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

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)

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

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)

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

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)

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

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)

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

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)

(6/341) 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.

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)

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

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)

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

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)

*  Fatty acid metabolism
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 ...
*  3-hydroxyacyl-CoA dehydrogenase
L-hydroxyacyl-CoA dehydrogenase 3beta-hydroxyacyl coenzyme A dehydrogenase beta-hydroxy acid dehydrogenase beta-hydroxyacyl CoA ... dehydrogenase beta-hydroxyacyl dehydrogenase beta-hydroxyacyl-coenzyme A synthetase beta-hydroxyacylcoenzyme A dehydrogenase ... beta-Hydroxyacyl coenzyme A dehydrogenase". J. Biol. Chem. 207 (2): 631-8. PMID 13163047. Molecular and Cellular Biology portal ... Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme ...
*  3-hydroxyacyl-coenzyme A dehydrogenase deficiency
People with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency have inadequate levels of an enzyme required for a step that ... Problems related to 3-hydroxyacyl-coenzyme A dehydrogenase deficiency can be triggered by periods of fasting or by illnesses ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (HADH deficiency) is a rare condition that prevents the body from converting ... Individuals with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency are also at risk for complications such as seizures, life- ...
*  List of OMIM disorder codes
SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... PC Pyruvate dehydrogenase deficiency; 312170; PDHA1 Pyruvate dehydrogenase E2 deficiency; 245348; DLAT Pyruvate dehydrogenase ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... TMPRSS6 Isobutyryl-CoA dehydrogenase deficiency; 611283; ACAD8 Isovaleric acidemia; 243500; IVD IVIC syndrome; 147750; SALL4 ...
*  Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency
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, often shortened to LCHAD deficiency, is a rare autosomal ... Mutations in the HADHA gene lead to inadequate levels of an enzyme called long-chain 3-hydroxyacyl-coenzyme A ( ...
*  3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase
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 ...
*  HADHA
"hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit". Cardiac ... Trifunctional enzyme subunit alpha, mitochondrial also known as hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA ... Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit". Zong NC, Li ... The enzyme converts medium- and long-chain 2-enoyl-CoA compounds into the following 3-ketoacyl-CoA when NAD is solely present, ...
*  Hydroxyacyl-Coenzyme A dehydrogenase
... such that HIV-1 Vpr regulates mitochondrial respiration and enhances the activity of hydroxyacyl-CoA dehydrogenase (HADH) ... Hydroxyacyl-Coenzyme A dehydrogenase also known as HADH is an enzyme which in humans is encoded by the HADH gene. The HADH gene ... "Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB). Molven A, Matre ... "A preliminary analysis of the segregation of human hydroxyacyl coenzyme A dehydrogenase in human-mouse somatic cell hybrids". ...
*  EHHADH
HSD17B4 "EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. 6 September 2017. ...
*  Acute fatty liver of pregnancy
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 ...
*  HADHB
... hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein)". ]Zong ... which yields an acetyl CoA molecule and an acyl CoA molecule, which is two carbons shorter. The encoded protein can also bind ... Middleton B (1994). "The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase ... acetyl-CoA acyltransferase, or beta-ketothiolase is an enzyme that in humans is encoded by the HADHB gene. HADHB is a subunit ...
*  HSD17B10
"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- ...
*  Mitochondrial trifunctional protein deficiency
"OMIM Entry - * 600890 - HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, ALPHA SUBUNIT; HADHA". omim. ... "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Homo ... "Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ...
*  HSD17B4
1998). "Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in ... acyl-CoA oxidase (see, e.g., ACOX1, MIM 609751); the 'D-bifunctional enzyme,' with enoyl-CoA hydratase and D-3-hydroxyacyl-CoA ... 1999). "17Beta-hydroxysteroid dehydrogenases in human bone cells". J. Bone Miner. Res. 13 (10): 1539-46. doi:10.1359/jbmr. ... 1999). "17Beta-hydroxysteroid dehydrogenase type 1, 2, 3, and 4 expression and enzyme activity in human anterior pituitary ...
*  HELLP syndrome
Lactate dehydrogenase is a marker of hemolysis and is elevated (>600 U/l). Proteinuria is present but can be mild. In one 1995 ... HELLP is characterized by hemolysis on peripheral blood smear with serum lactate dehydrogenase >600 IU/l; serum aspartate ... 60 (3): 829-36, 839. PMID 10498110. Gillingham M, Van Calcar S, Ney D, Wolff J, Harding C. Dietary management of long-chain 3- ... hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey. Journal of inherited metabolic disease. 1999;22(2 ...
*  Enoyl CoA isomerase
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 ...
*  Acetoacetyl-CoA reductase
... 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- ...
*  Lipid metabolism
Acyl CoA dehydrogenase, Enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase. Since lipids (fats) ... The resulting Acyl-CoA activates the process of beta oxidation to further break down into Acetyl-CoA (which is used in the ... Fatty acid metabolism begins in the cytoplasm of epithelial cells as Acyl-CoA synthetase and hydrolysis of ATP cleaves a ... King, Michael W. Fatty Acid, Omega-3 and Omega-6 Fatty Acid, Triglyceride, and Phospholipid Synthesis and Metabolism. The ...
*  List of EC numbers (EC 1)
... glutaryl-CoA dehydrogenase EC 1.3.8.7: medium-chain acyl-CoA dehydrogenase EC 1.3.8.8: long-chain acyl-CoA dehydrogenase EC 1.3 ... glutaryl-CoA dehydrogenase EC 1.3.99.8: 2-furoyl-CoA dehydrogenase EC 1.3.99.9: now *EC 1.21.99.1 EC 1.3.99.10: isovaleryl-CoA ... succinate dehydrogenase EC 1.3.99.2: butyryl-CoA dehydrogenase EC 1.3.99.3: acyl-CoA dehydrogenase EC 1.3.99.4: 3-oxosteroid 1- ... benzylsuccinyl-CoA dehydrogenase EC 1.3.8.4: isovaleryl-CoA dehydrogenase EC 1.3.8.5: 2-methyl-branched-chain-enoyl-CoA ...
*  List of causes of hypoglycemia
... 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 ...
*  List of MeSH codes (D08)
... acyl-coa dehydrogenases MeSH D08.811.682.660.150.100 --- acyl-coa dehydrogenase MeSH D08.811.682.660.150.150 --- acyl-coa ... Glutaryl-CoA dehydrogenase MeSH D08.811.682.660.462 --- isovaleryl-coa dehydrogenase MeSH D08.811.682.660.490 --- 15- ... acyl-CoA oxidase MeSH D08.811.682.660.150.300 --- butyryl-coa dehydrogenase MeSH D08.811.682.660.200 --- cholestenone 5alpha- ... prephenate dehydrogenase MeSH D08.811.399.520.625 --- methylmalonyl-coa mutase MeSH D08.811.399.520.750 --- phosphotransferases ...
*  Short-chain acyl-CoA dehydrogenase
... (EC 1.3.8.1, butyryl-CoA dehydrogenase, butanoyl-CoA dehydrogenase, butyryl dehydrogenase, ... butyryl coenzyme A dehydrogenase, short-chain acyl CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, 3-hydroxyacyl ... Short-chain acyl-CoA dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH) Molecular and ... Thorpe, C.; Kim, J.J. (1995). "Structure and mechanism of action of the acyl-CoA dehydrogenases". FASEB J. 9 (9): 718-725. PMID ...
*  Acetyl-CoA
... 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 ...
*  Beta oxidation
... 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 ...
*  List of disorders included in newborn screening programs
Short-chain hydroxy Acyl-CoA dehydrogenase deficiency (SCHAD) Long-chain acyl-CoA dehydrogenase deficiency (LCAD) Multiple acyl ... 1 in 75,000 Medium-chain acyl-CoA dehydrogenase deficiency (MCAD) > 1 in 25,000 Very-long-chain acyl-CoA dehydrogenase ... butyric aciduria Isobutyryl-CoA dehydrogenase deficiency 2-Methylbutyryl-CoA dehydrogenase deficiency 3-Methylglutaconyl-CoA ... 1 in 100,000 Inborn errors of fatty acid metabolism Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) > ...
*  3-hydroxybutyryl-CoA epimerase
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 ...
Michael J. Bennett, PhD - AACC.org  Michael J. Bennett, PhD - AACC.org
... the fatal clinical phenotype and the first to identify neonatal metabolite abnormalities in medium-chain acyl-CoA dehydrogenase ... He is currently studying the hyperinsulinism associated with deficiency of short-chain L-3-hydroxyacyl-CoA dehydrogenase (SCHAD ...
more infohttps://www.aacc.org/Community/Awards/Hall-of-Fame/Bios/L-to-S/Michael-Bennett.aspx
3HYDROXYACYLCoADEHYDROGENASEDEFICIENCYSCHAD  3HYDROXYACYLCoADEHYDROGENASEDEFICIENCYSCHAD
Genetic Information Center Inborn erros of metabolism 3-HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY (SCHAD) HADH DEFICIENCY, SCHAD ... Short-chain 3-hydroxyacyl-CoA dehydrogenase. 2.48. 5.10. 8.70. -Ámol/min/g tissue. muscle. ... 3-Hydroxybutyric acid. 0.00. 0.00. 0.00. 3.00. increased. urine. childhood 6-12y. ... short-chain L-3-hydroxyacyl-CoA (SCHAD, HADH) deficiency is characterised by hypoglycemia with hyperinsulinism in the neonatal ...
more infohttp://www.metagene.de/disease/3HYDROXYACYLCoADEHYDROGENASEDEFICIENCYSCHAD_393.html
Fatty acid metabolism - Wikipedia  Fatty acid metabolism - Wikipedia
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 ...
more infohttps://en.wikipedia.org/wiki/Fatty_acid_metabolism
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial  Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
HIV-1 Vpr regulates mitochondrial respiration and enhances the activity of hydroxyacyl-CoA dehydrogenase (HADH) through ... Acetoacetyl-CoA ( Butanoate metabolism ) 4 NAD+ + (S)-3-Hydroxy-butanoyl-CoA = NADH + Acetoacetyl-CoA ( Tryptophan degradation ... Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) 8 Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty ... NAD+ + (S)-3-Hydroxy-2-methyl-butanoyl-CoA = NADH + 2-Methyl-3-acetoacetyl-CoA ( Valine,Leucine and Isoleucine degradation ) 4 ...
more infohttps://pharos.nih.gov/idg/targets/Q16836
Acyl-CoA dehydrogenase, very long chain, deficiency of (Symptoms, signs, causes, treatments & definition) - Medigest  Acyl-CoA dehydrogenase, very long chain, deficiency of (Symptoms, signs, causes, treatments & definition) - Medigest
Medigest has all you need to know about Acyl-CoA dehydrogenase, very long chain, deficiency of - Symptoms and Signs, Causes, ... Looking for information on Acyl-CoA dehydrogenase, very long chain, deficiency of? ... Acyl-CoA dehydrogenase, very long chain, deficiency of Below you will find more information about Acyl-CoA dehydrogenase, very ... Discuss Acyl-CoA dehydrogenase, very long chain, deficiency of in our forums Discuss Acyl-CoA dehydrogenase, very long chain, ...
more infohttps://www.medigest.uk/diseases/acyl-coa-dehydrogenase-very-long-chain-deficiency-of/
3-hydroxyacyl-CoA dehydrogenase - Wikipedia  3-hydroxyacyl-CoA dehydrogenase - Wikipedia
L-hydroxyacyl-CoA dehydrogenase 3beta-hydroxyacyl coenzyme A dehydrogenase beta-hydroxy acid dehydrogenase beta-hydroxyacyl CoA ... dehydrogenase beta-hydroxyacyl dehydrogenase beta-hydroxyacyl-coenzyme A synthetase beta-hydroxyacylcoenzyme A dehydrogenase ... beta-Hydroxyacyl coenzyme A dehydrogenase". J. Biol. Chem. 207 (2): 631-8. PMID 13163047. Molecular and Cellular Biology portal ... Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme ...
more infohttps://en.wikipedia.org/wiki/3-hydroxyacyl-CoA_dehydrogenase
3-hydroxyacyl-CoA dehydrogenase | definition of 3-hydroxyacyl-CoA dehydrogenase by Medical dictionary  3-hydroxyacyl-CoA dehydrogenase | definition of 3-hydroxyacyl-CoA dehydrogenase by Medical dictionary
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-drok'sē-as'il dē'hī-drō'jen-ā), β-Hydroxyacyl dehydrogenase; an enzyme catalyzing the oxidation ... 3-hydroxyacyl-CoA dehydrogenase. Also found in: Acronyms, Wikipedia. 3-hy·drox·y·ac· ...
more infohttp://medical-dictionary.thefreedictionary.com/3-hydroxyacyl-CoA+dehydrogenase
Human Metabolome Database: Showing Protein 3-hydroxyacyl-CoA dehydrogenase type-2 (HMDBP00379)  Human Metabolome Database: Showing Protein 3-hydroxyacyl-CoA dehydrogenase type-2 (HMDBP00379)
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 → ...
more infohttp://www.hmdb.ca/proteins/HMDBP00379
Structure Cluster 









- 1E3W: Rat brain 3-hydroxyacyl-CoA dehydrogenase binary complex with NADH and 3-keto butyrate 3D...  Structure Cluster - 1E3W: Rat brain 3-hydroxyacyl-CoA dehydrogenase binary complex with NADH and 3-keto butyrate 3D...
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 ...
more infohttp://www.rcsb.org/pdb/explore/structureCluster.do?structureId=1E3W
Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) | CHEO NSO  Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) | CHEO NSO
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 ...
more infohttps://www.newbornscreening.on.ca/en/node/197
Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency : a cross-sectional study with...  Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency : a cross-sectional study with...
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 ...
more infohttp://uu.diva-portal.org/smash/record.jsf?faces-redirect=true&language=sv&searchType=SIMPLE&query=&af=%5B%5D&aq=%5B%5B%5D%5D&aq2=%5B%5B%5D%5D&aqe=%5B%5D&pid=diva2%3A220279&noOfRows=50&sortOrder=author_sort_asc&sortOrder2=title_sort_asc&onlyFullText=false&sf=all
Fatal pitfalls in newborn screening for mitochondrial trifunctional protein (MTP)/long-chain 3-Hydroxyacyl-CoA dehydrogenase ...  Fatal pitfalls in newborn screening for mitochondrial trifunctional protein (MTP)/long-chain 3-Hydroxyacyl-CoA dehydrogenase ...
... 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 ...
more infohttps://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0875-6
Vitamin E Treatment for Long-Chain 3-Hydroxyacyl Coenzyme A (CoA) Dehydrogenase (LCHAD) Associated Neuropathy - Full Text View ...  Vitamin E Treatment for Long-Chain 3-Hydroxyacyl Coenzyme A (CoA) Dehydrogenase (LCHAD) Associated Neuropathy - Full Text View ...
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 ( ...
more infohttps://clinicaltrials.gov/ct2/show/NCT00840112?cond=%22Mitochondrial+trifunctional+protein+deficiency%22&rank=1
LCHAD (Long-Chain 3-Hydroxyacyl CoA Dehydrogenase) deficiency  LCHAD (Long-Chain 3-Hydroxyacyl CoA Dehydrogenase) deficiency
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 ...
more infohttp://www.brainology.net/neurology/lchad.html
GeneDx  GeneDx
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, ...
more infohttps://www.genedx.com/test-catalog/disorders/long-chain-3-hydroxyacyl-coa-dehydrogenase-deficiency-lchad/
EHHADH Gene - GeneCards | ECHP Protein | ECHP Antibody  EHHADH Gene - GeneCards | ECHP Protein | ECHP Antibody
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 ...
more infohttps://www.genecards.org/cgi-bin/carddisp.pl?gene=EHHADH
Anti-Alcohol Dehydrogenase 2 Antibody Products | Biocompare.com  Anti-Alcohol Dehydrogenase 2 Antibody Products | Biocompare.com
Compare Anti-Alcohol Dehydrogenase 2 Antibody Products from leading suppliers on Biocompare. View specifications, prices, ... Rabbit anti-human zinc binding alcohol dehydrogenase domain containing 2 polyclonal Antibody ...
more infohttps://www.biocompare.com/pfu/110447/soids/144790/Antibodies/Alcohol_Dehydrogenase_2
3-hydroxyacyl-coenzyme A dehydrogenase deficiency - Wikipedia  3-hydroxyacyl-coenzyme A dehydrogenase deficiency - Wikipedia
People with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency have inadequate levels of an enzyme required for a step that ... Problems related to 3-hydroxyacyl-coenzyme A dehydrogenase deficiency can be triggered by periods of fasting or by illnesses ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (HADH deficiency) is a rare condition that prevents the body from converting ... Individuals with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency are also at risk for complications such as seizures, life- ...
more infohttps://en.wikipedia.org/wiki/3-hydroxyacyl-coenzyme_A_dehydrogenase_deficiency
Fatty acid oxidation complex, alpha subunit, mitochondrial (IPR012803) | InterPro | EMBL-EBI  Fatty acid oxidation complex, alpha subunit, mitochondrial (IPR012803) | InterPro | EMBL-EBI
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. ...
more infohttp://www.ebi.ac.uk/interpro/entry/IPR012803
CPT1a-Dependent Long-Chain Fatty Acid Oxidation Contributes to Maintaining Glucagon Secretion from Pancreatic Islets.  CPT1a-Dependent Long-Chain Fatty Acid Oxidation Contributes to Maintaining Glucagon Secretion from Pancreatic Islets.
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 ...
more infohttps://www.bioportfolio.com/resources/pmarticle/2069128/CPT1a-Dependent-Long-Chain-Fatty-Acid-Oxidation-Contributes-to-Maintaining-Glucagon-Secretion.html
Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy - Tabular View - ClinicalTrials.gov  Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy - Tabular View - ClinicalTrials.gov
Medium-chain Acyl-CoA Dehydrogenase Deficiency. *Multiple Acyl-CoA Dehydrogenase Deficiency. *Carnitine Transporter Deficiency ... Test day 3:. •Long exercise test #2. The purpose of this test is to investigate exercise capacity and performance measured as ... Subjects arrive at the laboratory after 3-9 hours of fasting. One IV-catheter is inserted in the cubital vein in one arm and ... Subjects arrive at the laboratory after 3-9 hours fasting. After 2 hours of rest, the subjects exercise on the cycle-ergometer ...
more infohttps://clinicaltrials.gov/ct2/show/record/NCT02635269?recrs=abc&cond=%22Muscular+Diseases%22&rank=26
fadJ - Fatty acid oxidation complex subunit alpha - Salmonella paratyphi A (strain ATCC 9150 / SARB42) - fadJ gene & protein  fadJ - Fatty acid oxidation complex subunit alpha - Salmonella paratyphi A (strain ATCC 9150 / SARB42) - fadJ gene & protein
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. ... Catalyzes the formation of a hydroxyacyl-CoA by addition of ... Enoyl-CoA hydratase/3-hydroxybutyryl-CoA epimeraseUniRule annotation. ,p>Manual validated information which has been generated ... Enoyl-CoA hydrataseUniRule annotation. ,p>Manual validated information which has been generated by the UniProtKB automatic ...
more infohttp://www.uniprot.org/uniprot/Q5PCX6
fadB - Fatty acid oxidation complex subunit alpha - Vibrio campbellii (strain ATCC BAA-1116 / BB120) - fadB gene & protein  fadB - Fatty acid oxidation complex subunit alpha - Vibrio campbellii (strain ATCC BAA-1116 / BB120) - fadB gene & protein
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 hydratase/Delta(3)-cis-Delta(2)-trans-enoyl-CoA isomerase/3-hydroxybutyryl-CoA epimeraseUniRule annotation. ,p>Manual ... Enoyl-CoA hydratase/isomeraseUniRule annotation. ,p>Manual validated information which has been generated by the UniProtKB ... In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.UniRule annotation. ,p>Manual validated ...
more infohttp://www.uniprot.org/uniprot/A7N1D2
HADH Gene - GeneCards | HCDH Protein | HCDH Antibody  HADH Gene - GeneCards | HCDH Protein | HCDH Antibody
Hydroxyacyl-CoA Dehydrogenase, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The ... HADH (Hydroxyacyl-CoA Dehydrogenase) is a Protein Coding gene. Diseases associated with HADH include 3-Hydroxyacyl-Coa ... Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA. Beta oxidation of octanoyl-CoA to hexanoyl-CoA ... Among its related pathways are Mitochondrial Fatty Acid Beta-Oxidation and Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA. ...
more infohttp://www.genecards.org/cgi-bin/carddisp.pl?id_type=entrezgene&id=3033
Frontiers | Transcriptomic Analysis of Thermally Stressed Symbiodinium Reveals Differential Expression of Stress and Metabolism...  Frontiers | Transcriptomic Analysis of Thermally Stressed Symbiodinium Reveals Differential Expression of Stress and Metabolism...
... acyl-CoA dehydrogenase, enoyl-CoA hydratase, HCDH, and β-ketothiolase) of the fatty acid β-oxidation pathway (Figure 4B; ... acyl-CoA dehydrogenase; ech, enoyl-CoA hydratase; fadj, fatty acid oxidation complex subunit; mfea, peroxisomal multifunctional ... Six transcripts encoding acyl-CoA dehydrogenases (ACADs) were detected with differential expression over the course of the ... malate dehydrogenase; sdh, succinate dehydrogenase (ubiquinone) flavoprotein subunit; pepck, phosphoenolpyruvate carboxykinase ...
more infohttps://www.frontiersin.org/articles/10.3389/fpls.2017.00271/full
  • Once inside the cell long-chain-fatty-acid-CoA ligase catalyzes the reaction between a fatty acid molecule with ATP (which is broken down to AMP and inorganic pyrophosphate) to give a fatty acyl-adenylate, which then reacts with free coenzyme A to give a fatty acyl-CoA molecule. (wikipedia.org)
  • It is convenient to think of this reaction as marking the "starting point" of the cycle, as this is when fuel - acetyl-CoA - is added to the cycle, which will be dissipated as CO2 and H2O with the release of a substantial quantity of energy captured in the form of ATP, during the course of each turn of the cycle. (wikipedia.org)
  • 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)
  • Purification and characterization of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. (wikipedia.org)
  • This citrate is then exported to other organelles outside the mitochondria to be broken into acetyl-CoA and oxaloacetate by the enzyme ATP citrate lyase (ACL). (wikipedia.org)
  • All cells with mitochondria can take ketone bodies up from the blood and reconvert them into acetyl-CoA, which can then be used as fuel in their citric acid cycles, as no other tissue can divert its oxaloacetate into the gluconeogenic pathway in the way that the liver does. (wikipedia.org)
  • The D-bifunctional protein catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids and also acts in shortening cholesterol for bile acid formation. (wikipedia.org)
  • Enoyl-CoA-(∆) isomerase, also known as dodecenoyl-CoA-(∆) isomerase, 3,2-trans-enoyl-CoA isomerase, ∆3(cis),∆2(trans)-enoyl-CoA isomerase, or acetylene-allene isomerase, (EC 5.3.3.8) is an enzyme that catalyzes the conversion of cis-or trans-double bonds of fatty acids at gamma-carbon (position 3) to trans double bonds at beta-carbon (position 2). (wikipedia.org)
  • Since the key step in the degradation of fatty acids with double bonds at even-numbered carbon positions also produces 3-trans-enoyl-CoA in mammals and yeasts, enoyl-CoA isomerase is technically required for their metabolism as well. (wikipedia.org)
  • Fatty acids are first converted to acyl-CoA. (wikipedia.org)
  • If the fatty acyl-CoA contains a short chain, these short-chain fatty acids can simply diffuse through the inner mitochondrial membrane. (wikipedia.org)
  • The beta subunit has acetyl-CoA C-acyltransferase ( EC:2.3.1.16 ) activity. (ebi.ac.uk)
  • The enzyme converts medium- and long-chain 2-enoyl-CoA compounds into the following 3-ketoacyl-CoA when NAD is solely present, and acetyl-CoA when NAD and CoASH are present. (wikipedia.org)
  • It is convenient to think of this reaction as marking the "starting point" of the cycle, as this is when fuel - acetyl-CoA - is added to the cycle, which will be dissipated as CO2 and H2O with the release of a substantial quantity of energy captured in the form of ATP, during the course of each turn of the cycle. (wikipedia.org)
  • The acetyl group (indicated in blue in the structural diagram on the right) of acetyl-CoA is linked to the sulfhydryl substituent of the β-mercaptoethylamine group. (wikipedia.org)
  • Acetyl-CoA then enters the citric acid cycle, where the acetyl group is oxidized to carbon dioxide and water, and the energy released captured in the form of 11 ATP and one GTP per acetyl group. (wikipedia.org)
  • Konrad Bloch and Feodor Lynen were awarded the 1964 Nobel Prize in Physiology and Medicine for their discoveries linking acetyl-CoA and fatty acid metabolism. (wikipedia.org)
  • At low glucose levels: CoA is acetylated using acetate by acetyl-CoA synthetase (ACS), also coupled with ATP hydrolysis. (wikipedia.org)
  • Isovaleryl-CoA undergoes dehydrogenation, carboxylation and hydration to form another CoA-derivative intermediate before it is cleaved into acetyl-CoA and acetoacetate. (wikipedia.org)
  • page needed] At high glucose levels, acetyl-CoA is produced through glycolysis. (wikipedia.org)
  • Pyruvate undergoes oxidative decarboxylation in which it loses its carboxyl group (as carbon dioxide) to form acetyl-CoA, giving off 33.5 kJ/mol of energy. (wikipedia.org)
  • The oxidative conversion of pyruvate into acetyl-CoA is referred to as the pyruvate dehydrogenase reaction. (wikipedia.org)
  • Other conversions between pyruvate and acetyl-CoA are possible. (wikipedia.org)
  • For example, pyruvate formate lyase disproportionates pyruvate into acetyl-CoA and formic acid. (wikipedia.org)
  • The cycle produces a new acyl-CoA with two fewer carbons and acetyl-CoA as a byproduct. (wikipedia.org)
  • In Cellular Respiration Citric acid cycle: Acetyl-CoA reacts with oxaloacetate to form citrate, which is then oxidized to CO2 in the cycle. (wikipedia.org)
  • Two acetyl-CoA molecules condense to form acetoacetyl-CoA, which gives rise to the formation of acetoacetate and β-hydroxybutyrate. (wikipedia.org)
  • Cleavage of two-carbon segments resulting in acetyl-CoA. (wikipedia.org)
  • This releases the first two carbon units, as acetyl CoA, and a fatty acyl CoA minus two carbons. (wikipedia.org)
  • The process continues until all of the carbons in the fatty acid are turned into acetyl CoA. (wikipedia.org)
  • The final cycle produces two separate acetyl CoAs, instead of one acyl CoA and one acetyl CoA. (wikipedia.org)
  • Chains with an odd-number of carbons are oxidized in the same manner as even-numbered chains, but the final products are propionyl-CoA and Acetyl CoA Propionyl-CoA is first carboxylated using a bicarbonate ion into D-stereoisomer of methylmalonyl-CoA, in a reaction that involves a biotin co-factor, ATP, and the enzyme propionyl-CoA carboxylase. (wikipedia.org)
  • In order for the acyl-CoA to enter the mitochondrion the carnitine shuttle is used: Acyl-CoA is transferred to the hydroxyl group of carnitine by carnitine palmitoyltransferase I, located on the cytosolic faces of the outer and inner mitochondrial membranes. (wikipedia.org)