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)

Metabolic adaptation of two pig muscles to cold rearing conditions. (1/1)

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3-Hydroxyacyl CoA Dehydrogenases (3-HADs) are a group of enzymes that play a crucial role in the beta-oxidation of fatty acids. These enzymes catalyze the third step of the beta-oxidation process, which involves the oxidation of 3-hydroxyacyl CoA to 3-ketoacyl CoA. This reaction is an essential part of the energy-generating process that occurs in the mitochondria of cells and allows for the breakdown of fatty acids into smaller molecules, which can then be used to produce ATP, the primary source of cellular energy.

There are several different isoforms of 3-HADs, each with specific substrate preferences and tissue distributions. The most well-known isoform is the mitochondrial 3-hydroxyacyl CoA dehydrogenase (M3HD), which is involved in the oxidation of medium and long-chain fatty acids. Other isoforms include the short-chain 3-hydroxyacyl CoA dehydrogenase (SCHAD) and the long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD), which are involved in the oxidation of shorter and longer chain fatty acids, respectively.

Deficiencies in 3-HADs can lead to serious metabolic disorders, such as 3-hydroxyacyl-CoA dehydrogenase deficiency (3-HAD deficiency), which is characterized by the accumulation of toxic levels of 3-hydroxyacyl CoAs in the body. Symptoms of this disorder can include hypoglycemia, muscle weakness, cardiomyopathy, and developmental delays. Early diagnosis and treatment of 3-HAD deficiency are essential to prevent serious complications and improve outcomes for affected individuals.

Acyl-CoA dehydrogenases are a group of enzymes that play a crucial role in the body's energy production process. They are responsible for catalyzing the oxidation of various fatty acids, which are broken down into smaller molecules called acyl-CoAs in the body.

More specifically, acyl-CoA dehydrogenases facilitate the removal of electrons from the acyl-CoA molecules, which are then transferred to coenzyme Q10 and eventually to the electron transport chain. This process generates energy in the form of ATP, which is used by cells throughout the body for various functions.

There are several different types of acyl-CoA dehydrogenases, each responsible for oxidizing a specific type of acyl-CoA molecule. These include:

* Very long-chain acyl-CoA dehydrogenase (VLCAD), which oxidizes acyl-CoAs with 12 to 20 carbon atoms
* Long-chain acyl-CoA dehydrogenase (LCAD), which oxidizes acyl-CoAs with 14 to 20 carbon atoms
* Medium-chain acyl-CoA dehydrogenase (MCAD), which oxidizes acyl-CoAs with 6 to 12 carbon atoms
* Short-chain acyl-CoA dehydrogenase (SCAD), which oxidizes acyl-CoAs with 4 to 8 carbon atoms
* Isovaleryl-CoA dehydrogenase, which oxidizes isovaleryl-CoA, a specific type of branched-chain acyl-CoA molecule

Deficiencies in these enzymes can lead to various metabolic disorders, such as medium-chain acyl-CoA dehydrogenase deficiency (MCADD) or long-chain acyl-CoA dehydrogenase deficiency (LCADD), which can cause symptoms such as hypoglycemia, muscle weakness, and developmental delays.

Acyl-CoA dehydrogenase is a group of enzymes that play a crucial role in the body's energy production process. Specifically, they are involved in the breakdown of fatty acids within the cells.

More technically, acyl-CoA dehydrogenases catalyze the removal of electrons from the thiol group of acyl-CoAs, forming a trans-double bond and generating FADH2. This reaction is the first step in each cycle of fatty acid beta-oxidation, which occurs in the mitochondria of cells.

There are several different types of acyl-CoA dehydrogenases, each specific to breaking down different lengths of fatty acids. For example, very long-chain acyl-CoA dehydrogenase (VLCAD) is responsible for breaking down longer chain fatty acids, while medium-chain acyl-CoA dehydrogenase (MCAD) breaks down medium-length chains.

Deficiencies in these enzymes can lead to various metabolic disorders, such as MCAD deficiency or LC-FAOD (long-chain fatty acid oxidation disorders), which can cause symptoms like vomiting, lethargy, and muscle weakness, especially during periods of fasting or illness.

Lipid A is the biologically active component of lipopolysaccharides (LPS), which are found in the outer membrane of Gram-negative bacteria. It is responsible for the endotoxic activity of LPS and plays a crucial role in the pathogenesis of gram-negative bacterial infections. Lipid A is a glycophosphatidylinositol (GPI) anchor, consisting of a glucosamine disaccharide backbone with multiple fatty acid chains and phosphate groups attached to it. It can induce the release of proinflammatory cytokines, fever, and other symptoms associated with sepsis when introduced into the bloodstream.

Acyltransferases are a group of enzymes that catalyze the transfer of an acyl group (a functional group consisting of a carbon atom double-bonded to an oxygen atom and single-bonded to a hydrogen atom) from one molecule to another. This transfer involves the formation of an ester bond between the acyl group donor and the acyl group acceptor.

Acyltransferases play important roles in various biological processes, including the biosynthesis of lipids, fatty acids, and other metabolites. They are also involved in the detoxification of xenobiotics (foreign substances) by catalyzing the addition of an acyl group to these compounds, making them more water-soluble and easier to excrete from the body.

Examples of acyltransferases include serine palmitoyltransferase, which is involved in the biosynthesis of sphingolipids, and cholesteryl ester transfer protein (CETP), which facilitates the transfer of cholesteryl esters between lipoproteins.

Acyltransferases are classified based on the type of acyl group they transfer and the nature of the acyl group donor and acceptor molecules. They can be further categorized into subclasses based on their sequence similarities, three-dimensional structures, and evolutionary relationships.

Fatty acids are carboxylic acids with a long aliphatic chain, which are important components of lipids and are widely distributed in living organisms. They can be classified based on the length of their carbon chain, saturation level (presence or absence of double bonds), and other structural features.

The two main types of fatty acids are:

1. Saturated fatty acids: These have no double bonds in their carbon chain and are typically solid at room temperature. Examples include palmitic acid (C16:0) and stearic acid (C18:0).
2. Unsaturated fatty acids: These contain one or more double bonds in their carbon chain and can be further classified into monounsaturated (one double bond) and polyunsaturated (two or more double bonds) fatty acids. Examples of unsaturated fatty acids include oleic acid (C18:1, monounsaturated), linoleic acid (C18:2, polyunsaturated), and alpha-linolenic acid (C18:3, polyunsaturated).

Fatty acids play crucial roles in various biological processes, such as energy storage, membrane structure, and cell signaling. Some essential fatty acids cannot be synthesized by the human body and must be obtained through dietary sources.

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 ...
... 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- ...
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; ...
... such that HIV-1 Vpr regulates mitochondrial respiration and enhances the activity of hydroxyacyl-CoA dehydrogenase (HADH) ... Hydroxyacyl-Coenzyme A dehydrogenase (HADH) is an enzyme which in humans is encoded by the HADH gene. The HADH gene is located ... Craig I, Tolley E, Bobrow M (1976). "A preliminary analysis of the segregation of human hydroxyacyl coenzyme A dehydrogenase in ... "Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB). Molven A, Matre ...
"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, ...
HSD17B4 "EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. 6 September 2017. ... v t e (Protein pages needing a picture, Genes on human chromosome 3, Human gene pages with Wikidata item, All stub articles, ...
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 ...
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 ...
"Disorders of mitochondrial fatty acyl-CoA beta-oxidation" (PDF). Journal of Inherited Metabolic Disease. 22 (4): 442-487. doi: ... 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 fetus, caused by long-chain 3- ... hydroxyacyl-coenzyme A dehydrogenase deficiency. This leads to decreased metabolism of long chain fatty acids by the feto- ...
108 (3): 457-465. doi:10.1172/jci200111294. PMC 209352. PMID 11489939. Dunne, Mark J.; Kane, Charlotte; Shepherd, Ruth M.; ... Butler, Patrick (3 August 2001). "Call to establish children's commissioner". Guardian News and Media Limited. The Guardian. ... Wardrop, Murray (3 February 2010). "'Britain is one of world's most unfriendly countries towards children'". Telegraph Media ... Retrieved 3 November 2018. The National Service Framework for Children, Young People and Maternity Services - Emerging findings ...
"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 ...
"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- ...
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. doi:10.1016/ ... Enoyl-CoA-(∆) isomerase (EC 5.3.3.8, also known as dodecenoyl-CoA-(∆) isomerase, 3,2-trans-enoyl-CoA isomerase, ∆3(cis),∆2( ...
... 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- ...
... 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 ...
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 ... The propionyl-CoA is later converted into succinyl-CoA through biotin-dependant propionyl-CoA carboxylase (PCC) and Vitamin B12 ... CoA dehydrogenase, yielding 1 NADH + H+ Cleavage by thiolase, yielding 1 acetyl-CoA and a fatty acid that has now been ...
... 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 ...
"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 ...
... glutaryl-CoA dehydrogenase (ETF) EC 1.3.8.7: medium-chain acyl-CoA dehydrogenase EC 1.3.8.8: long-chain acyl-CoA dehydrogenase ... butyryl-CoA dehydrogenase. EC 1.3.99.3: now EC 1.3.8.7, medium-chain acyl-CoA dehydrogenase, EC 1.3.8.8, long-chain acyl-CoA ... benzylsuccinyl-CoA dehydrogenase EC 1.3.8.4: isovaleryl-CoA dehydrogenase EC 1.3.8.5: 2-methyl-branched-chain-enoyl-CoA ... 4-dicarboxylate dehydrogenase EC 1.3.1.62: pimeloyl-CoA dehydrogenase EC 1.3.1.63: Now EC 1.21.1.2, 2,4-dichlorobenzoyl-CoA ...
"Entrez Gene: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional ... 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 ...
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 ...
... (EC 1.3.8.1, butyryl-CoA dehydrogenase, butanoyl-CoA dehydrogenase, butyryl dehydrogenase, ... Acyl-CoA dehydrogenase Medium-chain acyl-CoA dehydrogenase Butyryl-CoA (also known as butanoyl-CoA) Mahler HR (January 1954). " ... butyryl coenzyme A dehydrogenase, short-chain acyl CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, 3-hydroxyacyl ... Short-chain+acyl-CoA+dehydrogenase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology ( ...
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 ...
... 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. ...
"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- ...
... deficiency of subtypes of acyl CoA dehydrogenase (LCAD, SCAD, MCAD, VLCAD, 3-hydroxyacyl-coenzyme A dehydrogenase deficiency), ... Elevated concentrations of the enzyme lactate dehydrogenase (LDH) may be detected. Other markers of muscle damage, such as ... glucose-6-phosphate dehydrogenase deficiency, myoadenylate deaminase deficiency and muscular dystrophies Damage to skeletal ... thiolase deficiency Mitochondrial myopathies: deficiency of succinate dehydrogenase, cytochrome c oxidase and coenzyme Q10 ...
... acyl-coa 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- ... malate dehydrogenase MeSH D08.811.682.047.748 - malate dehydrogenase (nadp+) MeSH D08.811.682.047.892 - xanthine dehydrogenase ...
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 ...
... (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 U.S. National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology (Articles with ... Wallace KK, Bao ZY, Dai H, Digate R, Schuler G, Speedie MK, Reynolds KA (November 1995). "Purification of crotonyl-CoA ...
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 ...
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 ( ... Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency ( ...
... also known as 3-hydroxyacyl-CoA dehydrogenase type II (Type II HADH), Endoplasmic reticulum-associated amyloid beta-peptide ... 3-hydroxyacyl-CoA dehydrogenase type II. A. 261. Homo sapiens. Mutation(s): 0 Gene Names: HADH2, ERAB, XH98G2, SCHAD. EC: 1.1. ... we demonstrate that Abeta-binding alcohol dehydrogenase (ABAD) is a direct molecular link from Abeta to mitochondrial toxicity ... Abeta-bound human ABAD structure [also known as 3-hydroxyacyl-CoA dehydrogenase type II (Type II HADH), Endoplasmic reticulum- ...
... dehydrogenase (dismutase) involved in the degradation pathway for pyridoxine (vitamin B(6)). The homogenously purified ... in Mesorhizobium loti was identified as the gene encoding 5-formyl-3-hydroxy-2-methylpyridine 4-carboxylic acid (FHMPC) ... FHMPC dehydrogenase catalyses practically irreversible oxidation (k(cat) = 204 s(-1)) of FHMPC (K(m) = 48.2 microM) by NAD(+) ( ... FHMPC dehydrogenase belongs to the 3-hydroxyacyl-CoA dehydrogenase family with 31% identity with the human enzyme: it has ...
... and medium-chain acyl-CoA dehydrogenase (MCAD). (b) In addition to the testing requirements prescribed in subsection (a) of ... The fees to be charged pursuant to subdivision (3) of this subsection shall be set at a minimum of fifty-six dollars. The ... 3) on and after January 1, 2013, a screening test for] (C) critical congenital heart disease, and (2) any newborn infant who ... 3) of subsection (b) of this section to the Department of Public Health in a form and manner prescribed by the Commissioner of ...
Universal BiGG reaction HACD4m. 3-hydroxyacyl-CoA dehydrogenase (C10:0).
Among these genes are those encoding fatty acyl-CoA oxidase (AOx) and enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase (HD ... Orphan Nuclear Hormone Receptor RevErbα Modulates Expression from the Promoter of the Hydratase-dehydrogenase Gene by ...
TFP has three enzymatic activities - long-chain enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA dehydrogenase, and long-chain ... Long-chain hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency and trifunctional protein (TFP) deficiency are caused by impairment ... In individuals with LCHAD deficiency, there is isolated deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase, while ... Long-Chain Hydroxyacyl-CoA Dehydrogenase Deficiency / Trifunctional Protein Defi.... Long-Chain Hydroxyacyl-CoA Dehydrogenase ...
Sets of 4 enzymes (an acyl dehydrogenase, a hydratase, a hydroxyacyl dehydrogenase, and a lyase) specific for different chain ... Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) This deficiency is the most common defect in the beta-oxidation cycle. ... Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) This deficiency is similar to LCHADD but is commonly associated with ... multiple acyl-coA dehydrogenase deficiency); oxidation of several amino acids is also affected. ...
Short Chain Acyl-Coa Dehydrogenase Deficiency. Ketotic hypoglycemia, Elevated circulating acylcarnitine concentration, Feeding ... SPC3, prohormone convertase 1/3, Phpp-1, PC3, PC1, Nec1, Nec-1 ... Methylmalonic Aciduria Due To Methylmalonyl-Coa Mutase ...
IEMs that produce Reye-like syndromes include fatty-acid oxidation defects, particularly medium-chain acyl dehydrogenase (MCAD ... and long-chain acyl dehydrogenase deficiency (LCAD) inherited and acquired forms, urea-cycle defects, amino and organic ... 3, 4, 5] Reye syndrome typically occurs after a viral illness, particularly an upper respiratory tract infection, influenza, ... Approximately 3% of patients have neurologic sequelae if ammonia levels are below 45 µg/dL, whereas nearly 11% have sequelae if ...
Match: DECR2_DANRE (Peroxisomal 2,4-dienoyl-CoA reductase OS=Danio rerio GN=decr2 PE=2 SV=1). HSP 1 Score: 69.3218 bits (168), ... Match: XYLL_PSEPU (1,6-dihydroxycyclohexa-2,4-diene-1-carboxylate dehydrogenase OS=Pseudomonas putida GN=xylL PE=3 SV=1). HSP 1 ... Peroxisomal 2,4-dienoyl-CoA reductase OS=Danio rer... [more]. BDH2_BOVIN. 3.402e-11. 29.73. 3-hydroxybutyrate dehydrogenase ... 3-hydroxyacyl-CoA dehydrogenase type-2 OS=Rattus n... [more]. CBR4_RAT. 2.605e-11. 27.83. Carbonyl reductase family member 4 OS ...
6-oxocyclohex-1-ene-1-carbonyl-CoA hydrolase; 6-OCH-CoA hydrolase; 6-oxocyclohex-1-ene-1-carbonyl-CoA hydratase; EC 3.7.1.21 ( ... 1 candidates for oah: 6-oxocyclohex-1-ene-1-carbonyl-CoA hydratase. Score. Gene. Description. Similar to. Id.. Cov.. Bits. ... 6-carboxyhex-2-enoyl-CoA hydratase. 53%. 701.0. Confidence: high confidence medium confidence low confidence. transporter - ...
... as Deduced from Structures of Complexes with 3S-Hydroxy- Acyl-Coa. pubmed doi rcsb ... N-(1-d-carboxylethyl)-l-norvaline Dehydrogenase; domain 2. N-(1-d-carboxylethyl)-l-norvaline Dehydrogenase; domain 2. 3zw8A03. ... 2-enoyl-CoA Hydratase; Chain A, domain 1. 3zw8A01. 3H5AA 4FD3A 2OGUA 5JAZA 1O6ZA 2X4GA 1ENPA 3E82A 2QZZA 4XDZA 1ZJZA 2DC1A 5C90 ... 3-Layer(aba) Sandwich. Rossmann fold. NAD(P)-binding Rossmann-like Domain. 3zw8A02. ...
... lactate dehydrogenase(LDH), cyclic adenosine monophosphate(cAMP), and cyclic guanosine monophosphate(cGMP). The liver was ... 3. [Mechanism of Zhenwu Decoction in improving renal inflammatory injury in mice with DN of spleen-kidney Yang deficiency ... The biomarker 3-oxo-4,6-choladienoic acid may have potential diagnostic value in patients with adenomatous polyp of the colon ... The treatment was given once a day, 6 times a week for 3 weeks in both groups. Before and after treatment, the short form of ...
Medium chain acyl CoA dehydrogenase deficiency 9% * Amish 9% * Protein-Restricted Diet 9% ...
... dehydrogenase 0.0 C-terminus uagpf_v2_unigene7091 uagpf v2.0 399 • AutoFact: enoyl-CoA hydratase/3-hydroxyacyl-CoA ... AutoFact: enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase [Arabidopsis thaliana] sp,Q9ZPI5.1,MFP2_ARATH RecName: Full= ... AutoFact: enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase [Arabidopsis thaliana] sp,Q9ZPI5.1,MFP2_ARATH RecName: Full= ... AutoFact: enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase [Arabidopsis thaliana] sp,Q9ZPI5.1,MFP2_ARATH RecName: Full= ...
In the aging animals, training did not alter (P greater than 0.05) activities of CS, HADH, GPX, or lactate dehydrogenase in the ... In contrast, training had no influence (P greater than 0.05) on the activity of lactate dehydrogenase within the costal ... 3) old trained (n = 10), and 4) old untrained (n = 6). Both young and old endurance-trained animals performed the same training ... activities of 3-hydroxyacyl-CoA dehydrogenase (HADH), glutathione peroxidase (GPX), and citrate synthase (CS) in both the ...
3-alpha hydroxyacyl-CoA dehydrogenase deficiency From NCATS Genetic and Rare Diseases Information Center ...
Malonyl CoA-acyl carrier protein transacylase (NCBI ptt file). 166, 238. CAC2288. CAC2288. Acyl-protein synthetase, luxE (NCBI ... Protein of short-chain alcohol dehydrogenase family (NCBI ptt file). 48, 224. ... Biotin-(acetyl-CoA carboxylase) ligase (NCBI ptt file). 132, 224. CAC0329. spoVD. Sporulation specific penicillin-binding ... 3-Hydroxyacyl-CoA dehydrogenase (NCBI ptt file). 97, 166. CAC2010. CAC2010. Predicted Fe-S oxidoreductase (NCBI ptt file). 97, ...
Short/Branched Chain Acyl-CoA Dehydrogenase Deficiency Very Long-chain Acyl-CoA Dehydrogenase Deficiency ... 3. infections of the central nervous system, 4. toxicological diseases, 5. tumors, 6. chromosomal abnormalities, 7. with the ... hepatosplenomegaly). 3) Metabolic acidosis with an increase in the anion gap. 4) Multiple fractures. 5) Child mortality in the ...
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 ... encoded search term (Long-Chain Acyl CoA Dehydrogenase Deficiency) and Long-Chain Acyl CoA Dehydrogenase Deficiency What to ... Scalais E, Bottu J, Wanders RJ, Ferdinandusse S, Waterham HR, De Meirleir L. Familial very long chain acyl-CoA dehydrogenase ...
Medium-Chain Acyl-CoA Dehydrogenase Deficiency. Methylmalonic Acidemia (Cobalamin Disorders). Methylmalonic Acidemia ( ... Methymalonyl-CoA Mutase Deficiency). Primary Congenital Hypothyroidism. Propionic Acidemia. S, Beta-Thalassemia. S, C Disease. ... 3-Hydroxy-3-Methylglutaric Aciduria. 3-Methylcrotonyl-CoA Carboxylase Deficiency. Argininosuccinic Aciduria. Beta-Ketothiolase ... Long-Chain L-3 Hydroxyacyl-CoA Dehydrogenase Deficiency. Maple Syrup Urine Disease. ...
","putative dehydrogenase [Ensembl]. Enoyl-CoA hydratase/isomerase, 3-hydroxyacyl-CoA dehydrogenase [Interproscan].","protein_ ... ","Probable enoyl-CoA hydratase EchA1 (enoyl hydrase) (unsaturated acyl-CoA hydratase) (crotonase) [Ensembl]. Enoyl-CoA ... ","enoyl-CoA hydratase EchA2 (enoyl hydrase) (unsaturated acyl-CoA hydratase) (crotonase) [Ensembl]. Enoyl-CoA hydratase/ ... ","Probable enoyl-CoA hydratase EchA3 (enoyl hydrase) (unsaturated acyl-CoA hydratase) (crotonase) [Ensembl]. Enoyl-CoA ...
... 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 ...
Ortholog function: Enoyl-CoA hydratase (EC 4.2.1.17) / 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35) ...
Booked 3 times in the last 24 hours Last booked 1 hour ago. In the s they succeeded in building a new monastery on part of the ... Geplande rijlessen dienen 3 x 24 uur vooraf telefonisch of via Whats App niet via E-mail geannuleerd te worden bij de ... They are blocking our sexual force in the chakras of survival, the first 3 vortices of body energy so that the sexual force is ... Inna hates taking breaks, if shes not singing in English, she releases Spanish singles, and now shes gone back free arma 3 ...
  • 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)
  • Some patients who are deficient in all 3 enzymatic activities of the protein have been described, although most have an isolated LCHAD deficiency, which results in the inability to metabolize long-chain fatty acids. (medscape.com)
  • Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency (LCHAD) deficiency. (medscape.com)
  • Patients with LCHAD deficiency may develop a profound CNS deficiency of docosahexanoic acid ethyl ester (DHA), 22:6n-3. (medscape.com)
  • The etiology of the severe peripheral neuropathy of trifunctional protein deficiency may result from the unique metabolite, 3-keto-acyl-CoA, after conversion to a methylketone via spontaneous decarboxylation. (medscape.com)
  • Long-chain hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency and trifunctional protein (TFP) deficiency are caused by impairment of mitochondrial TFP. (nih.gov)
  • In individuals with LCHAD deficiency, there is isolated deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase, while deficiency of all three enzymes occurs in individuals with TFP deficiency. (nih.gov)
  • The diagnosis of LCHAD/TFP deficiency is established in a proband with elevation of long-chain 3-hydroxyacylcarnitine species in plasma and/or increased excretion of 3-hydroxy-dicarboxylic acids in urine in combination with identification of biallelic pathogenic variants in HADHA or HADHB by molecular genetic testing . (nih.gov)
  • Distinguishing LCHAD deficiency from TFP deficiency requires identification of isolated long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency on enzymatic assay in lymphocytes or skin fibroblasts. (nih.gov)
  • TFP deficiency is confirmed by the identification of deficiencies in all three TFP enzymatic activities (long-chain enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA dehydrogenase, and long-chain 3-ketoacyl-CoA thiolase) in lymphocytes or skin fibroblasts. (nih.gov)
  • Admit patients with long-chain 3-hydroxy acyl-coenzyme A dehydrogenase (LCHAD) deficiency for medical management of acute hypoketotic hypoglycemic encephalopathy. (medscape.com)
  • If diagnosis of long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficiency is suspected but workup facilities are inadequate and no metabolic specialists are available, transfer of patient to a tertiary care hospital for further workup and management may be necessary. (medscape.com)
  • Advise avoidance of exercise and dehydration with hot temperatures because rhabdomyolysis and myoglobinuria may occur with long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficiency. (medscape.com)
  • Screening for long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficiency should be performed in newborns from mothers with hepatic complications during pregnancy such as acute fatty liver of pregnancy or severe hemolytic anemia, elevated liver enzymes, low platelet count (HELLP) syndrome. (medscape.com)
  • Pregnancy: Pregnancy complications reported in long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficiency carriers (with a long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficient fetus), include HELLP syndrome and acute fatty liver of pregnancy. (medscape.com)
  • 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)
  • 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)
  • Compared with young untrained animals, the young trained group had significantly elevated (P less than 0.05) activities of 3-hydroxyacyl-CoA dehydrogenase (HADH), glutathione peroxidase (GPX), and citrate synthase (CS) in both the costal diaphragm and the plantaris muscle. (tamu.edu)
  • In the aging animals, training did not alter (P greater than 0.05) activities of CS, HADH, GPX, or lactate dehydrogenase in the costal diaphragm but significantly (P less than 0.05) increased CS, HADH, and GPX activities in the plantaris muscle. (tamu.edu)
  • Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase. (medlineplus.gov)
  • The Commissioner of Public Health shall publish a list of all the abnormal conditions for which the department screens newborns under the newborn screening program, which shall include screening for amino acid disorders, organic acid disorders and fatty acid oxidation disorders, including, but not limited to, long-chain 3-hydroxyacyl CoA dehydrogenase (L-CHAD) and medium-chain acyl-CoA dehydrogenase (MCAD). (ct.gov)
  • The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase (LCKT). (medscape.com)
  • Among these genes are those encoding fatty acyl-CoA oxidase (AOx) and enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase (HD), the first two enzymes of the peroxisomal beta-oxidation pathway. (mcmaster.ca)
  • TFP has three enzymatic activities - long-chain enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA dehydrogenase, and long-chain 3-ketoacyl-CoA thiolase. (nih.gov)
  • Sets of 4 enzymes (an acyl dehydrogenase, a hydratase, a hydroxyacyl dehydrogenase, and a lyase) specific for different chain lengths (very long chain, long chain, medium chain, and short chain) are required to catabolize fatty acids completely. (msdmanuals.com)
  • protein_coding" "AAC75401","fadJ","Escherichia coli","enoyl-CoA hydratase/epimerase and isomerase/3-hydroxyacyl-CoA dehydrogenase [Ensembl]. (ntu.edu.sg)
  • protein_coding" "AAC76849","fadB","Escherichia coli","fused 3-hydroxybutyryl-CoA epimerase/delta(3)-cis-delta(2)-trans-enoyl-CoA isomerase/enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase [Ensembl]. (ntu.edu.sg)
  • protein_coding" "CCP42950","echA1","Mycobacterium tuberculosis","Probable enoyl-CoA hydratase EchA1 (enoyl hydrase) (unsaturated acyl-CoA hydratase) (crotonase) [Ensembl]. (ntu.edu.sg)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that make up the trifunctional protein of the inner mitochondrial membrane. (medscape.com)
  • Here, we demonstrate that Abeta-binding alcohol dehydrogenase (ABAD) is a direct molecular link from Abeta to mitochondrial toxicity. (rcsb.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)
  • 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)
  • 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)
  • FHMPC dehydrogenase belongs to the 3-hydroxyacyl-CoA dehydrogenase family with 31% identity with the human enzyme: it has probable catalytic diad residues, i.e. (nih.gov)
  • protein_coding" "AAL22827","STM3983","Salmonella enterica","3-hydroxyacyl-coA dehydrogenase of 4-enzyme FadB protein [Ensembl]. (ntu.edu.sg)
  • According to the target regulation map in Fig. 6C,D, ERK2 Activator web essential enzyme genes in oxidation gene-G6O67_007081 (3-hydroxyacyl-CoA dehydrogenase, targeted by n_os_milR90) and ecological adapting-related gene gene-G6O67_007081 (tat pathway signal sequence, targeted by n_os_milR16) had been upregulated. (ack1inhibitor.com)
  • Acetyl CoA is generated from fatty acids through repeated beta-oxidation cycles. (msdmanuals.com)
  • the oxidation of L-3-hydroxyacyl CoA by NAD+. (wikipedia.org)
  • Thus, the clinical features may result from either toxicity due to long-chain acyl-CoA esters that cause cardiomyopathy and cardiac arrhythmias or from a block in long-chain fatty acid oxidation that leads to an inability to synthesize ketone bodies and/or adenosine triphosphate from long-chain fatty acids. (medscape.com)
  • FHMPC dehydrogenase catalyses practically irreversible oxidation (k(cat) = 204 s(-1)) of FHMPC (K(m) = 48.2 microM) by NAD(+) (K(m) = 34.3 microM) to 3-hydroxy-2-methyl-pyridine 4, 5-dicarboxylic acid (HMPDC), and practically irreversible reduction (k(cat) = 217 s(-1)) of FHMPC (K(m) = 24.9 microM) by NADH (K(m) = 12.4 microM) to 4-pyridoxic acid. (nih.gov)
  • The E149Q one was stable in contrast to the corresponding human 3-hydroxyacyl-CoA dehydrogenase mutant, and showed unique pH optima depending on the co-substrates used for the reaction. (nih.gov)
  • 3) Metabolic acidosis with an increase in the anion gap. (who.int)
  • Clinical manifestations typically begin after 2 to 3 months of age and usually follow fasting (as little as 12 hours). (msdmanuals.com)
  • In contrast, training had no influence (P greater than 0.05) on the activity of lactate dehydrogenase within the costal diaphragm in young animals. (tamu.edu)
  • 2001;50(No. RR-3):[inclusive page numbers]. (cdc.gov)
  • [ 3 , 4 ] These episodes typically appear for the first time after a fast, which usually occurs in the context of intercurrent illness with vomiting. (medscape.com)
  • d) The administrative officer or other person in charge of each institution caring for newborn infants shall report any case of cytomegalovirus that is confirmed as a result of a screening test administered pursuant to subdivision (3) of subsection (b) of this section to the Department of Public Health in a form and manner prescribed by the Commissioner of Public Health. (ct.gov)
  • Less than 0.1% of children who took aspirin developed Reye syndrome, but more than 80% of patients diagnosed with Reye syndrome had taken aspirin in the past 3 weeks. (medscape.com)
  • 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)
  • Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myopathy and cardiomyopathy in early childhood. (medscape.com)
  • A five-year-old patient, with a diagnosis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, was referred for an ophthalmological examination. (nih.gov)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that make up the trifunctional protein of the inner mitochondrial membrane. (medscape.com)
  • TFP has three enzymatic activities - long-chain enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA dehydrogenase, and long-chain 3-ketoacyl-CoA thiolase. (nih.gov)
  • In individuals with LCHAD deficiency, there is isolated deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase, while deficiency of all three enzymes occurs in individuals with TFP deficiency. (nih.gov)
  • Distinguishing LCHAD deficiency from TFP deficiency requires identification of isolated long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency on enzymatic assay in lymphocytes or skin fibroblasts. (nih.gov)
  • TFP deficiency is confirmed by the identification of deficiencies in all three TFP enzymatic activities (long-chain enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA dehydrogenase, and long-chain 3-ketoacyl-CoA thiolase) in lymphocytes or skin fibroblasts. (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)
  • 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)
  • 3-OH-dodecanoic FA demonstrated a modest, fivefold increase in LCHAD-deficient cells. (sigmaaldrich.com)
  • Measurement of 3-OHFA excretion from LCHAD- or MTFP-deficient cell lines can be used as a diagnostic tool. (sigmaaldrich.com)
  • 4. Peroxisome proliferator-activated receptor alpha-responsive genes induced in the newborn but not prenatal liver of peroxisomal fatty acyl-CoA oxidase null mice. (nih.gov)
  • 6. Peroxisomal and mitochondrial fatty acid beta-oxidation in mice nullizygous for both peroxisome proliferator-activated receptor alpha and peroxisomal fatty acyl-CoA oxidase. (nih.gov)
  • 12. Coordinate induction of hepatic fatty acyl-CoA oxidase and P4504A1 in rat after activation of the peroxisome proliferator-activated receptor (PPAR) by sulphur-substituted fatty acid analogues. (nih.gov)
  • HSD17B10, hydroxysteroid (17-beta) dehydrogenase 10, is a member of the short-chain dehydrogenase/reductase superfamily. (nih.gov)
  • The multisystemic features of HSD10MD most likely result from the adverse effect of HSD17B10 mutations on mitochondrial function, rather than from the effects on the dehydrogenase activity (see PATHOGENESIS). (nih.gov)
  • The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase (LCKT). (medscape.com)
  • In OA-loaded HepaRG cells, FOH increased fatty acid oxidation, which was accompanied by up-regulation of PPARα target genes involved in mitochondrial fatty acid oxidation, including hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase and acetyl-coenzyme A acyltransferase 2. (nih.gov)
  • 3. Regulation of fatty acid transport protein and mitochondrial and peroxisomal beta-oxidation gene expression by fatty acids in developing rats. (nih.gov)
  • Catalysis of the reaction: a (3R)-hydroxyacyl-[acyl-carrier-protein] = a (2E)-enoyl-[acyl-carrier-protein] + H2O. (virtualflybrain.org)
  • This gene is responsible for the production of an enzyme called methylmalonyl-CoA mutase, which plays a crucial role in breaking down certain proteins and fats in the body. (austrahealth.com.au)
  • Role of 3-hydroxy fatty acid-induced hepatic lipotoxicity in acute fatty liver of pregnancy. (medscape.com)
  • Thus, the clinical features may result from either toxicity due to long-chain acyl-CoA esters that cause cardiomyopathy and cardiac arrhythmias or from a block in long-chain fatty acid oxidation that leads to an inability to synthesize ketone bodies and/or adenosine triphosphate from long-chain fatty acids. (medscape.com)
  • An NAD-dependent 3-hydroxyacyl CoA dehydrogenase that has broad specificity with regards to the acyl chain length of the substrate. (nih.gov)
  • An NAD -dependent 3-hydroxyacyl CoA dehydrogenase that has specificity for acyl chains containing 8 and 10 carbons. (bvsalud.org)
  • Methylmalonic acidemia (MMA) is a genetic disorder related to the methylmalonyl-CoA mutase (MMAB) gene. (austrahealth.com.au)
  • Through early identification, newborn screening provides an opportunity for treatment and significant reductions in morbidity and mortality ( 2 , 3 ). (cdc.gov)
  • Newborn screening can help prevent death or disability, if treatment follows (1, 3 ). (cdc.gov)
  • All infants aged 0-3 months who have done newborn genetic screening at Dubai Health Authority facilities between January and December 2018 were included. (karger.com)
  • Several animal species especially domestic swine, wild boar and wild deer are reservoirs of genotype HEV-3 and HEV-4 in these countries. (medscape.com)
  • 13. Differential induction of genes in liver and brown adipose tissue regulated by peroxisome proliferator-activated receptor-alpha during fasting and cold exposure in acyl-CoA dehydrogenase-deficient mice. (nih.gov)
  • Cecatto C, Wajner A, Vargas CR, Wajner SM, Amaral AU, Wajner M. High vulnerability of the heart and liver to 3‐hydroxypalmitic acid-induced disruption of mitochondrial functions in intact cell systems. (medscape.com)
  • The cover of this edition of Clinical Science features representative images of Periodic acid-Schiff (PAS) stained sections of the cortex in sham and 5/6 nephrectomy (Nx) rats following 2 weeks of treatment with either 0.1M NaHCO 3 , equimolar NaCl, or tap water (H 2 O). Panels show high power image of representative glomeruli. (silverchair.com)
  • Effects of unbound mycophenolic acid on inosine monophosphate dehydrogenase inhibition in pediatric kidney transplant patients. (ucdenver.edu)
  • The lack of these enzymes leaves the body short of energy and allows breakdown products, such as acyl-CoA, to accumulate. (msdmanuals.com)
  • The concentrations of all 3-OHFAs were similar whether or not the medium samples were hydrolyzed to release conjugated species such as acylcarnitines, suggesting that 3-OHFAs accumulate in the media as free FAs. (sigmaaldrich.com)
  • Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase. (medlineplus.gov)
  • Bis(sulfonamide) isosters of mycophenolic adenine dinucleotide analogues: inhibition of inosine monophosphate dehydrogenase. (ucdenver.edu)
  • Inhibition of inosine monophosphate dehydrogenase. (ucdenver.edu)
  • Although the diagnosis can be aided by the observation of increased urinary levels of metabolites of isoleucine breakdown (2-methyl-3 hydroxybutyrate and tiglylglycine), there is not a correlation between these laboratory features and the phenotype. (nih.gov)
  • 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)
  • For example, respiration-deficient myoblasts devoid of mitochondrial DNA (rho° cells) by EtBr, an inhibitor of mtDNA replication and transcription, fail to differentiate into myotubes [ 1 - 3 ]. (hindawi.com)
  • Accumulation of free 3-hydroxy fatty acids in the culture media of fibroblasts from patients deficient in long-chain l-3-hydroxyacyl-CoA dehydrogenase: a useful diagnostic aid. (sigmaaldrich.com)
  • Specifically, it is now generally accepted that double bonds extending from even-numbered carbon atoms are reductively removed after they have moved to the 4 position by chain shortening of the fatty acyl-CoA and after they have become part of a 2-trans,4-cis-dienoyl-CoA system owing to the introduction of a 2-trans double bond catalysed by medium-chain acyl-CoA dehydrogenase (EC 1.3.99.3) [2]. (pdffox.com)
  • Those fatty acids having conjugated double bonds at evennumbered carbon atoms might yield 2,4,6-trienoyl-CoA thioesters as a result of chain shortening and the introduction of a double bond at the 2 position. (pdffox.com)
  • Ex vivo selection and expansion of cells based on expression of a mutated inosine monophosphate dehydrogenase 2 after HIV vector transduction: effects on lymphocytes, monocytes, and CD34+ stem cells. (ucdenver.edu)
  • Also exhibits 20-beta-OH and 21-OH dehydrogenase activities with C21 steroids. (joplink.net)