydrogenase (HADH), also known as ABAD (amyloid- binding alcohol dehydrogenase) and amyloid-beta peptide (Aβ). This complex has been identified in the development of Alzheimers disease (AD), and this study tries to identify if ABAD is a useful biomarker for genetic risk profiling strategies for the early diagnosis of Alzheimers disease, or a suitable target for disease-modifying drug development. The major aim of this project is to explore the biochemical and cellular processes activated as a result of the interaction of ABAD and Aβ. Understanding the cellular responses to these interactions could help identify important biomarkers and/or drug targets for the diagnosis or treatment of Alzheimers disease. This study assesses the cytotoxic effects of Aβ in tissue culture and in animal models overexpressing ABAD. An in vitro cell system using SK-N-SH cells was developed for investigating ...
Macaca fascicularis brain cDNA clone: QorA-10176, similar to human L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain(HADHSC), mRNA, RefSeq: NM_005327.1 {ECO:0000313,EMBL:BAE87864.1 ...
Complete information for HADH gene (Protein Coding), Hydroxyacyl-CoA Dehydrogenase, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Oppermann UC, Salim S, Tjernberg LO, Terenius L, Jörnvall H. Binding of amyloid beta-peptide to mitochondrial hydroxyacyl-CoA dehydrogenase (ERAB): regulation of an SDR enzyme activity with implications for apoptosis in Alzheimers disease ...
Gentaur molecular products has all kinds of products like :search , ATGen \ HADH, 13_314aa, Human, His tag, E.coli \ ATGP0501 for more molecular products just contact us
Star Lotulelei, one of the elite prospects for the 2013 NFL draft, will not be allowed to work out Monday at the scouting combine after an echocardiogram revealed that the former Utah defensive tackle has a heart condition that requires more testing, according to league sources. Lotuleleis agent, Bruce Tollner, confirmed to ESPNs Joe Schad that the first team All-American had an abnormal test result as part of his physical and will visit a specialist this week. Lotulelei was discovered to
If you know of any papers that use this antibody, please contact us at antibodies [at] alzforum [dot] org for consideration in the References section.. ...
マウス・モノクローナル抗体 ab10260 交差種: Hu 適用: WB,ELISA,IHC-P,IHC-Fr,Dot,ICC/IF…ERAB抗体一覧…画像、プロトコール、文献などWeb上の情報が満載のアブカムの Antibody…
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?
Catalyzes the formation of a hydroxyacyl-CoA by addition of water on enoyl-CoA. Also exhibits 3-hydroxyacyl-CoA epimerase and 3-hydroxyacyl-CoA dehydrogenase activities. Strongly involved in the anaerobic degradation of long and medium-chain fatty acids in the presence of nitrate and weakly involved in the aerobic degradation of long-chain fatty acids.
PURPOSE. To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency - a life-threatening metabolic disease - and the relation to age at diagnosis, treatment and other clinical parameters.. METHODS. Ten children with LCHAD deficiency underwent repeated ophthalmological evaluations including ERG.. RESULTS. All 10 children developed chorioretinal pathology. Regardless of age at diagnosis, initiation of treatment and age at examination, inter-individual differences were present. Profound chorioretinal atrophy, severe visual impairment and progressive myopia had developed in two teenagers. Milder chorioretinopathy with or without subnormal visual acuity was present in all other children. ERG was pathological in seven children. The chorioretinopathy often started in the peripapillary or perimacular areas. In one patient, unilateral visual impairment was associated with fibrosis.. CONCLUSION. Early ...
The purpose of this study was to determine the effect of different fat type of high fat diets on enzyme activities in rat skeletal muscle. Fifty-six male Wistar rats were fed one of four diets: low fat (12% calories as fat), lard (60% calories as fat), safflower oil (60% calories as fat) or fish oil (60% calories as fat). They ate their diets ad libitum for 5 weeks. Each group was further divided into sedentary and exercise trained groups. There were no significant differences in body weight among each diet group. Epidydimal adipose tissue weight was higher in lard and safflower oil groups than in low fat. Endurance training decreased body weight and adipose tissue weight in each diet group. Plasma leptin were also significantly higher in lard and safflower oil groups than in low fat group. Safflower and fish oil diet induced significant (P , 0.05) increases in citrate synthase (∼30% and ∼35%, respectively) and 3 hydroxyacyl CoA dehydrogenase activities (∼58%) in red portion of ...
If a metabolic crisis is not treated, breathing problems, seizures, coma, brain damage and sometimes death can occur.. Between episodes of metabolic crisis, babies with LCHAD and TFP may not show any signs of the disease. Other babies with LCHAD or TFP may have problems with their heart, liver and muscles.. Screening and treatment aim to prevent metabolic crises and other symptoms and help children with LCHAD and TFP to lead the healthiest lives possible.. ...
Involved in the aerobic and anaerobic degradation of long-chain fatty acids via beta-oxidation cycle. Catalyzes the formation of 3-oxoacyl-CoA from enoyl-CoA via L-3-hydroxyacyl-CoA. It can also use D-3-hydroxyacyl-CoA and cis-3-enoyl-CoA as substrate.
InterPro provides functional analysis of proteins by classifying them into families and predicting domains and important sites. We combine protein signatures from a number of member databases into a single searchable resource, capitalising on their individual strengths to produce a powerful integrated database and diagnostic tool.
All the info about Dominican republic company TERMINALES SCHAD (133125). Company ranked 3.13 out of 5. It is possible that in this page you will find TERMINALES SCHAD address, fax, phone, e-mail, website and other information. (mobile version)
The PDB archive contains information about experimentally-determined structures of proteins, nucleic acids, and complex assemblies. As a member of the wwPDB, the RCSB PDB curates and annotates PDB data according to agreed upon standards. The RCSB PDB also provides a variety of tools and resources. Users can perform simple and advanced searches based on annotations relating to sequence, structure and function. These molecules are visualized, downloaded, and analyzed by users who range from students to specialized scientists.
To elucidate the molecular mechanisms behind physical inactivity-induced insulin resistance in skeletal muscle, 12 young, healthy male subjects completed 7 days of bed rest with vastus lateralis muscle biopsies obtained before and after. In six of the subjects, muscle biopsies were taken from both legs before and after a 3-h hyperinsulinemic euglycemic clamp performed 3 h after a 45-min, one-legged exercise. Blood samples were obtained from one femoral artery and both femoral veins before and during the clamp. Glucose infusion rate and leg glucose extraction during the clamp were lower after than before bed rest. This bed rest-induced insulin resistance occurred together with reduced muscle GLUT4, hexokinase II, protein kinase B/Akt1, and Akt2 protein level, and a tendency for reduced 3-hydroxyacyl-CoA dehydrogenase activity. The ability of insulin to phosphorylate Akt and activate glycogen synthase (GS) was reduced with normal GS site 3 but abnormal GS site 2+2a phosphorylation after bed rest. ...
Alzheimers disease (AD) is a conformational disease that is characterized by amyloid-β (Aβ) deposition in the brain. Aβ exerts its toxicity in part by receptor-mediated interactions that cause down-stream protein misfolding and aggregation, as well as mitochondrial dysfunction. Recent reports indicate that Aβ may also interact directly with intracellular proteins such as the mitochondrial enzyme ABAD (Aβ binding alcohol dehydrogenase) in executing its toxic effects. Mitochondrial dysfunction occurs early in AD, and Aβs toxicity is in part mediated by inhibition of ABAD as shown previously with an ABAD decoy peptide. Here, we employed AG18051, a novel small ABAD-specific compound inhibitor, to investigate the role of ABAD in Aβ toxicity. Using SH-SY5Y neuroblastoma cells, we found that AG18051 partially blocked the Aβ-ABAD interaction in a pull-down assay while it also prevented the Aβ42-induced down-regulation of ABAD activity, as measured by levels of estradiol, a known hormone and ...
As you can see the once weekly workouts had a significant impact on both the parameters of glucose management and the cardiovascular and muscular fitness parameters (see Figure 1). The additional skeletal muscle biopsy samples the scientist obtained before and 72 h after training revealed that the above changes went hand in hand with an increase in maximal activity of citrate synthase and protein content of cytochrome oxidase 4 (p , 0.01, main effect) and increases in the maximal activity of b-hydroxy acyl CoA dehydrogenase in men only (p , 0.05 ...
As a starting point for evaluating a broader range of conditions for H2 oxidation complexes, in this work we investigate an efficient and reversible Ni-based H2 oxidation and production complex with an arginine in the outer ...
One year ago, the now-famous E. coli outbreak arising from contaminated spinach rattled the natural-food industry and gave carnivores a moment of schad ...
Adlung, Lorenz; Sabah, Jude Al; Bayer, Philipp; Berrens, Rebecca; Cristiano, Elena; Flocke, Lea; Kleinsorg, Stefan; Kolodziejczyk, Aleksandra; Kraemer, Stephen; Torre, Alejandro Macias; Mathur, Aastha; Mayilo, Dmytro; Neumann, Stefan; Niopek, Dominik; Pisa, Rudolf; Schad, Jan-Ulrich; Schumacher, Laura-Nadine; Uhlig, Thomas; Wu, Xiaoting; Keienburg, Jens; Boerner, Kathleen; Grimm, Dirk; Eils, Roland (2010-12-05) ...
Complete information for HACL1 gene (Protein Coding), 2-Hydroxyacyl-CoA Lyase 1, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Mouse Monoclonal Anti-HADH Antibody (4B5) [DyLight 488]. Validated: WB, ELISA, ICC/IF, IHC, IHC-P, IP. Tested Reactivity: Human. 100% Guaranteed.
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?
Benzoyl-CoA is a common intermediate in the anaerobic bacterial metabolism of many aromatic substrates. Two enzymes and ferredoxin of the central benzoyl-CoA pathway in Thauera aromatica have been purified so far. Benzoyl-CoA reductase reduces the aromatic ring with reduced ferredoxin yielding cyclohexa-1,5-diene-1-carbonyl-CoA [Boll, M. & Fuchs, G. (1995) Eur. J. Biochem. 234, 921-933]. Dienoyl-CoA hydratase subsequently adds one molecule of water and thereby produces 6-hydroxycyclohex-1-ene-1-carbonyl-CoA [Laempe, D., Eisenreich, W., Bacher, A., & Fuchs, G. (1998) Eur. J. Biochem. 255, 618-627]. Here two new enzymes, which convert this intermediate to the noncyclic product 3-hydroxypimelyl-CoA, were purified from T. aromatica and studied. 6-Hydroxycyclohex-1-ene-1-carbonyl-CoA dehydrogenase is an NAD(+)-specific beta-hydroxyacyl-CoA dehydrogenase that catalyzes 6-hydroxycyclohex-1-ene-1-carbonyl-CoA + NAD(+) --| 6-oxocyclohex-1-ene-1-carbonyl-CoA + NADH + H(+). 6-Oxocyclohex-1-ene-1-carbonyl-CoA
Disorders of mitochondrial fatty acid oxidation with an emphasis on control of insulin secretion by short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. The Neuronal-Ceroid Lipofuscinoses. A study of the function of CLN3P, the protein responsible for Juvenile Batten Disease.. Clinical ...
Disorders of mitochondrial fatty acid oxidation with an emphasis on control of insulin secretion by short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. The Neuronal-Ceroid Lipofuscinoses. A study of the function of CLN3P, the protein responsible for Juvenile Batten Disease.. Clinical ...
SWISS-MODEL Repository entry for B5BIZ0 (FADB_SALPK), Fatty acid oxidation complex subunit alpha. Salmonella paratyphi A (strain AKU_12601)
Ross MT, Grafham DV, Coffey AJ, Scherer S, McLay K, Muzny D, Platzer M, Howell GR, Burrows C, Bird CP, Frankish A, Lovell FL, Howe KL, Ashurst JL, Fulton RS, Sudbrak R, Wen G, Jones MC, Hurles ME, Andrews TD, Scott CE, Searle S, Ramser J, Whittaker A, Deadman R, Carter NP, Hunt SE, Chen R, Cree A, Gunaratne P, Havlak P, Hodgson A, Metzker ML, Richards S, Scott G, Steffen D, Sodergren E, Wheeler DA, Worley KC, Ainscough R, Ambrose KD, Ansari-Lari MA, Aradhya S, Ashwell RI, Babbage AK, Bagguley CL, Ballabio A, Banerjee R, Barker GE, Barlow KF, Barrett IP, Bates KN, Beare DM, Beasley H, Beasley O, Beck A, Bethel G, Blechschmidt K, Brady N, Bray-Allen S, Bridgeman AM, Brown AJ, Brown MJ, Bonnin D, Bruford EA, Buhay C, Burch P, Burford D, Burgess J, Burrill W, Burton J, Bye JM, Carder C, Carrel L, Chako J, Chapman JC, Chavez D, Chen E, Chen G, Chen Y, Chen Z, Chinault C, Ciccodicola A, Clark SY, Clarke G, Clee CM, Clegg S, Clerc-Blankenburg K, Clifford K, Cobley V, Cole CG, Conquer JS, Corby N, ...
Transient overexpression lysate of hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit (HADHA), nuclear gene encoding mitochondrial protein ...
If a metabolic crisis is not treated, breathing problems, seizures, coma, brain damage and sometimes death can occur.. Between episodes of metabolic crisis, babies with VLCAD may not show any signs of the disease. Other babies with VLCAD may have problems with their heart, liver and muscles.. Screening and treatment aim to prevent metabolic crises and other symptoms and help children with VLCAD to lead the healthiest lives possible.. ...
Enoyl Coenzyme A hydratase, short chain, 1, mitochondrial, also known as ECHS1, is a human gene. The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. The gene product is a member of the hydratase/isomerase superfamily. It localizes to the mitochondrial matrix. Transcript variants utilizing alternative transcription initiation sites have been described in the literature. The ECHS1 gene is approximately 11 kb in length, and is composed of eight exons, with exons I and VIII containing the 5- and 3-untranslated regions, respectively. There are two major transcription start sites, located 62 and 63 bp upstream of the translation codon, were mapped by primer extension analysis. The 5-flanking region of the ECHS1 gene is GC-rich and contains several copies of the SP1 binding motive but no typical TATA or CAAT boxes are apparent. Alu ...
3-hydroxyacyl-CoA dehydrogenase / enoyl-CoA hydratase / 3-hydroxybutyryl-CoA epimerase / enoyl-CoA isomerase [EC:1.1.1.35 4.2.1.17 5.1.2.3 5.3.3.8 ...
3-hydroxyacyl-CoA dehydrogenase / enoyl-CoA hydratase / 3-hydroxybutyryl-CoA epimerase / enoyl-CoA isomerase [EC:1.1.1.35 4.2.1.17 5.1.2.3 5.3.3.8 ...
From NCBI Gene:. The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. The gene product is a member of the hydratase/isomerase superfamily. It localizes to the mitochondrial matrix. Transcript variants utilizing alternative transcription initiation sites have been described in the literature. [provided by RefSeq, Jul 2008]. From UniProt: ...
Murderpedia, the free online encyclopedic dictionary of murderers. The largest database about serial killers, mass murderers and spree killers around the world
In LCHAD deficiency, specific missense mutations within the alpha subunit (HADA) cause the disease. Clinical follow-up after a positive screen involves further testing to confirm diagnosis. If diagnosis is confirmed contact and educate the family.
SEDIMENTOLOGY: The ichnoassemblages of the Abad Member (Tortonian-Messinian), Vera Basin, SE Spain: implications for the regional tectonic and palaeogeographical evolution - Volume 155 Issue 6 - S. RÜTTERS, T. McCANN
Hello all, In the following you see visibility predictions for the ISS generated with element sets 11 and 4 days old (from Ray Hoad) and new ones (from space-track). Differences of about one and a half minutes do occur. Even the orbit number changes ! Either you use the newest data or you should be patient ;) Vy 73, Viktor OE1VKW -------------------------------------------------------------------------------- Element Set Number: 105 (Orbit 70873) Element Set Epoch : 31Mar11 21:27:39.138 UTC Date (UTC) Time (UTC) of Duration Azimuth at Peak Vis Orbit AOS MEL LOS of Pass AOS MEL LOS Elev Mon 11Apr11 20:58:54 21:02:49 21:06:47 00:07:53 188 135 81 9.8 NNN 71046 22:32:41 22:37:34 22:42:27 00:09:46 235 150 69 56.6* NNN 71047 -------------------------------------------------------------------------------- Element Set Number: 150 (Orbit 70981) Element Set Epoch : 07Apr11 18:09:13.138 UTC Date (UTC) Time (UTC) of Duration Azimuth at Peak Vis Orbit AOS MEL LOS of Pass AOS MEL LOS Elev Mon 11Apr11 20:59:48 ...
Hedden, Peter, Hoad, Gordon V., Gaskin, Paul, Lewis, Mervyn J., Green, Julia, Furber, Mark and Mander, Lewis N. (1993) Kaurenoids and Gibberellins, including the newly characterized Gibberellin A88, in developing apple seeds. Phytochemistry, 32 (2). pp. 231-237. ISSN 0031-9422 ...
A severe thunderstorm warning issued for parts of Los Angeles County has expired, but forecasters say showers and isolated thunderstorms are expected to continue into Wednesday.
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 ...
6.. Fanin M, Anichini A, Cassandrini D, Fiorillo C, Scapolan S, Minetti C, Cassanello M, Donati MA, Siciliano G, DAmico A, Lilliu F, Bruno C, Angelini C (2012) Allelic and phenotypic heterogeneity in 49 Italian patients with the muscle form of CPT-II deficiency. Clin Genet 82:232-239. https://doi.org/10.1111/j.1399-0004.2011.01786.x ...
Free, official coding info for 2018 ICD-10-CM E71.311 - includes detailed rules, notes, synonyms, ICD-9-CM conversion, index and annotation crosswalks, DRG grouping and more.
Metabolic networks are extensively regulated to facilitate tissue-specific metabolic programs and robustly maintain homeostasis in response to dietary changes. Homeostatic metabolic regulation is achieved through metabolite sensing coupled to feedback regulation of metabolic enzyme activity or expression. With a wealth … Continue reading →. ...
C.M. Wacker, A.W. Hartlep, M. Bock, G. van Kaick, G. Ertl, W.R. Bauer and L.R. Schad, Deutsches Krebsforschungszentrum (DKFZ), Heidelberg, Germany and Universitt Mannheim/Heidelberg, Mannheim, Germany ...
Pathway:Human:Mitochondrial fatty acid betaoxidation]] moved to [[Pathway:Homo sapiens:Mitochondrial fatty acid betaoxidation]]: Renaming ...
The graph above shows the effect of 3% DMSO on YBR033W. From the data represented the average doubling rate for the YBR033W knockout strain without DMSO was 634 minutes. When DMSO is added the average doubling time increased to 1292 minutes. This shows that 3% DMSO did add stress to this knockout strain. The average doubling time of WT:BY4735 without DMSO was 514 minutes. The average doubling rate for WT:BY4735 with DMSO was 1529 minutes. If we compare the average doubling rate of WT:BY4735 with DMSO to YBR033W with DMSO we can see that without the YBR033W gene the yeast was able to grow more quickly under DMSO induced stress. ...
ABear ACourt ADean ABear ACourt ADean Aabend Aach Aachen Aadio Aadland Aadnesen Aafedt Aafidt Aafitt Aagaard Aagesen Aaglan Aagland Aagotnes Aag rd Aakal Aaker Aal Aalbrecht Aalbregt Aalbregts Aalbregtse Aalbue Aalfs Aalseth Aalvik Aamodt Aanda Aanondsen Aanonsen Aantjes Aar Aarbo Aardewijn Aarhusiander Aarhusianer Aarnes Aarness Aarnio Aaron Aaronov Aaronovitz Aarons Aaronsen Aaronsohn Aaronson Aarstad Aartsen Aasberg Aase Aasen Aasgard Aasheim Aaslie Aba Abaceta Abache Abachea Abachi Abad Abada Abadal Abadam Abade Abadechea Abades Abadessa Abadi Abadia Abadiano Abadie Abady Abaiga Abaigar Abainza Abaiov Abaitua Abajian Abajo Abal Abalain Abalan Abalcisqueta Abalia Aballain Aballan Aballay Aballe Abalon Abalos Abalotta Abanades Abanathey Abando Abandond Abano Abao Abarbanel Abarca Abarca De Bolea Abarcrumby Abarelo Abargues Abaria Abario Abarisqueta Abarizqueta Abarno Abaroa Abarough Abarquero Abarrategui Abarta Abarthur Abartur Abartus Abarza Abarzua Abarzuza Abas Abascal Abasgoitia ...
Need some antibodies - posted in Immunology: I need antibody against LCHAD, MTP/TFP, VLCAD, SCHAD, MCAD, and dont 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!!!
Metabolic & Genetic Information Center Inborn erros of metabolism MITOCHONDRIAL TRIFUNCTIONAL PROTEIN DEFICIENCY (MTPD) HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, ALPHA SUBUNIT HADHA
Human trifunctional protein catalyzes three steps in mitochondrial beta-oxidation of fatty acids, including the long chain 3-hydroxyacyl-CoA dehydrogenase step. Deficiency of this heterocomplex, which contains 4 alpha and 4 beta subunits, causes sudden unexplained infant death, a Reye-like syndrome, cardiomyopathy, or skeletal myopathy. We determined the molecular basis of this deficiency in a patient with neonatal presentation and later sudden death using reverse transcription and PCR amplification of his alpha subunit mRNA. We demonstrated a universal deletion of exon 3 (71 bp) in his mRNA. This deletion causes a frameshift and very early premature termination. Amplification of genomic DNA demonstrated that the patient was a compound heterozygote with two different mutations in the 5 donor splice site following exon 3: a paternally inherited G to A transversion at the invariant position +1 and a maternally inherited A to G mutation at position +3. Both allelic mutations apparently cause exon ...
Enoyl-coenzyme A hydratase/3-hydroxyacyl-coenzyme A (EHHADH) is an important enzyme which catalyze two steps in fatty acid oxidation. There are four acetylated lysine residues been identified in EHHADH, which are Lys165, Lys171, Lys346 and Lys584. Immunoprecipitation of ectopically expressed FLAG-tagged EHHADH and Western blotting with antibody to acetyllysine confirmed that EHHADH was indeed acetylated(Zhao, et al). In order to explore the effect of acetylation on fatty acid oxidation. Isobaric tags are used, which is TSA and NAM. TSA and NAM treatment increased all the four lysine residues acetylation. Consistently, corresponding unacetylated peptide was decreased. Scientists treat TSA and NAM to Chang Human Liver cells doubled the activity of EHHADH, which indicates that acetylation of EHHADH would increase fatty acid oxidation pathway. In order to confirm the result, site-directed mutagenesis was used and the four lysine residue was replaced by glutamine, TSA and NAM can no longer ...
Very long chain fatty acids (VLCFAs) are involved in plant development and particularly in several cellular processes such as membrane trafficking, cell division and cell differentiation. However, the precise role of VLCFAs in these different cellular processes is still poorly understood in plants. In order to identify new factors associated with the biosynthesis or function of VLCFAs, a yeast multicopy suppressor screen was carried out in a yeast mutant strain defective for fatty acid elongation. Loss of function of the elongase 3 hydroxyacyl-CoA dehydratase PHS1 in yeast and PASTICCINO2 in plants prevents growth and induces cytokinesis defects. PROTEIN TYROSIN PHOSPHATASE-LIKE (PTPLA) previously characterized as an inactive dehydratase was able to restore yeast phs1 growth and VLCFAs elongation but not the plant pas2-1 defects. PTPLA interacted with elongase subunits in the Endoplasmic Reticulum (ER) and its absence induced the accumulation of 3-hydroxyacyl-CoA as expected from a dehydratase involved
Anti- HADHA Antibody product for laboratory research available in multiple sizes: 100ug ; the related gene is: HADHA - hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha , please call us for more info about this product catalog number: GEN8505836 . Gentaur: November 19, 2019, 5:37 pm
Fill in any or all of the fields below. Click on the label to the left of each search field for more information or read the Help ...
OBJECTIVE The aim of this study was to explore the genetic features of a family with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (MHBDD) which may provide the basis for the diagnosis and genetic counseling. METHOD Clinical data of the proband was collected, total RNA and genomic DNA were extracted from the peripheral blood. The whole coding region of the ACAT1 gene was amplified by RT-PCR. 5 noncoding region of the ACAT1 gene and all 6 exons and flanking intron regions of the HADH2 gene were amplified by PCR. All amplification products were directly sequenced and compared with the reference sequence. RESULT (1) The patient was a one-year-old boy who presented with psychomotor retardation and astasia when he was admitted to the hospital. Biochemical test revealed slight hyperlactatemia (3.19 mmol/L) and magnetic resonance imaging showed delayed myelination. 2-Methylacetoacetyl-CoA thiolase deficiency was suggested by gas chromatography-mass spectrometry. (2) There was no mutation in the
Yeast co-immunoprecipitation dataset ; Gavin et al, 2006 YBR119W YPL178W YBR119W YML046W YBR119W YKL012W YBR119W YDR235W YBR119W YIL061C YBR119W YDR240C YBR119W YGR013W YBR119W YMR125W YBR152W YER172C YBR152W YMR240C YBR152W YMR288W YBR152W YPL213W YBR152W YIR009W YBR152W YDL043C YBR152W YJL203W YBR152W YDR473C YBR152W YGR091W YBR152W YGR075C YBR152W YPR178W YBR152W YBR055C YBR152W YHR165C YBR152W YDL030W YBR152W YML049C YBR152W YER029C YBR152W YKL173W YBR152W YDL098C YBR152W YOR308C YDL087C YER172C YDL087C YMR288W YDL087C YHR086W YDL087C YER165W YDL087C YML046W YDL087C YKL012W YDL087C YDR235W YDL087C YHR165C YDL087C YML049C YDL087C YER029C YDL087C YLR275W YDL087C YLR147C YDL087C YIL061C YDL087C YKL173W YDL087C YDR240C YDL087C YGR013W YDL087C YMR125W YDL087C YGR162W YDL087C YLR298C YDL175C YJL050W YDL175C YPL190C YDL175C YOL115W YDR235W YHR086W YDR235W YML046W YDR235W YKL012W YDR235W YIL061C YDR235W YGR013W YDR235W YMR125W YDR240C YHR086W YDR240C YML046W YDR240C YDR235W YDR240C YHR165C YDR240C ...
Complete fatty acid beta-oxidation pathway for saturated and unsaturated fatty acids, developed and curated internally by BiGCaT Bioinformatics. This pathway was previously split into three parts plus a meta file for statistics. If you still have these you can replace all four with this single pathway ...
Expression of HSD17B10 (17b-HSD10, ABAD, CAMR, ERAB, HADH2, MHBD, MRPP2, MRXS10, SDR5C1) in smooth muscle tissue. Antibody staining with HPA001432 in immunohistochemistry.
Protein deficiency is surprisingly common. Learn about the unexpected signs of protein deficiency, and several ways to get back on track.
Learn about the signs you dont eat enough protein. From loss of muscle mass to weak immunity, weve listed 10 of the most common symptoms of protein deficiency.
ERAB antibody, C-term (hydroxysteroid (17-beta) dehydrogenase 10) for WB. Anti-ERAB pAb (GTX89602) is tested in Human samples. 100% Ab-Assurance.
ERAB antibody (hydroxysteroid (17-beta) dehydrogenase 10) for WB. Anti-ERAB pAb (GTX50767) is tested in Human, Mouse, Rat samples. 100% Ab-Assurance.
www.philippinessanfrancisco.org/news/4990/591/Ballot-Packets-marked-Return-to-Sender/d,phildet. Apr 21, 2016 ... Return To Sender. If your name is included in this list, it means that USPS attempted to deliver your ballot envelope but could not do so due to incomplete or invalid addresses. USPS returns these envelopes to the sender. Return To Sender - As of 06 May 2016. Abad, Carisse E. Abad, Theresa B.. ...
D-bifunctional enzyme deficiency (sometimes referred to as pseudo-Zellweger syndrome) is a genetic disorder typically characterized by hypotonia (low muscle tone) and seizures within the first month of life, vision and hearing problems, distinct facial features, and developmental delay. Some children with D-bifunctional enzyme deficiency also go on to develop liver disease and/or a progressive leukodystrophy. Most people who have D-bifunctional enzyme deficiency pass within the first 2 years of life; however, there have been a few reported cases of patients living beyond 2 years of life. Treatment is symptomatic and supportive. D-bifunctional enzyme deficiency is caused by mutations in the HSD17B4 gene and is believed to be inherited in an autosomal recessive fashion ...
Peroxisomal beta-oxidation is an essential step in bile acid synthesis, since it is required for shortening of C27-bile acid intermediates to produce mature C24-bile acids. D-Bifunctional protein (DBP) is responsible for the second and third step of this beta-oxidation process. However, both patients and mice with a DBP deficiency still produce C24-bile acids, although C27-intermediates accumulate. An alternative pathway for bile acid biosynthesis involving the peroxisomal L-bifunctional protein (LBP) has been proposed. We investigated the role of LBP and DBP in bile acid synthesis by analyzing bile acids in bile, liver, and plasma from LBP, DBP, and LBP:DBP double knock-out mice. Bile acid biosynthesis, estimated by the ratio of C27/C24-bile acids, was more severely affected in double knock-out mice as compared with DBP-/- mice but was normal in LBP-/- mice. Unexpectedly, trihydroxycholestanoyl-CoA oxidase was inactive in double knock-out mice due to a peroxisomal import defect, preventing us ...
Looking for online definition of 3-ketoacyl-CoA thiolase in the Medical Dictionary? 3-ketoacyl-CoA thiolase explanation free. What is 3-ketoacyl-CoA thiolase? Meaning of 3-ketoacyl-CoA thiolase medical term. What does 3-ketoacyl-CoA thiolase mean?
This pathway mainly shows the oxidation of fatty acids. The fatty acid oxidation takes place in mitochondria in animals. This is the reverse of fatty acid biosynthesis and utilises CoA as acyl carrier. The four main enzymes involved in the degradation of fatty acids are acyl-CoA oxidase (acyl-CoA dehydrogenase), Enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase and 3-oxoacyl-CoA thiolase. Each cycle of activities of these enzymes removes 2-carbon units in the form of acetyl-CoA. This cycle of activities can continue until the fatty acid chain is degraded to 4-carbon acetoacetyl-CoA. Acetoacetyl-CoA can then be cleaved to 2 acetyl-CoAs by the reverse action of the enzyme acetyl-CoA C-acetyltransferase.. This pathway may provide a carbon source in the form of acetyl-CoA for mitochondrial TCA cycle and other biosynthesis pathways. The enzymes acyl-CoA oxidase and 3-hydroxyacyl-CoA dehydrogenase are absent in Plasmodium falciparum. There is no biochemical evidence of this pathway taking place in ...
Mutations in this gene, along with mutations in HADHA, result in trifunctional protein deficiency.[5] Mutations in either gene have similar clinical presentations.[8] Trifunctional protein deficiency is characterized by decreased activity of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), long-chain enoyl-CoA hydratase, and long-chain thiolase. This deficiency can be classified into 3 main clinical phenotypes: neonatal onset of a severe, lethal condition resulting in sudden infant death syndrome (SIDS),[9] infantile onset of a hepatic Reye-like syndrome, and late-adolescent onset of primarily a skeletal myopathy.[10] Additionally, some presents showed symptoms associated with myopathy, recurrent and episodic rhabdomyolysis, and sensorimotor axonal neuropathy.[11] In some cases, symptoms of the deficiency can present as dilated cardiomyopathy, congestive heart failure, and respiratory failure. The deficiency has presented as hydrops fetalis and HELLP syndrome in fetuses.[12] A compound ...
HADHA - HADHA (untagged)-Human hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit (HADHA), nuclear gene encoding mitochondrial protein available for purchase from OriGene - Your Gene Company.
He said the study demonstrates that the enzyme is essential for fetal development and survival of the newborn. "Deficiency of mitochondrial trifunctional protein causes fetal growth retardation, neonatal hypoglycemia and sudden death," Ibdah said. "All are serious and common human disorders.". He said the mouse model provides mechanisms and understanding for the human disease. "It confirms that there is an association between impairment in breaking down fat and sudden death. It makes it clear cut that human beings after birth require these enzymes to survive.". Ibdah said that because the enzyme is missing, fatty acid products accumulate, producing a toxic effect that probably leads to heart arrythmias as well as respiratory arrest.. The mouse model also demonstrates that these enzymes are important for fetal growth and development. "When they are not present, there is fetal growth retardation," Ibdah said. Previously, he said, it had been thought that these enzymes werent needed by the fetus ...
This week was spent growing up massive E. coli cultures for cell harvest, which will eventually be used for more Mnx protein purification. This process of making competent cells to transform with the pASK vector that contains either a full manganese oxidation complex (MnxDG) or a partial complex (MnxEF), then growing the cells up, harvesting them at the optimal time for protein expression, then purifying the protein is long and complex. You need to pay close attention at every stage of culture growth because man do these babies grow up fast. The doubling time of E. ...
Cotarelo, Cristina L.; Schad, Arno; Kirkpatrick, Charles James; Sleeman, Jonathan P.; Springer, Erik; Schmidt, Marcus; Thaler, ...
Octanoate and palmitate beta-oxidation in human leukocytes: implications for the rapid diagnosis of fatty acid beta-oxidation disorders.:
1IQ6: Crystal structure of the (R)-specific enoyl-CoA hydratase from Aeromonas caviae involved in polyhydroxyalkanoate biosynthesis
A block of rooms has been set aside at the group rates shown below. These rates will also be available 3 days before and after the symposium if anyone would like to arrive early or stay longer to explore the many sights the area has to offer.. Guests can reserve a room(s) online (http://coa.st/w9tx) or by calling central reservations (1-800-716-6199).. Refer to the group name "Northern Wild Sheep & Goat Symposium" or group code "CCM-GFC4211". .tg {border-collapse:collapse;border-spacing:0;border-width:1px;border-style:solid;border-color:#9ABAD9;} .tg td{font-family:Arial, sans-serif;font-size:14px;padding:10px 20px;border-style:solid;border-width:0px;overflow:hidden;word-break:normal;border-color:#9ABAD9;color:#444;background-color:#EBF5FF;} .tg th{font-family:Arial, sans-serif;font-size:14px;font-weight:normal;padding:10px 20px;border-style:solid;border-width:0px;overflow:hidden;word-break:normal;border-color:#9ABAD9;color:#fff;background-color:#409cff;} .tg ...
Sisters were diagnosed with mitochondrial trifunctional protein deficiency, a rare condition that stops the body from converting fats to energy. One of the girls experienced a remarkable recovery.
Measuring proteins involved in glucose and fatty acid metabolism before and after an 8-wk VLED reflects the metabolic adaptations occurring in adipose tissue linked to energy expenditure. More specifically, the decrease of AldoC, an enzyme of glycolysis, is correlated with the decrease in AEE, and the non-significant change HADHsc, a crucial enzyme for mitochondrial beta-oxidation, is negatively correlated with the adaptation in REE. Furthermore, there is a correlation between the increase in FABP4, the intracellular fatty acid transporter, and the decrease in fat mass, and a correlation between the decrease in AldoC and the decrease in fat free mass. FABP4, AldoC and HADHsc are all positively correlated.. The increased FABP4 after the VLED weight loss is in accordance with previous results in obese subjects [11, 12, 44]. It is in line with an elevation in intracellular trafficking of fatty acids, which is expected during a negative energy balance when lipolysis is stimulated, with the release ...
Mitochondrial fatty-acid beta-oxidation (mFAO) plays a central role in mammalian energy metabolism. Multiple severe diseases are associated with defects in this pathway. Its kinetic structure is characterized by a complex wiring of which the functional implications have hardly been explored. Repetitive cycles of reversible reactions, each cycle shortening the fatty acid by two carbon atoms, evoke competition between intermediates of different chain lengths for a common set of promiscuous enzymes (enzymes with activity towards multiple substrates). In our validated kinetic model of the pathway, substrate overload causes a steep and detrimental flux decline. Here, we unravel the underlying mechanism and the role of enzyme promiscuity in it. Comparison of alternative model versions elucidated the role of promiscuity of individual enzymes. Promiscuity of the last enzyme of the pathway, medium-chain ketoacyl-CoA thiolase (MCKAT), was both necessary and sufficient to elicit the flux decline. ...
Individuals with deficiencies of these factors or platelets exhibit to bleed; they do not bleed more very likely than people without these conditions, but it is justifiable more difficult allowing for regarding the clot to form, and bleeding cannot be stopped easily. J Biol Chem 282:12377В-12387 Schurigt U, Schad C, Glowa C, Baum U, Thomale K, Schnitzer JK, Schultheis M, Schaschke N, Schirmeister T, Moll H (2010) Aziridine-2,3-dicarboxylate-based cysteine cathepsin inhibitors bring about apartment death in Leishmania crucial associated with accumulation of debris in autophagy- kindred lysosome-like vacuoles. What if benzol levels are really uttermost greater discount pilex 60 caps with mastercard prostate cancer oncologist. In physiological terms, interactions between natural networks result in changes chief to another form of equilibrium that okay better coping with the unfamiliar condition. Morbidity statistics are revised less frequently because of the formidableness in defining or obtaining ...
Meštrović, Nevenka; Pavlek, Martina; Car, Ana; Castagnone-Sereno, Philippe; Abad, Pierre; Plohl, Miroslav (2013) Conserved DNA motifs, including the CENP-B box-like, are involved in satellite DNA array rearrangements. PLoS ONE, 8 (6). e67328/1-e67328/10. ISSN 1932-6203 ...
Rabbit polyclonal ERAB antibody validated for WB, ELISA, IHC, ICC/IF and tested in Human. Immunogen corresponding to synthetic peptide
Top 10 cell-lines for Q96LK0 (Homo sapiens, UniProt): My-La CD4+, NCI-H1581, Sez-4, A2058, NCI-H841, MFE-280, MFE-296, SK-UT-1B, U-251MG-PARK2, Kasumi-6
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 ...
Background The origin of eukaryotes remains a fundamental question in evolutionary biology. Although it is clear that eukaryotic genomes are a chimeric combination of genes of eubacterial and archaebacterial ancestry, the specific ancestry of most eubacterial genes is still unknown. The growing availability of microbial genomes offers the possibility of analyzing the ancestry of eukaryotic genomes and testing previous hypotheses on their origins. Methodology/Principal Findings Here, we have applied a phylogenomic analysis to investigate a possible contribution of the Myxococcales to the first eukaryotes. We conducted a conservative pipeline with homologous sequence searches against a genomic sampling of 40 eukaryotic and 357 prokaryotic genomes. The phylogenetic reconstruction showed that several eukaryotic proteins traced to Myxococcales. Most of these proteins were associated with mitochondrial lipid intermediate pathways, particularly enzymes generating reducing equivalents with pivotal roles ...
Protein is one of three primary types of macronutrients that provide your body with energy, regulate its functioning and facilitate development. A protein...
The protein encoded by this gene, a member of the carnitine/choline acetyltransferase family, is the rate-controlling enzyme of the long-chain fatty acid beta-oxidation pathway in muscle mitochondria. This enzyme is required for the net transport of long-chain fatty acyl-CoAs from the cytoplasm into the mitochondria. Multiple transcript variants encoding different isoforms have been found for this gene, and read-through transcripts are expressed from the upstream locus that include exons from this gene. [provided by RefSeq, Jun 2009 ...
Amyloid-beta peptide (Abeta) binding alcohol dehydrogenase (ABAD), an enzyme present in neuronal mitochondria, is a cofactor facilitating Abeta-induced cell stress. We hypothesized that ABAD provides a direct link between Abeta and cytotoxicity via mitochondrial oxidant stress. Neurons cultured from …
Laver came out on top in various experts polls for the best of all time. In 1986, the US magazine Inside Tennis polled 37 experts, which resulted in a computerised tournament. Laver ranked first on this list ahead of John McEnroe, Don Budge, Kramer, Björn Borg, Gonzales, Tilden, Jimmy Connors, Fred Perry, and Lew Hoad. In a poll by the Associated Press in 2000, Laver was voted "The Male Tennis Player of the Century", ahead of Pete Sampras, Tilden, Borg, Budge, McEnroe and Hoad (tied), Rosewall and Roy Emerson (tied), and Kramer. In an article in Tennis Week in 2007, the tennis historian Raymond Lee statistically analysed the all-time best players. Laver topped his list ahead of Tilden and Borg (tied), Roger Federer, Gonzales, Rosewall, Budge, Ivan Lendl, Connors, Sampras, McEnroe, and Kramer. In 2009 it was written that Rod Laver "is considered by most folks who saw him play and many whove heard of his accomplishments, to be as great a tennis player that ever lived-current players ...
My major area of research centers on the control of autoinfection in infections with the nematode Strongyloides stercoralis, a parasite of dogs and man. To this end, in collaboration with Dr. Gerry Schad, we have developed the gerbil as a model for this infection. The gerbil is the only rodent in which all aspects of the parasites life cycle, including autoinfection, have been shown to take place. In the gerbil model we have shown that autoinfection will take place under certain immunosuppressive conditions (inhibitors of T-cell IL 2 regulation, i.e. treatment with tacrolimus, or general immunosuppression, i.e. steroid treatment). Autoinfection has also been observed in immunologically naive gerbils (i.e., neonatally infected gerbils or gerbils infected per os with adult worms). Th 1 inducing infections (i.e. Toxoplasma gondii) and a large initial infection can also induce subsequent autoinfection. We are currently looking at the role of the amphidial neurons in developmental decisions made by ...
The Schad Gallery is our space to explore and display life on planet earth: life in the recent past, now and in the future. Through this gallery, we raise awareness of the challenges facing the conservation, diversity and survival of life on earth.. ...
Summary of Facts and Submissions. I. The appeal was lodged by the Applicant (Appellant) against the decision of the Examining Division to refuse under Article 97(1) EPC 1973 the patent application EP 03 781 200.5 (published as WO 2004/054 615), having the title: Antilymphoma targeting agents with effector and affinity functions linked by a trifunctional reagent.. II. The Examining Division decided that claim 1 of the sole request before it, namely claims 1 to 21 filed with letter of 26 January 2007, did not meet the requirements of Articles 84 and 123(2) EPC and that its subject-matter did not involve an inventive step contrary to the requirements of Article 56 EPC.. III. In its statement of grounds of appeal, dated 20 March 2008, the Appellant requested that the decision under appeal be set aside and a patent be granted on the basis of claims 1 to 21 filed therewith.. The Board expressed its preliminary opinion in a communication dated 29 January 2010 which was annexed to the summons to oral ...
The Katlehong Arts Centre was born in the shadow of the 1976 upsurge, a rebellion that began as a confrontation between township youth and the state but which widened out to a general uprising that included artists and cultural workers. In the accompanying repression, for example, Lionel Davis and Winston Saoli were jailed while other such as Dumile Feni, Gavin Jantjes and Louis Maqhubela, left the country. Growing out of the Katlehong Arts Society, the KAC was established in 1977 as a project of the East Rand Administration Board (ERAB). The antecedent Katlehong Arts Society was founded in 1969 by a group of six artists, including Stanley Nkosi, Lucas Sithole, Napo Mokoena and Morningstar Motaung. The artists approached ERAB and negotiated the establishment of a community arts centre. Although many community arts centres did not take the route of working with the state, this approach wasnt outlandish - Cecil Skotnes had previously used the Johannesburg municipalitys facilities to create a ...
You probably know by now that you need to be eating a good amount of protein if youre trying to lose weight or gain some muscle. But there are plenty of
SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... PC Pyruvate dehydrogenase deficiency; 312170; PDHA1 Pyruvate dehydrogenase E2 deficiency; 245348; DLAT Pyruvate dehydrogenase ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... TMPRSS6 Isobutyryl-CoA dehydrogenase deficiency; 611283; ACAD8 Isovaleric acidemia; 243500; IVD IVIC syndrome; 147750; SALL4 ...
"hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit". Cardiac ... Trifunctional enzyme subunit alpha, mitochondrial also known as hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA ... Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit". Zong NC, Li ... The enzyme converts medium- and long-chain 2-enoyl-CoA compounds into the following 3-ketoacyl-CoA when NAD is solely present, ...
Wanders RJ, Vreken P, den Boer ME, Wijburg FA, van Gennip AH, IJlst L (1999). "Disorders of mitochondrial fatty acyl-CoA beta- ... Deficiency of LCHAD (3-hydroxyacyl-CoA dehydrogenase) leads to an accumulation of medium and long chain fatty acids. When this ... 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 ...
HSD17B4 "EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. 6 September 2017. ...
"OMIM Entry - * 600890 - HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, ALPHA SUBUNIT; HADHA". omim. ... "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Homo ... "Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ...
"Binding of amyloid beta-peptide to mitochondrial hydroxyacyl-CoA dehydrogenase (ERAB): regulation of an SDR enzyme activity ... 17beta-hydroxysteroid dehydrogenase 10 is a member of the short-chain dehydrogenase/reductase superfamily. This homotetrameric ... He XY, Yang YZ, Schulz H, Yang SY (Jan 2000). "Intrinsic alcohol dehydrogenase and hydroxysteroid dehydrogenase activities of ... "Intrinsic alcohol dehydrogenase and hydroxysteroid dehydrogenase activities of human mitochondrial short-chain L-3-hydroxyacyl- ...
1998). "Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in ... acyl-CoA oxidase (see, e.g., ACOX1, MIM 609751); the 'D-bifunctional enzyme,' with enoyl-CoA hydratase and D-3-hydroxyacyl-CoA ... 1999). "17Beta-hydroxysteroid dehydrogenases in human bone cells". J. Bone Miner. Res. 13 (10): 1539-46. doi:10.1359/jbmr. ... 1999). "17Beta-hydroxysteroid dehydrogenase type 1, 2, 3, and 4 expression and enzyme activity in human anterior pituitary ...
Lactate dehydrogenase is a marker of hemolysis and is elevated (>600 U/l). Proteinuria is present but can be mild. In one 1995 ... HELLP is characterized by hemolysis on peripheral blood smear with serum lactate dehydrogenase >600 IU/l; serum aspartate ... 60 (3): 829-36, 839. PMID 10498110. Gillingham M, Van Calcar S, Ney D, Wolff J, Harding C. Dietary management of long-chain 3- ... hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey. Journal of inherited metabolic disease. 1999;22(2 ...
Estradiol 17-beta-dehydrogenase 12 is an enzyme that in humans is encoded by the HSD17B12 gene. The enzyme 17-beta ... Luu-The V, Tremblay P, Labrie F (2006). "Characterization of type 12 17beta-hydroxysteroid dehydrogenase, an isoform of type 3 ... "Entrez Gene: HSD17B12 hydroxysteroid (17-beta) dehydrogenase 12". Maruyama K, Sugano S (1994). "Oligo-capping: a simple method ... 2006). "Systemic distribution and tissue localizations of human 17beta-hydroxysteroid dehydrogenase type 12". J. Steroid ...
"Long-Chain Acyl CoA Dehydrogenase Deficiency: eMedicine Pediatrics: Genetics and Metabolic Disease". Retrieved 2009-07-11. Wang ... CoA) hydratase, long-chain 3-hydroxy acyl-coenzyme A dehydrogenase and long-chain 3-ketoacyl CoA thiolase. Fatty acid beta- ...
4 Dienoyl-CoA Reductase Deficiency Electron Transfer Flavoprotein (ETF) Dehydrogenase Deficiency (GAII & MADD) 3-Hydroxy-3 ... Medium-chain acyl-coenzyme A dehydrogenase deficiency (MCAD deficiency) Short-chain acyl-coenzyme A dehydrogenase deficiency ( ... Methylglutaryl-CoA Lyase (HMG) Deficiency Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD deficiency) Long- ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (M/SCHAD deficiency) The term fatty acid oxidation disorder (FAOD) is ...
Dehydrogenation by acyl-CoA dehydrogenase, yielding 1 FADH2 Hydration by enoyl-CoA hydratase Dehydrogenation by 3-hydroxyacyl- ... Acetyl-CoA is formed into malonyl-CoA by acetyl-CoA carboxylase, at which point malonyl-CoA is destined to feed into the fatty ... CoA dehydrogenase, yielding 1 NADH + H+ Cleavage by thiolase, yielding 1 acetyl-CoA and a fatty acid that has now been ... acetyl-CoA and 1 molecule of propionyl-CoA per molecule of fatty acid. Each beta oxidative cut of the acyl-CoA molecule yields ...
11 beta hydroxysteroid dehydrogenase type 2 deficiency 17 alpha hydroxylase deficiency 17 beta hydroxysteroide dehydrogenase ... 2-Methylacetoacetyl CoA thiolase deficiency, rare (NIH) 2,8 dihydroxy-adenine urolithiasis 21 hydroxylase deficiency 22q11.2 ... coa hydratase deficiency 3-hydroxy 3-methyl glutaryl-coa lyase deficiency 3-hydroxyacyl-coa dehydrogenase deficiency 3 ... deficiency 17-beta-hydroxysteroid dehydrogenase deficiency, rare (NIH) 17q21.31 microdeletion syndrome 18-Hydroxylase ...
... hypoglycaemia Levomepromazine Liver cancer Liver glycogen synthase deficiency Long-chain hydroxyacyl-CoA dehydrogenase ... Trimethoprim Triple A syndrome Tumors Tyrosinaemia type 1 Urea cycle disorder Uremia Very-long-chain acyl-CoA dehydrogenase ... Reye syndrome Ritonavir Saquinavir Sepsis Septic shock Severe hepatitis Sheehan syndrome Short-chain acyl-CoA dehydrogenase ... deficiency Maple syrup urine disease Mcquarrie type infantile idiopathic hypoglycemia Medium chain acyl-CoA dehydrogenase ...
... glutaryl-CoA dehydrogenase EC 1.3.8.7: medium-chain acyl-CoA dehydrogenase EC 1.3.8.8: long-chain acyl-CoA dehydrogenase EC 1.3 ... glutaryl-CoA dehydrogenase EC 1.3.99.8: 2-furoyl-CoA dehydrogenase EC 1.3.99.9: now *EC 1.21.99.1 EC 1.3.99.10: isovaleryl-CoA ... succinate dehydrogenase EC 1.3.99.2: butyryl-CoA dehydrogenase EC 1.3.99.3: acyl-CoA dehydrogenase EC 1.3.99.4: 3-oxosteroid 1- ... benzylsuccinyl-CoA dehydrogenase EC 1.3.8.4: isovaleryl-CoA dehydrogenase EC 1.3.8.5: 2-methyl-branched-chain-enoyl-CoA ...
... deficiency of subtypes of acyl CoA dehydrogenase (LCAD, SCAD, MCAD, VLCAD, 3-hydroxyacyl-coenzyme A dehydrogenase deficiency), ... Elevated concentrations of the enzyme lactate dehydrogenase (LDH) may be detected. Other markers of muscle damage, such as ... glucose-6-phosphate dehydrogenase deficiency, myoadenylate deaminase deficiency and muscular dystrophies Damage to skeletal ... thiolase deficiency Mitochondrial myopathies: deficiency of succinate dehydrogenase, cytochrome c oxidase and coenzyme Q10 ...
Acyl CoA dehydrogenase, Enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase. Since lipids (fats) ... The resulting Acyl-CoA activates the process of beta oxidation to further break down into Acetyl-CoA (which is used in the ... Fatty acid metabolism begins in the cytoplasm of epithelial cells as Acyl-CoA synthetase and hydrolysis of ATP cleaves a ... King, Michael W. Fatty Acid, Omega-3 and Omega-6 Fatty Acid, Triglyceride, and Phospholipid Synthesis and Metabolism. The ...
... hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein)". ]Zong ... which yields an acetyl CoA molecule and an acyl CoA molecule, which is two carbons shorter. The encoded protein can also bind ... Middleton B (1994). "The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase ... acetyl-CoA acyltransferase, or beta-ketothiolase is an enzyme that in humans is encoded by the HADHB gene. HADHB is a subunit ...
... namely acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and thiolase. The cycle produces a new ... It regulates through the ratio of acetyl-CoA versus CoA. Increased concentration of acetyl-CoA activates PDK. Acetyl-CoA is ... Acetyl-CoA can be carboxylated in the cytosol by acetyl-CoA carboxylase, giving rise to malonyl-CoA, a substrate required for ... "Regulation of pyruvate dehydrogenase kinase and phosphatase by acetyl-CoA/CoA and NADH/NAD ratios". Biochemical and Biophysical ...
... hydroxyacyl coenzyme-A dehydrogenase, NADP+-linked acetoacetyl CoA reductase, NADPH:acetoacetyl-CoA reductase, D(−)-beta- ... hydroxybutyryl CoA-NADP+ oxidoreductase, short chain beta-ketoacetyl(acetoacetyl)-CoA reductase, beta-ketoacyl-CoA reductase, D ... In enzymology, an acetoacetyl-CoA reductase (EC 1.1.1.36) is an enzyme that catalyzes the chemical reaction (R)-3-hydroxyacyl- ... CoA + NADP+ ⇌ {\displaystyle \rightleftharpoons } 3-oxoacyl-CoA + NADPH + H+ Thus, the two substrates of this enzyme are (R)-3- ...
... (EC 1.3.1.86, butyryl-CoA dehydrogenase, butyryl dehydrogenase, unsaturated acyl-CoA reductase, ethylene ... short-chain acyl CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, 3-hydroxyacyl CoA reductase, butanoyl-CoA:( ... Crotonyl-CoA reductase at the US National Library of Medicine Medical Subject Headings (MeSH) Molecular and Cellular Biology ... This enzyme catalyses the following chemical reaction butanoyl-CoA + NADP+ ⇌ {\displaystyle \rightleftharpoons } (E)-but-2- ...
The dehydrogenase activity of enoyl-CoA occurs in the carboxyl-terminal. Upon further investigation of the CoA binding site on ... delta2-Enoyl-CoA Isomerase and Peroxisomal Multifunctional delta3,delta2- Enoyl-CoA Isomerase, 2-Enoyl-CoA Hydratase, 3- ... Hydroxyacyl-CoA Dehydrogenase Enzyme in Rat Liver" (PDF). Journal of Biological Chemistry. 266 (17): 10750-10753. PMID 2040594 ... Enoyl-CoA-(∆) isomerase, also known as dodecenoyl-CoA-(∆) isomerase, 3,2-trans-enoyl-CoA isomerase, ∆3(cis),∆2(trans)-enoyl-CoA ...
4 Dienoyl-CoA reductase deficiency - 2,4 Dienoyl-CoA reductase 3-hydroxy-3-methylglutaryl-CoA lyase deficiency - 3-hydroxy-3- ... Medium-chain acyl-coenzyme A dehydrogenase Short-chain acyl-coenzyme A dehydrogenase deficiency (SCAD) - Short-chain acyl- ... methylglutaryl-CoA lyase Malonyl-CoA decarboxylase deficiency - Malonyl-CoA decarboxylase Primary carnitine deficiency - ... Some of the more common fatty acid metabolism disorders are: Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD ...
Wakil SJ (1955). "D(-)beta-Hydroxybutyryl CoA dehydrogenase". Biochim. Biophys. Acta. 18 (2): 314-315. doi:10.1016/0006-3002(55 ... Stern JR, del Campillo A, Lehninger AL (1955). "Enzymatic racemization of beta-hydroxybutyryl-S-CoA and the stereospecificity ... In enzymology, a 3-hydroxybutyryl-CoA epimerase (EC 5.1.2.3) is an enzyme that catalyzes the chemical reaction (S)-3- ... hydroxybutanoyl-CoA ⇌ {\displaystyle \rightleftharpoons } (R)-3-hydroxybutanoyl-CoA Hence, this enzyme has one substrate, (S)-3 ...
People with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency have inadequate levels of an enzyme required for a step that ... Problems related to 3-hydroxyacyl-coenzyme A dehydrogenase deficiency can be triggered by periods of fasting or by illnesses ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (HADH deficiency) is a rare condition that prevents the body from converting ... Individuals with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency are also at risk for complications such as seizures, life- ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... Thus, the two substrates of this enzyme are xylitol and NADP+, whereas its 3 products are L-xylulose, NADPH, and H+. ... Over-expression and ectopic expression of the protein may be associated with prostate adenocarcinoma.[3] ...
L-hydroxyacyl-CoA dehydrogenase 3beta-hydroxyacyl coenzyme A dehydrogenase beta-hydroxy acid dehydrogenase beta-hydroxyacyl CoA ... dehydrogenase beta-hydroxyacyl dehydrogenase beta-hydroxyacyl-coenzyme A synthetase beta-hydroxyacylcoenzyme A dehydrogenase ... beta-Hydroxyacyl coenzyme A dehydrogenase". J. Biol. Chem. 207 (2): 631-8. PMID 13163047. Molecular and Cellular Biology portal ... Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme ...
Hydroxyacyl-CoA dehydrogenase (EC1.1.1.35) from mitochondria at the resolution 2.0 A, Northeast Structural Genomics Consortium ... Hydroxyacyl-CoA dehydrogenase (EC1.1.1.35) from mitochondria at the resolution 2.0 A, Northeast Structural Genomics Consortium ... Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial. A, B. 324. Homo sapiens. Mutation(s): 0 Gene Names: HADH (HAD, HADHSC, ... GLYCERIN; PROPANE-1,2,3-TRIOL. C3 H8 O3. PEDCQBHIVMGVHV-UHFFFAOYSA-N. ...
Recognition of Structurally Diverse Substrates by Type II 3-Hydroxyacyl-Coa Dehydrogenase (Hadh II) Amyloid-Beta Binding ... 3] Andreeva A., Howorth D., Chandonia J.-M., Brenner S.E., Hubbard T.J.P., Chothia C., Murzin A.G. (2008).. Data growth and its ... Rat brain 3-hydroxyacyl-CoA dehydrogenase binary complex with NADH and 3-keto butyrate. ... If available, the SCOP 1.75 domain assignment [3] is used. Otherwise algorithmic domain assignments are computed using the ...
CoA) Dehydrogenase (LCHAD) Associated Neuropathy. The safety and scientific validity of this study is the responsibility of the ... Long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD). Mitochondrial trifunctional protein (TFP). LCHAD/TFP deficiency with ... Vitamin E Treatment for Long-Chain 3-Hydroxyacyl Coenzyme A ( ...
What is 3-hydroxyacyl-CoA dehydrogenase? Meaning of 3-hydroxyacyl-CoA dehydrogenase medical term. What does 3-hydroxyacyl-CoA ... Looking for online definition of 3-hydroxyacyl-CoA dehydrogenase in the Medical Dictionary? 3-hydroxyacyl-CoA dehydrogenase ... yl-CoA de·hy·dro·gen·ase. (hī-droksē-asil dēhī-drōjen-ā), β-Hydroxyacyl dehydrogenase; an enzyme catalyzing the oxidation ... 3-hydroxyacyl-CoA dehydrogenase. Also found in: Acronyms, Wikipedia. 3-hy·drox·y·ac· ...
2-Methylacetoacetyl-CoA + NADH. details 2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Ion. ... Submicroscopic duplications of the hydroxysteroid dehydrogenase HSD17B10 and the E3 ubiquitin ligase HUWE1 are associated with ... 2004 Sep 17;342(3):943-52. [PubMed:15342248 ] *Lustbader JW, Cirilli M, Lin C, Xu HW, Takuma K, Wang N, Caspersen C, Chen X, ... S)-3-hydroxyacyl-CoA + NAD → 3-oxoacyl-CoA + NADH. details 2-Methyl-3-hydroxybutyryl-CoA + NAD → ...
... of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for 3-alpha ... 3-alpha hydroxyacyl-CoA dehydrogenase deficiency Title Other Names:. 3-hydroxylacyl-CoA dehydrogenase deficiency; Medium and ... PubMed is a searchable database of medical literature and lists journal articles that discuss 3-alpha hydroxyacyl-CoA ... dehydrogenase deficiency. Click on the link to view a sample search on this topic. ...
86, nr 3, 329-337 s. Nyckelord [en] Chorioretinal atrophy, electroetinography, LCHAD deficiency, myopia Nationell ämneskategori ... 86, nr 3, 329-337 s.Artikel i tidskrift (Refereegranskat) Published Abstract [en] PURPOSE ... Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a cross-sectional study with ... To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA ...
Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)Download version for offline viewing or printing. [463.65kB] ... Babies with the diseases Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) or Trifunctional Protein Deficiency (TFP ...
... and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency are long-chain fatty acid oxidation disorders with ... For very-long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the most common long-chain fatty oxidation defect, it has been ... Ficicioglu C, Coughlin CR 2nd, Bennett MJ, Yudkoff M. Very long-chain acyl-CoA dehydrogenase deficiency in a patient with ... A near-miss: very long chain acyl-CoA dehydrogenase deficiency with normal primary markers in the initial well-timed newborn ...
HADHB hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit be... HADHB hydroxyacyl-CoA dehydrogenase ... acetyl-CoA acyltransferase. beta-ketothiolase. hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase ( ... Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA, organism-specific biosystem (from REACTOME) Beta oxidation of lauroyl-CoA to ... Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA, organism-specific biosystem (from REACTOME) Beta oxidation of decanoyl-CoA ...
The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase ( ... 2002). Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients. Pediatrics. ... LCHAD (Long-Chain 3-Hydroxyacyl CoA Dehydrogenase) deficiency. LCHAD (long-chain 3-hydroxyacyl CoA dehydrogenase) deficiency is ... Chronically 3-hydroxylated dicarboxylic acids and nonhydroxylated dicarboxylic acids will be positive. Plasma carnitine may be ...
People with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency have inadequate levels of an enzyme required for a step that ... Problems related to 3-hydroxyacyl-coenzyme A dehydrogenase deficiency can be triggered by periods of fasting or by illnesses ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (HADH deficiency) is a rare condition that prevents the body from converting ... Individuals with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency are also at risk for complications such as seizures, life- ...
SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... PC Pyruvate dehydrogenase deficiency; 312170; PDHA1 Pyruvate dehydrogenase E2 deficiency; 245348; DLAT Pyruvate dehydrogenase ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... TMPRSS6 Isobutyryl-CoA dehydrogenase deficiency; 611283; ACAD8 Isovaleric acidemia; 243500; IVD IVIC syndrome; 147750; SALL4 ...
... such that HIV-1 Vpr regulates mitochondrial respiration and enhances the activity of hydroxyacyl-CoA dehydrogenase (HADH) ... Hydroxyacyl-Coenzyme A dehydrogenase also known as HADH is an enzyme which in humans is encoded by the HADH gene. The HADH gene ... "Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB). Molven A, Matre ... "A preliminary analysis of the segregation of human hydroxyacyl coenzyme A dehydrogenase in human-mouse somatic cell hybrids". ...
β-hydroxyacyl CoA dehydrogenase (HOAD). The assay contained (in mmol l-1): acetoacetylCoA (0.1), NADH (0.15) in imidazole (50) ... Lactate dehydrogenase (LDH). The assay contained (in mmol l-1): pyruvate (1), NADH (0.15) in Hepes (50) at pH 7.0. ... The assay contained (in mmol l-1): 5,5′-dithiobis-(2-nitrobenzoic acid (0.1), palmitoyl CoA (0.1) and carnitine (5) in Tris-HCl ... The assay contained (in mmol l-1): 5,5′-dithiobis-(2-nitrobenzoic acid) (0.1), acetyl CoA (0.3), oxaloacetate (0.5), in Tris- ...
Compare Anti-Alcohol Dehydrogenase 2 Antibody Products from leading suppliers on Biocompare. View specifications, prices, ... Rabbit anti-human zinc binding alcohol dehydrogenase domain containing 2 polyclonal Antibody ...
... medium-chain acyl-CoA dehydrogenase deficiency (Table) (3.6). Newborn screening can help prevent death or disability, if ... The five most commonly diagnosed conditions in the United States are 1) hearing loss, 2) primary congenital hypothyroidism, 3) ... 3). Additional conditions for screening continue to be identified and nominated for inclusion in the panel. ... 3). Through early identification, newborn screening provides an opportunity for treatment and significant reductions in ...
We describe two children with deficiency of short-chain L-3-hydroxyacyl-CoA dehydrogenase, a new disorder of the mitochondrial ... Mitochondrial Short-Chain L-3-hydroxyacyl-coenzyme A Dehydrogenase Deficiency: A New Defect of Fatty Acid Oxidation Pediatr Res ... We describe two children with deficiency of short-chain L-3-hydroxyacyl-CoA dehydrogenase, a new disorder of the mitochondrial ...
Relationship between the different dehydrogenases and evidence that fatty acids and the C27 bile acids di- and tri- ... An inducible fatty acyl-CoA oxidase, a noninducible fatty acyl-CoA oxidase, and a noninducible trihydroxycoprostanoyl-CoA ... THC-CoA oxidase, THCA-CoA oxidase, 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA oxidase, 3alpha,7alpha,12alpha- ... Schepers L, Van Veldhoven PP, Casteels M, Eyssen HJ, Mannaerts GP (1990). "Presence of three acyl-CoA oxidases in rat liver ...
Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase, including: function, proteins, disorders, pathways, orthologs, and ... Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase 2 3 5 * Enoyl-Coenzyme A, Hydratase/3-Hydroxyacyl Coenzyme A ... EHHADH (Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase) is a Protein Coding gene. Diseases associated with EHHADH ... The GeneCards human gene database index: 2 3 5 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z ...
Fraser H1, Geppert J2, Johnson R3, Johnson S4, Connock M2, Clarke A2, Taylor-Phillips S2, Stinton C2. ... 3. Faculty of Health and Life Sciences, Coventry University, Coventry, CV1 5RW, UK.. 4. Warwick Library, University of Warwick ... 3. Forest plot showing mortality and incidence of cardiac and liver problems across symptomatically and asymptomatically ... For follow up analysis 3 we found a significant difference for only one comparison, in the incidence of cardiomyopathy between ...
GO:0004300 enoyl-CoA hydratase activity Cellular Component. GO:0016507 mitochondrial fatty acid beta-oxidation multienzyme ... Enoyl-CoA hydratase/isomerase (IPR001753) *Fatty acid oxidation complex, alpha subunit, mitochondrial (IPR012803) ... Subunit activities include: enoyl-CoA hydratase (EC:4.2.1.17) and 3-hydroxyacyl-CoA dehydrogenase (EC:1.1.1.35). Some ... The beta subunit has acetyl-CoA C-acyltransferase (EC:2.3.1.16) activity. ...
... hydroxyacyl-CoA dehydrogenase fragment of rat peroxisomal MFE-2 (PDB code 1GZ6) and molecule A of the (2E)-enoyl-CoA hydratase ... hydroxyacyl-CoA dehydrogenase domain, a C-terminal (2E)-enoyl-CoA hydratase 2 domain and a short loop which connects the two ... Hydroxyacyl-CoA dehydrogenase domain of Dm. MFE-2. The (3R)-hydroxyacyl-CoA dehydrogenase domain of DmMFE-2 consists of amino ... Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional ...
... acyl-CoA dehydrogenase encoded by fadE; ECOAH3, enoyl-CoA hydratase encoded by fadB; HACD3, 3-hydroxyacyl-CoA dehydrogenase ... acyl-CoA dehydrogenase (ACOAD3), enoyl-CoA hydratase (ECOAH3), 3-hydroxyacyl-CoA dehydrogenase (HACD3), 3-ketoacyl-CoA thiolase ... acetyl-CoA is used by acetyl-CoA carboxylase (ACC) to produce malonyl-CoA, which is subsequently converted to malonyl-ACP by ... Fatty acid is firstly acylated by fatty acyl-CoA synthetase (FadD) to form fatty acyl-CoA, which then enters into the β- ...
  • Upon further investigation of the CoA binding site on the amino-terminal half of the multifunctional protein, the CoA substrate is not transferred through the aqueous phase from the isomerization phase to the site of hydration or does not have a bulk phase. (wikipedia.org)
  • The (2 E )-enoyl-CoA intermediate formed becomes hydrated in the second step, which is followed by NAD + -dependent dehydrogenation, which converts the intermediate into 3-oxoacyl-CoA (3-ketoacyl-CoA). (portlandpress.com)