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.
Enzymes that catalyze the first step in the beta-oxidation of FATTY ACIDS.
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 is the biologically active component of lipopolysaccharides. It shows strong endotoxic activity and exhibits immunogenic properties.
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.
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)
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.
An NAD-dependent 3-hydroxyacyl CoA dehydrogenase that has specificity for acyl chains containing 8 and 10 carbons.
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.
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.
A zinc-containing enzyme which oxidizes primary and secondary alcohols or hemiacetals in the presence of NAD. In alcoholic fermentation, it catalyzes the final step of reducing an aldehyde to an alcohol in the presence of NADH and hydrogen.
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.
The dynamic collection of metabolites which represent a cell's or organism's net metabolic response to current conditions.
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.
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.
A visual impairment characterized by the accumulation of fluid under the retina through a defect in the retinal pigment epithelium.
Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.
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.
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.
Diazo derivatives of aniline, used as a reagent for sugars, ketones, and aldehydes. (Dorland, 28th ed)
Adaptation to a new environment or to a change in the old.
An absence of warmth or heat or a temperature notably below an accustomed norm.
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.
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.
A condition of inadequate circulating red blood cells (ANEMIA) or insufficient HEMOGLOBIN due to premature destruction of red blood cells (ERYTHROCYTES).
A reduction in the number of circulating ERYTHROCYTES or in the quantity of HEMOGLOBIN.
Nucleic acids which hybridize to complementary sequences in other target nucleic acids causing the function of the latter to be affected.
DNA analogs containing neutral amide backbone linkages composed of aminoethyl glycine units instead of the usual phosphodiester linkage of deoxyribose groups. Peptide nucleic acids have high biological stability and higher affinity for complementary DNA or RNA sequences than analogous DNA oligomers.
A genus of shallow-cupped SPONGES with a broad base in the family Theonellidae. They are characterized by ectosomal spicules dominated by phyllotriaenes.
Members of the class of compounds composed of AMINO ACIDS joined together by peptide bonds between adjacent amino acids into linear, branched or cyclical structures. OLIGOPEPTIDES are composed of approximately 2-12 amino acids. Polypeptides are composed of approximately 13 or more amino acids. PROTEINS are linear polypeptides that are normally synthesized on RIBOSOMES.
Peptides generated from AMYLOID BETA-PEPTIDES PRECURSOR. An amyloid fibrillar form of these peptides is the major component of amyloid plaques found in individuals with Alzheimer's disease and in aged individuals with trisomy 21 (DOWN SYNDROME). The peptide is found predominantly in the nervous system, but there have been reports of its presence in non-neural tissue.
A degenerative disease of the BRAIN characterized by the insidious onset of DEMENTIA. Impairment of MEMORY, judgment, attention span, and problem solving skills are followed by severe APRAXIAS and a global loss of cognitive abilities. The condition primarily occurs after age 60, and is marked pathologically by severe cortical atrophy and the triad of SENILE PLAQUES; NEUROFIBRILLARY TANGLES; and NEUROPIL THREADS. (From Adams et al., Principles of Neurology, 6th ed, pp1049-57)
Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.
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 containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.
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).
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.
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).
Enzymes of the oxidoreductase class that catalyze the dehydrogenation of hydroxysteroids. (From Enzyme Nomenclature, 1992) EC 1.1.-.
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 produced by a single clone of cells.
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.
Proteins prepared by recombinant DNA technology.
Catalyze the oxidation of 3-hydroxysteroids to 3-ketosteroids.

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)

Short chain L-3-hydroxyacyl CoA dehydrogenase (SCHAD) is a mitochondrial enzyme involved in the metabolism of fatty acids. It catalyzes the oxidation of the hydroxyl group of L-3-hydroxyacyl CoA to a keto group, concomitant with the reduction of NAD+ to NADH. Deficiencies in this enzyme result in various disease states, including hypertrophic cardiomyopathy, skeletal myopathy, hypoketotic hypoglycemia, and liver dysfunction. The proposed research utilizes a multi-faceted approach to understanding the structure-function relationships present in human SCHAD. SCHAD will be purified to homogeneity from an appropriate expression system and its kinetic properties characterized. Crystals of human SCHAD suitable for x-ray diffraction studies will then be prepared in order to resolve the three-dimensional structure of the apoenzyme, the NAD+-complexed enzyme, and the acyl-CoA-complexed enzyme. Site directed mutagenesis and rational inhibitor design studies will be initiated to confirm or further develop ...
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 ...
The neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) impairs mitochondrial respiration and damages dopaminergic neurons as seen in Parkinsons disease (PD). Here, we report that L-3-hydroxyacyl-CoA dehydrogenase type II/amyloid binding alcohol dehydrogenase (HADH II/ABAD), a mitochondr …
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 ...
This enzyme was purified from the mitochondrial inner membrane. The enzyme has a preference for long-chain substrates, and activity with a C16 substrate was 6- to 15-fold higher than with a C4 substrate (cf. EC 1.1.1.35 3-hydroxyacyl-CoA dehydrogenase ...
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.
Fatty acid oxidation complex subunit alpha; 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; In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase ...
Fatty acid oxidation complex subunit alpha; 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; In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase ...
Title:Recent Process in the Inhibitors of UDP-3-O-(R-3-hydroxyacyl)-Nacetylglucosamine Deacetylase (LpxC) Against Gram-Negative Bacteria. VOLUME: 18 ISSUE: 4. Author(s):Fang Liu and Shutao Ma*. Affiliation:Department of Medicinal Chemistry, Key Laboratory of Chemical Biology (Ministry of Education), School of Pharmaceutical Sciences, Shandong University, 44 West Culture Road, Jinan 250012, Department of Medicinal Chemistry, Key Laboratory of Chemical Biology (Ministry of Education), School of Pharmaceutical Sciences, Shandong University, 44 West Culture Road, Jinan 250012. Keywords:Anti-bacterial agent, biosynthesis, UDP-3-O-(R-3-hydroxymyristoyl)-N-acetylglucosamine deacetylase (LpxC), LpxC inhibitors, Gram-negative bacterial, lipid A biosynthesis.. Abstract:Infections caused by pathogenic bacteria are a major health concern throughout the world. There is a great need to develop novel antibacterial agents with new mechanisms of action. Lipopolysaccharides (LPS) are the main component of the ...
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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.. ...
After a diagnosis of unexplained infertility in 2013, Schad and her husband decided to adopt. They already had a 3-year-old son, to whom Schad had given birth and breastfed. She believed in the health benefits of breastfeeding and enjoyed its intimacy. So, when the couple began the adoption process, Schad researched how she might re-lactate in order to offer those same benefits to her adopted child. After learning in 2015 that they were matched with a health-challenged baby whose symptoms might be lessened by breastfeeding, Schad became more determined to try.. Induced lactation has slowly grown in popularity for adoptive mothers as well as the non-birth mothers of same-sex couples and mothers who have used gestational carriers. Breast milk induction is possible, but not simple. The most common method for non-birthing mothers to produce milk is by following the Newman-Goldfarb protocols. This method, published by a Canadian physician in 2000, involves birth control hormones, frequent breast ...
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: ...
Introduction. Acute fatty liver of pregnancy (AFLP) is a rare and life-threatening disorder that affects 1 in 13000 pregnancies1,2. It was first described as acute yellow atro-phy of the liver in pregnancy in 1940 by Sheehan2. AFLP usually occurs at third trimester (between 30 and 38 weeks of gestation) or during postpartum pe-riod1-3. This clinical condition may return at subsequent pregnancies and is more common in underweight women, primigravidas, male fetus and multiple gestations2,4,5. There arent any geographical or ethnicity diffe-rences in AFLP3.. The etiology and pathogenesis of AFLP remains unknown but recent studies suggested that dysfunction of beta-oxidation of long chain fatty acids could be involved2,4. Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) is a mitochondrial enzyme involved in fatty acid oxidation that is the primary mechanism of ketone bodies production. Ketone bodies are the major source of energy for the brain and heart when glucose blood levels are low, for ...
1. The activities of phosphofructokinase (PFK), citrate synthetase (CS), lactate dehydrogenase (LDH), 3-hydroxyacyl-CoA dehydrogenase (ACDH) and cytochrome- c oxidase (Cyt-ox) in the calf muscle tissue were compared in subjects with intermittent claudication ( n = 38) and controls ( n = 20). The activities of CS, ACDH and Cyt-ox were increased and the activity of Cyt-ox was positively correlated to the maximal walking distance (MWD) in the patients. 2. Thirty-three patients with intermittent claudication were randomized to three treatment groups: (1) operative surgery, (2) operative surgery supplemented with physical training and (3) physical training alone. Before and after 6-12 months of treatment, symptom-free walking distance (SFWD), MWD, ankle-brachial blood pressure quotient (ankle index), maximal plethysmographic calf blood flow (MPBF) and the activities of PFK, CS, LDH, ACDH and Cyt-ox were measured. 3. SFWD and MWD increased in all three groups. Ankle index and MPBF increased in groups ...
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.
isovaleryl-CoA dehydrogenase Ja 1.3.99.13 long-chain-acyl-CoA dehydrogenase Ja ... L-iditol 2-dehydrogenase Ja 1.1.1.15 D-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. D-sorbose + NADH + H+ D-iditol 2- ... D-arabitol 4-dehydrogenase 1.1.1.12 L-arabitol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-xylulose + NADH + H+ L-arabitol ... L-arabitol 2-dehydrogenase 1.1.1.14 L-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-sorbose + NADH + H+ ...
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 ... DCX Succinic semialdehyde dehydrogenase deficiency; 271980; ALDH5A1 Succinyl-CoA:3-oxoacid CoA transferase deficiency; 245050; ...
HSD17B4 "EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. 6 September 2017. ...
"Entrez Gene: Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit ... "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 ... 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 ...
108 (3): 457-465. doi:10.1172/jci200111294. PMC 209352. PMID 11489939. Dunne, Mark J.; Kane, Charlotte; Shepherd, Ruth M.; ... Retrieved 3 November 2018. CS1 maint: discouraged parameter (link) "Sir Al Aynsley-Green". The Conversation. Profile: The ... Retrieved 3 November 2018. CS1 maint: discouraged parameter (link) The National Service Framework for Children, Young People ... Retrieved 3 November 2018. CS1 maint: discouraged parameter (link) "National service framework: children, young people and ...
... is not an appropriate substrate for acyl CoA dehydrogenase, or enoyl CoA hydratase: If the acyl CoA contains a cis-Δ3 bond, ... This is catalyzed by acyl CoA dehydrogenase to produce trans-delta 2-enoyl CoA. It uses FAD as an electron acceptor and it is ... The overall reaction for one cycle of beta oxidation is: Cn-acyl-CoA + FAD + NAD+ + H 2O + CoA → Cn-2-acyl-CoA + FADH 2 + NADH ... The final cycle produces two separate acetyl CoAs, instead of one acyl CoA and one acetyl CoA. For every cycle, the Acyl CoA ...
... protein is predicted to interact highly with enoyl-CoA hydratase and hydroxyacyl-CoA dehydrogenase, based on textmining ... Other predicted interacting proteins are acetyl-CoA carboxylases A and B, glycine dehydrogenase, 3-oxoacid CoA transferase 2. ... Three common missense mutations occur in the 3' UTR and in the coding sequence which change serine to leucine in the protein. ... O-linked glycosylation is predicted to occur at 3 sites toward the C-terminus. These sites are well-conserved in all homologs. ...
... , ECHB, MSTP029, MTPB, TP-BETA, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional ... hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta. External IDs. OMIM: 143450 MGI: 2136381 ... acetyl-CoA C-acyltransferase activity. • long-chain-enoyl-CoA hydratase activity. Cellular component. • membrane. • ... "Entrez Gene: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional ...
"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- ...
CS1 maint: discouraged parameter (link) "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase ( ... CS1 maint: discouraged parameter (link) "OMIM Entry - * 600890 - HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL- ... "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 ...
"Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional ... acyl-CoA oxidase (see, e.g., ACOX1, MIM 609751); the 'D-bifunctional enzyme,' with enoyl-CoA hydratase and D-3-hydroxyacyl-CoA ... van Grunsven EG, Mooijer PA, Aubourg P, Wanders RJ (August 1999). "Enoyl-CoA hydratase deficiency: identification of a new type ... It was first identified as a 17-beta-estradiol dehydrogenase (Leenders et al., 1996; van Grunsven et al., 1998). Peroxisomal ...
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 ...
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 ...
The process of beta oxidation requires the following enzymes: acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA ... The diagram to the left shows how fatty acids are converted into acetyl-CoA. The overall net reaction, using palmitoyl-CoA (16: ... The resulting acyl-CoA cross the mitochondria membrane and enter the process of beta oxidation. The main products of the beta ... Long chain fatty acids (more than 14 carbon) need to be converted to fatty acyl-CoA in order to pass across the mitochondria ...
"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- ... methylglutaryl-CoA lyase deficiency (HMG deficiency) Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD deficiency ... Medium-chain acyl-coenzyme A dehydrogenase deficiency (MCAD deficiency) Short-chain acyl-coenzyme A dehydrogenase deficiency ( ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (M/SCHAD deficiency) The term fatty acid oxidation disorder (FAOD) is ...
... 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 ... benzylsuccinyl-CoA dehydrogenase EC 1.3.8.4: isovaleryl-CoA dehydrogenase EC 1.3.8.5: 2-methyl-branched-chain-enoyl-CoA ... 2-cis-dihydrodiol dehydrogenase EC 1.3.1.62: pimeloyl-CoA dehydrogenase EC 1.3.1.63: 2,4-dichlorobenzoyl-CoA reductase EC 1.3. ... glutaryl-CoA dehydrogenase (non-decarboxylating) EC 1.4.1.1: alanine dehydrogenase EC 1.4.1.2: glutamate dehydrogenase EC 1.4. ...
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 ...
... 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 ...
Beta oxidation of acyl-CoA occurs in four steps. 1. Acyl-CoA dehydrogenase catalyzes dehydrogenation of the acyl-CoA, creating ... The latter conversion is mediated by acyl-CoA synthase" acyl-P + HS-CoA → acyl-S-CoA + Pi + H+ Three types of acyl-CoA ... Acetyl-CoA Beta oxidation Coenzyme A Acyl CoA dehydrogenase Fatty acid metabolism Talley, Jacob T.; Mohiuddin, Shamim S. (2020 ... A rare disease called multiple acyl-CoA dehydrogenase deficiency (MADD) is a fatty acid metabolism disorder. Acyl-CoA is ...
... 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) Biology portal. ... Wallace KK, Bao ZY, Dai H, Digate R, Schuler G, Speedie MK, Reynolds KA (November 1995). "Purification of crotonyl-CoA ...
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 ...
... is a rare condition that prevents the body from converting certain fats to ... 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 ... 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] ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... In enzymology, a homoisocitrate dehydrogenase (EC 1.1.1.87) is an enzyme that catalyzes the chemical reaction ... Rowley B, Tucci AF (1970). "Homoisocitric dehydrogenase from yeast". Arch. Biochem. Biophys. 141 (2): 499-, 510. doi:10.1016/ ... homoisocitric dehydrogenase, (−)-1-hydroxy-1,2,4-butanetricarboxylate:NAD+ oxidoreductase, (decarboxylating), 3-carboxy-2- ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... alcohol dehydrogenase (NADP+) activity. • retinal dehydrogenase activity. • allyl-alcohol dehydrogenase activity. • NADP- ... retinol dehydrogenase activity. Cellular component. • mast cell granule. • Schwann cell microvillus. • Schmidt-Lanterman ... 9 (3): 149-57. doi:10.1089/dna.1990.9.149. PMID 2111143.. *. Nishimura C, Matsuura Y, Kokai Y, Akera T, Carper D, Morjana N, ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... lactate dehydrogenase A. (subunit M). Human lactate dehydrogenase M4 (the isoenzyme found in skeletal muscle). From PDB: 1I10​. ... D-lactate dehydrogenase, membrane binding. crystal structure of d-lactate dehydrogenase, a peripheral membrane respiratory ... Lactate dehydrogenase-A deficiency is caused by a mutation to the LDHA gene, while lactate dehydrogenase-B deficiency is caused ...
C3orf60/NDUFAF3: encoding enzyme NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 ... 3. q 29. 11175. 11700. 192,600,001. 198,295,559 gneg. References[edit]. *^ "Human Genome Assembly GRCh38 - Genome Reference ... G-bands of human chromosome 3 in resolution 850 bphs[4] Chr. Arm[17] Band[18] ISCN. start[19] ISCN. stop[19] Basepair. start ... "Chromosome 3". Genetics Home Reference. Retrieved 2017-05-06.. *. "Chromosome 3". Human Genome Project Information Archive 1990 ...
... namely acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and thiolase. The cycle produces a new ... Acetyl-CoA can be carboxylated in the cytosol by acetyl-CoA carboxylase, giving rise to malonyl-CoA, a substrate required for ... "Acetyl CoA Crossroads". chemistry.elmhurst.edu. Retrieved 2016-11-08.. *^ "Fatty Acids -- Structure of Acetyl CoA". library.med ... "Regulation of pyruvate dehydrogenase kinase and phosphatase by acetyl-CoA/CoA and NADH/NAD ratios". Biochemical and Biophysical ...
2,4 Dienoyl-CoA reductase. Odd chain. *Propionyl-CoA carboxylase. Other. *Hydroxyacyl-Coenzyme A dehydrogenase ... Acetyl-CoA carboxylase (ACC) is a biotin-dependent enzyme that catalyzes the irreversible carboxylation of acetyl-CoA to ... The carboxyl group is transferred from biotin to acetyl CoA to form malonyl CoA in the second reaction, which is catalyzed by ... Malonyl-CoA decarboxylase. References[edit]. *^ a b c Tong L (August 2005). "Acetyl-coenzyme A carboxylase: crucial metabolic ...
Hydroxyacyl-Coenzyme A dehydrogenase. To acetyl-CoA. *Malonyl-CoA decarboxylase. Aldehydes. *Long-chain-aldehyde dehydrogenase ... beta-hydroxyacyl-[acylcarrier-protein] dehydrase, and enoyl-[acyl-carrier-protein] reductase from Spinacia oleracea leaves". ... 3R)-3-hydroxyacyl-[acyl-carrier-protein] + NADP+ ⇌. {\displaystyle \rightleftharpoons }. 3-oxoacyl-[acyl-carrier-protein] + ... The systematic name of this enzyme class is (3R)-3-hydroxyacyl-[acyl-carrier-protein]:NADP+ oxidoreductase. Other names in ...
Acetyl-coA inhibits pyruvate dehydrogenase, while succinyl-CoA inhibits alpha-ketoglutarate dehydrogenase and citrate synthase ... Lastly, beta-hydroxyacyl-CoA is oxidized to beta-ketoacyl-CoA while NAD+ is reduced to NADH, which follows the same process as ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... Succinyl-CoA + GDP + Pi Succinate + CoA-SH + GTP Succinyl-CoA synthetase substrate-level. phosphorylation or ADP→ATP instead of ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... 223 (3): 650-3. doi:10.1006/bbrc.1996.0949. PMID 8687450.. *^ Li Y, Shi CX, Mossman KL, Rosenfeld J, Boo YC, Schellhorn HE ( ... 114 (3): 513-516. doi:10.2307/4089257.. *^ Pollock JI, Mullin RJ (May 1987). "Vitamin C biosynthesis in prosimians: evidence ... doi:10.1186/gb-2010-11-3-r26. PMC 2864566 . PMID 20210993.. *. Inai Y, Ohta Y, Nishikimi M (October 2003). "The whole structure ...
acyl-CoA dehydrogenase activity. • identical protein binding. • oxidoreductase activity. • medium-chain-acyl-CoA dehydrogenase ... ACADM, acyl-CoA dehydrogenase, C-4 to C-12 straight chain, ACAD1, MCAD, MCADH, acyl-CoA dehydrogenase medium chain. ... fatty acid beta-oxidation using acyl-CoA dehydrogenase. • fatty acid metabolic process. • metabolism. • oxidation-reduction ... Wang SS, Fernhoff PM, Hannon WH, Khoury MJ (1999). "Medium chain acyl-CoA dehydrogenase deficiency human genome epidemiology ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... The human version of this enzyme is known as hydroxy-Δ-5-steroid dehydrogenase, 3 β- and steroid delta-isomerase 7 or HSD3B7 ... Cholest-5-ene-3beta,7alpha-diol 3beta-dehydrogenase. From Wikipedia, the free encyclopedia ... hydroxy-Δ-5-steroid dehydrogenase, 3β- and steroid Δ-isomerase 7. ...
2,4 Dienoyl-CoA reductase. Odd chain. *Propionyl-CoA carboxylase. Other. *Hydroxyacyl-Coenzyme A dehydrogenase ... beta-hydroxyacyl-[acylcarrier-protein] dehydrase, and enoyl-[acyl-carrier-protein] reductase from Spinacia oleracea leaves". ... 3R)-3-hydroxyacyl-[acyl-carrier-protein] + NADP+ ⇌. {\displaystyle \rightleftharpoons }. 3-oxoacyl-[acyl-carrier-protein] + ... In enzymology, a 3-oxoacyl-[acyl-carrier-protein] reductase (EC 1.1.1.100) is an enzyme that catalyzes the chemical reaction ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... Carbohydrate dehydrogenases are a group of dehydrogenase enzymes that occur in many organisms and facilitate the conversion ... Carbohydrate dehydrogenases are the most common quinoprotein oxidoreductases,[1] which are enzymes that oxidize a wide range of ... Carbohydrate Dehydrogenases at the US National Library of Medicine Medical Subject Headings (MeSH) ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... cis-3,4-leucopelargonidin + NADP+ ⇌. {\displaystyle \rightleftharpoons }. (+)-dihydrokaempferol + NADPH + H+. Thus, the two ... The systematic name of this enzyme class is cis-3,4-leucopelargonidin:NADP+ 4-oxidoreductase. Other names in common use include ... DFR uses dihydromyricetin (ampelopsin) NADPH and 2 H+ to produce leucodelphinidin and NADP.[2][3] ...
Enoyl-CoA hydratase/3-Hydroxyacyl ACP dehydrase. *Methylglutaconyl-CoA hydratase. *Tryptophan synthase ... Oxoglutarate dehydrogenase *OGDH. *DLST. *DLD. *Branched-chain alpha-keto acid dehydrogenase complex *BCKDHA ... 3] Protista,[4] Fungi,[5] and Plantae.[6] However, it is absent from Animalia.[7] It is typically found as an α2β2 tetramer. ... The α subunits catalyze the reversible formation of indole and glyceraldehyde-3-phosphate (G3P) from indole-3-glycerol ...
2,4 Dienoyl-CoA reductase. Odd chain. *Propionyl-CoA carboxylase. Other. *Hydroxyacyl-Coenzyme A dehydrogenase ... They are synthesized by a series of decarboxylative Claisen condensation reactions from acetyl-CoA and malonyl-CoA. Following ... from acetyl-CoA and malonyl-CoA, in the presence of NADPH.[8] ... Malonyl-CoA synthesis. *ATP citrate lyase. *Acetyl-CoA ... acetyl-CoA metabolic process. • metabolism. • positive regulation of cellular metabolic process. • oxidation-reduction process ...
CoA) dehydrogenase, which is part of a protein complex known as mitochondrial trifunctional protein. Long-chain fatty acids ... Medium chain acyl dehydrogenase deficiency Reference, Genetics Home. "LCHAD deficiency". Genetics Home Reference. Retrieved ... Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, is a rare autosomal recessive fatty acid oxidation disorder that ... Mutations in the HADHA gene lead to inadequate levels of an enzyme called long-chain 3-hydroxyacyl-coenzyme A ( ...
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 ...
3-hydroxyacyl-CoA dehydrogenase deficiency is an inherited condition that prevents the body from converting certain fats to ... Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency. Biochem Biophys Res Commun. 2008 Mar 28;368(1):6-11. ... Short-chain hydroxyacyl-coenzyme A dehydrogenase deficiency presenting as unexpected infant death: A family study. J Pediatr. ... Problems related to 3-hydroxyacyl-CoA dehydrogenase deficiency can be triggered by periods of fasting or by illnesses such as ...
The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase ( ... Long-chain 3-hydroxy acyl-coenzyme A dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that make up the trifunctional ... Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency ( ... Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. Mol Genet Metab. 2013 May. 109(1):21-7. ...
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". The Journal of Biological Chemistry. 207 (2): 631-8. doi:10.1016/S0021-9258(18) ... Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme ...
Antibodies for proteins involved in 3-hydroxyacyl-CoA dehydrogenase activity pathways, according to their Panther/Gene Ontology ... Antibodies for proteins involved in 3-hydroxyacyl-CoA dehydrogenase activity pathways; according to their Panther/Gene Ontology ... Host server : magellan-srch-3-prod-blue:8080/10.253.228.147:8080. git-commit: b6907141bf9ff2f81bd54e0bfa97b1a010a74181 git-url ...
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. ...
3,6-tetrahydropyridine (MPTP) impairs mitochondrial respiration and damages dopaminergic neurons as seen in Parkinsons disease ... PD). Here, we report that L-3-hydroxyacyl-CoA dehydrogenase type II/amyloid binding alcohol dehydrogenase (HADH II/ABAD), a ... L-3-hydroxyacyl-CoA dehydrogenase II protects in a model of Parkinsons disease Ann Neurol. 2004 Jul;56(1):51-60. doi: 10.1002/ ... The neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) impairs mitochondrial respiration and damages dopaminergic ...
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 → ...
Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations Biochem J ... such as KATP channelopathies and regulatory defects of glucokinase and glutamate dehydrogenase. Although defects of fatty acid ... Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose-fatty acid cycle? S. ... β-oxidation, carnitine palmitoyltransferase I (CPT I), 3-hydroxyacyl-CoA dehydrogenase, hyperinsulinism ...
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 ...
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 ...
... glutamate dehydrogenase; immune response; recombinant proteins; surface proteins; vaccine development. Abstract:. ... ... and glutamate dehydrogenase (gdhA)) against Flavobacterium psychrophilum were investigated in ayu (Plecoglossus altivelis). ... 3-hydroxyacyl-CoA dehydrogenase; Escherichia coli; Flavobacterium psychrophilum; H+/K+-exchanging ATPase; H-transporting ATP ... Protective efficacies of three antigenic proteins (3-hydroxyacyl-CoA dehydrogenase (HCD), ATP synthase beta subunit (atpD), ...
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- ...
... 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. ...
... 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 ...
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 ...
Catalase and the endoplasmic reticulum A beta binding dehydrogenase (ERAB) are both inhibited by characterized fragments of the ... Catalase and the endoplasmic reticulum A beta binding dehydrogenase (ERAB) are both inhibited by characterized fragments of the ...
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 ...
isovaleryl-CoA dehydrogenase Ja 1.3.99.13 long-chain-acyl-CoA dehydrogenase Ja ... L-iditol 2-dehydrogenase Ja 1.1.1.15 D-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. D-sorbose + NADH + H+ D-iditol 2- ... D-arabitol 4-dehydrogenase 1.1.1.12 L-arabitol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-xylulose + NADH + H+ L-arabitol ... L-arabitol 2-dehydrogenase 1.1.1.14 L-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-sorbose + NADH + H+ ...
... a secondary marker for medium chain acyl-CoA dehydrogenase deficiency), immunoreactive trypsinogen (a primary marker for cystic ... Suppl 3):61-7.. * Clinical and Laboratory Standards Institute. Application of a quality management system model for laboratory ... RR-3).. * National Newborn Screening and Genetics Resource Center. National newborn screening status report. Austin, TX: ... 3, 5,11,15,19,20,30,33,34,41-49). Data also were collected from state programs (50,51), CDC studies (8,52,53), and publicly ...
The most important finding of this study was the detection of the lipid-metabolizing enzyme, 3-hydroxyacyl-CoA dehydrogenase ( ... From: Identification of leptospiral 3-hydroxyacyl-CoA dehydrogenase released in the urine of infected hamsters ...
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. ...
Short-chain dehydrogenase/reductase SDR * Occurring in:. *3-hydroxyacyl-CoA dehydrogenase type-2. > NAD(P)-binding domain ... 2-methylacetoacetyl-CoA + NADH. 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholanate + NAD(+) = 3-alpha,12-alpha-dihydroxy-7- ... Sequence domains: short chain dehydrogenase Structure domains: NAD(P)-binding Rossmann-like Domain ... Virtual screening applications in short-chain dehydrogenase/reductase research. Beck et al. (2017) ...
... 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- ...
Acyl-coa Dehydrogenase, Long-chain. A flavoprotein oxidoreductase that has specificity for long-chain fatty acids. It forms a ... Acetyl-coa C-acyltransferase. Enzyme that catalyzes the final step of fatty acid oxidation in which ACETYL COA is released and ... 3-hydroxyacyl Coa Dehydrogenases. Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in ... the CoA ester of a fatty acid two carbons shorter is formed. ... are based on 3 strategies: - limitation of lipid intake in the ...
Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Long chain/very long chain acyl CoA dehydrogenase deficiency, ... Long chain/very long chain acyl CoA dehydrogenase deficiency, ...
  • 3-hydroxyacyl-CoA dehydrogenase deficiency is an inherited condition that prevents the body from converting certain fats to energy, particularly during prolonged periods without food (fasting). (medlineplus.gov)
  • Problems related to 3-hydroxyacyl-CoA dehydrogenase deficiency can be triggered by periods of fasting or by illnesses such as viral infections. (medlineplus.gov)
  • Mutations in the HADH gene cause 3-hydroxyacyl-CoA dehydrogenase deficiency. (medlineplus.gov)
  • As a result, these fatty acids are not converted to energy, which can lead to characteristic features of 3-hydroxyacyl-CoA dehydrogenase deficiency, such as lethargy and hypoglycemia. (medlineplus.gov)
  • Conditions that disrupt the metabolism of fatty acids, including 3-hydroxyacyl-CoA dehydrogenase deficiency, are known as fatty acid oxidation disorders. (medlineplus.gov)
  • Fatal hepatic short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency: clinical, biochemical, and pathological studies on three subjects with this recently identified disorder of mitochondrial beta-oxidation. (medlineplus.gov)
  • IJlst L, Wanders RJ, Ushikubo S, Kamijo T, Hashimoto T. Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein. (medscape.com)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a severe fatty acid oxidation disorder. (medscape.com)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid beta-oxidation. (medscape.com)
  • Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast. (medscape.com)
  • Clinical, biochemical, and morphologic investigations of a case of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (medscape.com)
  • C rearrangement in patients with long chain 3-hydroxyacyl-CoA dehydrogenase deficiency from Ukraine. (medscape.com)
  • C of HADHA gene causing long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) in the population of adult Kashubians from North Poland. (medscape.com)
  • Clinical outcome, biochemical and therapeutic follow-up in 14 Austrian patients with Long-Chain 3-Hydroxy Acyl CoA Dehydrogenase Deficiency (LCHADD). (medscape.com)
  • Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myopathy and cardiomyopathy in early childhood. (medscape.com)
  • Schiff M, Mohsen AW, Karunanidhi A, McCracken E, Yeasted R, Vockley J. Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. (medscape.com)
  • Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxy-acyl-CoA dehydrogenase deficiency. (medscape.com)
  • Pigmentary retinopathy in long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. (medscape.com)
  • The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with acute fatty liver of pregnancy. (thefreedictionary.com)
  • Common missense mutation G1528 in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (thefreedictionary.com)
  • Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (thefreedictionary.com)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: high frequency of the G1528C mutation with no apparent correlation with clinical phenotype. (thefreedictionary.com)
  • Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose-fatty acid cycle? (portlandpress.com)
  • Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency : a cross-sectional study with long-term follow-up. (diva-portal.org)
  • 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. (diva-portal.org)
  • Unusual chorioretinal findings should alert the ophthalmologist to the long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, especially if there is a history of neonatal hypoglycaemia or failure to thrive. (diva-portal.org)
  • Babies with the diseases Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) or Trifunctional Protein Deficiency (TFP) are identified through newborn screening because they have an elevated C160H level. (newbornscreening.on.ca)
  • Mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency are long-chain fatty acid oxidation disorders with particularly high morbidity and mortality. (biomedcentral.com)
  • LCHAD (long-chain 3-hydroxyacyl CoA dehydrogenase) deficiency is a recessively inherited genetic disorder of fatty acid oxidation. (brainology.net)
  • 1996). Pathology of skeletal muscle and impaired respiratory chain function in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528 mutation. (brainology.net)
  • 1997). Pathology of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation. (brainology.net)
  • The five most commonly diagnosed conditions in the United States are 1) hearing loss, 2) primary congenital hypothyroidism, 3) cystic fibrosis, 4) sickle cell disease, and 5) medium-chain acyl-CoA dehydrogenase deficiency ( Table ) (3.6). (cdc.gov)
  • Hyperinsulinism due to short chain 3 hydroxylacyl-CoA dehydrogenase (SCHAD) deficiency is a recently described mitochondrial fatty acid oxidation disorder characterized by hyperinsulinemic hypoglycemia with seizures , and in one case fulminant hepatic failure. (cdc.gov)
  • We describe two children with deficiency of short-chain L-3-hydroxyacyl-CoA dehydrogenase, a new disorder of the mitochondrial beta-oxidation of straight-chain fatty acids. (nih.gov)
  • Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficiency: a systematic review. (cdc.gov)
  • for this reason this disorder is sometimes called medium- and short-chain 3-hydroxyacyl-coenzyme A dehydrogenase (M/SCHAD) deficiency. (wikipedia.org)
  • Individuals with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency are also at risk for complications such as seizures, life-threatening heart and breathing problems, coma, and sudden unexpected death. (wikipedia.org)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD deficiency) and Mitochondrial Trifunctional protein deficiency (MTP deficiency) also known as Trifunctional Protein (TFP) deficiency are disorders due to different defects in the MTP. (genedx.com)
  • Both isolated LCHAD deficiency and MTP deficiency, which has deficiencies in all 3 enzyme activities, have overlapping clinical presentations. (genedx.com)
  • Over the past decade, several clinical observations have demonstrated an association between these maternal syndromes and a recessively inherited fatty acid oxidation disorder, long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency. (biomedsearch.com)
  • D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein deficiency : a newly identified peroxisomal disorder. (nii.ac.jp)
  • Deficiency of the mitochondrial enzyme 2-methyl-3-hydroxybutyryl-CoA dehydrogenase involved in isoleucine metabolism causes an organic aciduria with atypical neurodegenerative course. (semanticscholar.org)
  • HADHA-related disorders, including long chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) and mitochondrial trifunctional protein deficiency (MTPD), are inherited conditions in which the body lacks sufficient amounts of the enzyme(s) needed to turn certain fatty acids from food and body fat into energy. (counsyl.com)
  • The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. (genecards.org)
  • EHHADH (Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase) is a Protein Coding gene. (genecards.org)
  • Subunit activities include: enoyl-CoA hydratase ( EC:4.2.1.17 ) and 3-hydroxyacyl-CoA dehydrogenase ( EC:1.1.1.35 ). (ebi.ac.uk)
  • The HADH gene provides instructions for making an enzyme called 3-hydroxyacyl-CoA dehydrogenase. (medlineplus.gov)
  • Mutations in the HADH gene lead to a shortage of 3-hydroxyacyl-CoA dehydrogenase. (medlineplus.gov)
  • In humans, the following genes encode proteins with 3-hydroxyacyl-CoA dehydrogenase activity: HADH - Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme HSD17B4 - Peroxisomal multifunctional enzyme type 2 3-Hydroxyacyl CoA dehydrogenase is classified as an oxidoreductase. (wikipedia.org)
  • Here, we report that L-3-hydroxyacyl-CoA dehydrogenase type II/amyloid binding alcohol dehydrogenase (HADH II/ABAD), a mitochondrial oxidoreductase enzyme involved in neuronal survival, is downregulated in PD patients and in MPTP-intoxicated mice. (nih.gov)
  • It is caused by a mutation in the HADH gene (4q22-q26) encoding the SCHAD protein that has a dual function both as an enzyme and an inhibitor of glutamate dehydrogenase. (cdc.gov)
  • Mutations in the HADH gene lead to inadequate levels of an enzyme called 3-hydroxyacyl-coenzyme A dehydrogenase. (wikipedia.org)
  • HADH (Hydroxyacyl-CoA Dehydrogenase) is a Protein Coding gene. (genecards.org)
  • In enzymology, a 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35) is an enzyme that catalyzes the chemical reaction (S)-3-hydroxyacyl-CoA + NAD+ ⇌ {\displaystyle \rightleftharpoons } 3-oxoacyl-CoA + NADH + H+ Thus, the two substrates of this enzyme are (S)-3-hydroxyacyl-CoA and NAD+, whereas its 3 products are 3-oxoacyl-CoA, NADH, and H+. (wikipedia.org)
  • Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase. (medlineplus.gov)
  • this was accompanied by the up-regulation of beta-oxidation activity, and acyl-CoA oxidase (ACO) and medium-chain acyl-CoA dehydrogenase (MCAD) mRNA levels. (biomedsearch.com)
  • What energy molecule does Acyl CoA Dehydrogenase create? (brainscape.com)
  • A simple, straightforward assay for long-chain 3-hydroxyacyl-CoA dehydrogenase based on the use of 4ethylmaleimide: potential for pre and postnatal diagnosis. (thefreedictionary.com)
  • Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficien. (cdc.gov)
  • Mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiencies are rare fatty acid β-oxidation disorders. (cdc.gov)
  • MTP is an enzyme complex at the inner mitochondrial membrane with three enzymatic activities, long-chain 3-hydroxyacyl-CoA dehydrogenase, 2-enoyl hydratase and 3-keto acyl-CoA thiolase activities. (genedx.com)
  • 4. Sund, H. and Theorell, H. Alcohol dehydrogenase. (qmul.ac.uk)
  • 5. Theorell, H. Kinetics and equilibria in the liver alcohol dehydrogenase system. (qmul.ac.uk)
  • alcohol dehydrogenase 4 (class II), pi po. (broadinstitute.org)
  • Several molecular defects are known to cause hyperinsulinism of infancy, such as K ATP channelopathies and regulatory defects of glucokinase and glutamate dehydrogenase. (portlandpress.com)
  • Protective efficacies of three antigenic proteins (3-hydroxyacyl-CoA dehydrogenase (HCD), ATP synthase beta subunit (atpD), and glutamate dehydrogenase (gdhA)) against Flavobacterium psychrophilum were investigated in ayu (Plecoglossus altivelis). (usda.gov)
  • 1 In rarer cases of PHHI, mutations in the glucokinase gene ( GCK ), glutamate dehydrogenase gene ( GLUD1 ), and short-chain hydroxyacyl-coenzyme A dehydrogenase ( HADHSC ) gene are pathogenic and act mainly by subverting the operation of K ATP channels. (aappublications.org)
  • In other cases, including those involving mutations in glucokinase, glutamate dehydrogenase, and short-chain hydroxyacyl-coenzyme A dehydrogenase genes, diazoxide and appropriate diet are required for a longer time but are very effective in controlling hypoglycemia. (aappublications.org)
  • The 3-hydroxyacyl-CoA dehydrogenase enzyme is required for a step that metabolizes groups of fats called medium-chain fatty acids and short-chain fatty acids. (medlineplus.gov)
  • Belongs to the short-chain dehydrogenases/reductases (SDR) family. (abcam.com)
  • Some individuals have HSD17B10 gene mutations that affect only the breakdown of 2-methyl-3-hydroxybutyryl-CoA, which can be detected with laboratory tests, but do not have the signs and symptoms of HSD10 disease. (nih.gov)
  • The disease-causing gene is HSD17B10 and encodes 17beta-hydroxysteroid dehydrogenase type 10 (HSD10), a protein also implicated in the pathogenesis of Alzheimer's disease. (semanticscholar.org)
  • The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase (LCKT). (brainology.net)
  • This enzyme participates in 8 metabolic pathways: fatty acid elongation in mitochondria fatty acid metabolism valine, leucine and isoleucine degradation lysine degradation tryptophan metabolism benzoate degradation via coa ligation butanoate metabolism caprolactam degradation The systematic name of this enzyme class is (S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase. (wikipedia.org)
  • 3-HIBA is an intermediate in L-valine metabolism. (hmdb.ca)
  • In this review we discuss how metabolomics has contributed so far to characterizing TB, with emphasis on the resulting improved elucidation of M. tuberculosis in terms of (1) metabolism, (2) growth and replication, (3) pathogenicity, and (4) drug resistance, from the perspective of systems biology. (hindawi.com)
  • LCHAD is 1 of 3 enzymatic activities that make up the mitochondrial trifunctional protein (MTP) of the inner mitochondrial membrane. (brainology.net)
  • 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. (genecards.org)
  • In order to clarify the pathogenesis of X-linked adrenoleukodystrophy (ALD), complementary DNA for human very long chain fatty acyl-CoA synthetase (VLACS), which is deficient in ALD was cloned using rat cDNA.Human VLACS cDNA encodes 620 amino acids with high homology to rat enzyme and fatty acid transport protein and the gene was assinged to chromosome 15q21.2. (nii.ac.jp)
  • actin related protein 2/3 complex, subu. (broadinstitute.org)
  • 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. (diabetesjournals.org)
  • Overexpression of 17β-hydroxysteroid dehydrogenase type 10 increases pheochromocytoma cell growth and resistance to cell death. (nih.gov)
  • 17β-hydroxysteroid dehydrogenase type 10 (HSD10) has been shown to play a protective role in cells undergoing stress. (nih.gov)
  • 2-methyl-3-hydroxybutyric aciduria is an inherited disorder in which the body cannot effectively process the amino acid isoleucine. (nih.gov)
  • 603178 ). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. (hmdb.ca)
  • The beta subunit has acetyl-CoA C-acyltransferase ( EC:2.3.1.16 ) activity. (ebi.ac.uk)
  • The second and third reactions in the peroxisomal β-oxidation cycle ( Figure 1 ) are performed by an MFE (multifunctional enzyme) that consists of one single polypeptide chain harbouring both dehydrogenase and hydratase activities. (portlandpress.com)
  • Our results reveal that rapamycin inhibits the phosphorylation of TORC1 and its downstream effectors (S6K1, S6 and 4E-BP1), without affecting Akt and the Akt substrates Forkhead-box Class O1 (FoxO1) and glycogen synthase kinase 3α/β (GSK 3α/β). (biologists.org)
  • 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. (diabetesjournals.org)
  • HSD10 also generates a potent androgen called dihydrotestosterone from a weak androgen called 3α-androstanediol. (nih.gov)
  • A-B. Densitometry of MTT reduction in EV and HSD10 ov cells treated with A) 0, 0.1, 0.25, 0.5, 0.75, and 1 mM H2O2 (n = 3 for all groups), and B) 0, 0.1, and 0.25 mM TBH for 24 hours (n = 3 for all groups). (nih.gov)
  • C-D. ETC. complex IV enzyme activity was assessed in EV and HSD10 ov cells treated with 0.75 mM H2O2 for C) 24 hours (n = 3), and D) 0, 1, 6, and 16 hours (n = 6 for all time points). (nih.gov)
  • Recombinant fragment containing a sequence corresponding to a region within amino acids 18-231 of Human hydroxysteroid (17-beta) dehydrogenase 4 (AAH03098). (abcam.com)
  • Acyl-CoA esters are oxidized in the β-oxidation spiral that is the major pathway for the breakdown of fatty acids and is found, depending on the species, in peroxisomes and mitochondria. (portlandpress.com)
  • Leigh Syndrome is a rare genetic disorder caused by mutations in mitochondrial DNA or by deficiencies of an enzyme called pyruvate dehydrogenase. (rareshare.org)
  • Cecatto C, Godoy KDS, da Silva JC, Amaral AU, Wajner M. Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle. (medscape.com)
  • Congenital hyperinsulinism is the most common cause of hypoglycemia (low blood sugar) in infants more than 3 days old, as well as children. (cookchildrens.org)
  • 2 , 3 , 5 , 6 However, almost 80% of neonates with congenital hyperinsulinism fail to respond to medical treatment and require near-total pancreatectomy. (aappublications.org)
  • Both peroxisomal MFE-1 and the membrane-associated mitochondrial trifunctional enzyme complex house (3 S )-hydroxy-specific dehydrogenase [ 4 , 5 ]. (portlandpress.com)
  • These are organic compounds containing a 3-hydroxyl acylated coenzyme A derivative. (hmdb.ca)
  • A molecule of coenzyme A carrying an acetyl group is also referred to as acetyl-CoA. (hmdb.ca)
  • These are organic compounds containing a coenzyme A derivative, which is 3-oxo acylated long aliphatic chain of 13 to 21 carbon atoms. (hmdb.ca)
  • Among its related pathways are Mitochondrial Fatty Acid Beta-Oxidation and Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA . (genecards.org)
  • Enzymes catalyzing reactions in phosphagenic [creatine kinase (CK)] and glycolytic [hexokinase (HK), phosphofructokinase (PFK), and lactate dehydrogenase (LDH)] metabolic pathways had significantly higher activities (about 30-40%) in the Black African group than in the Caucasian group (P less than 0.01). (physicsforums.com)
  • Exerts it highest activity toward 3-hydroxybutyryl-CoA. (genecards.org)
  • 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 Alzheimer's disease . (alzforum.org)
  • The SOL muscles of SED SPORTS rats also contained more activity of β -hydroxy acylCoA dehydrogenase, a key enzyme of β -oxidation, indicating enhanced lipid oxidation. (scirp.org)
  • This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. (genecards.org)
  • 3 , 4 The overall experience of using PGD for single gene disorders is presently almost 1500 cases, showing that it is an established alternative to traditional prenatal diagnosis, which may be reliably applied as an integral part of genetic practices. (glowm.com)
  • AFG3 ATPase family gene 3-like 2 (S. ce. (broadinstitute.org)
  • Catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. (abcam.com)
  • belongs to the class of organic compounds known as long-chain 3-oxoacyl coas. (hmdb.ca)