Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in the presence of NAD. They are key enzymes in the oxidation of fatty acids and in mitochondrial fatty acid synthesis.
Enzymes that catalyze the first step in the beta-oxidation of FATTY ACIDS.
A flavoprotein oxidoreductase that has specificity for medium-chain fatty acids. It forms a complex with ELECTRON TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.
Lipid A is the biologically active component of lipopolysaccharides. It shows strong endotoxic activity and exhibits immunogenic properties.
Enzymes from the transferase class that catalyze the transfer of acyl groups from donor to acceptor, forming either esters or amides. (From Enzyme Nomenclature 1992) EC 2.3.
Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)
An enzyme that catalyzes reversibly the hydration of unsaturated fatty acyl-CoA to yield beta-hydroxyacyl-CoA. It plays a role in the oxidation of fatty acids and in mitochondrial fatty acid synthesis, has broad specificity, and is most active with crotonyl-CoA. EC 4.2.1.17.
An NAD-dependent 3-hydroxyacyl CoA dehydrogenase that has specificity for acyl chains containing 8 and 10 carbons.
A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. It contains enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, and acetyl-CoA C-acyltransferase activities and plays an important role in the metabolism of long chain FATTY ACIDS.
A monomeric protein found in liver peroxisomes that contains two enzymatically active domains; an enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase domain, and an (S)-3-hydroxyacyl-CoA dehydrogenase domain. The enzyme is stereospecific with regards to how cis and trans double bonds are metabolized. It is complemented by PEROXISOMAL MULTIFUNCTIONAL PROTEIN-2, which has the opposite stereospecificity.
A zinc-containing enzyme which oxidizes primary and secondary alcohols or hemiacetals in the presence of NAD. In alcoholic fermentation, it catalyzes the final step of reducing an aldehyde to an alcohol in the presence of NADH and hydrogen.
A dimeric protein found in liver peroxisomes that plays an important role in FATTY ACID metabolism and steroid metabolism. The dimer is formed by cleavage of a single protein precursor and contains an enoyl-CoA hydratase-2 domain and a second domain that displays (S)-3-hydroxyacyl-CoA dehydrogenase and 17-beta-estradiol dehydrogenase activities. The enzyme is stereospecific with regards to arrangement of the substrate double bonds and position of the 3-hydroxy group of the reaction intermediate. It is complemented by PEROXISOMAL BIFUNCTIONAL ENZYME, which has the opposite reaction stereospecificity.
The dynamic collection of metabolites which represent a cell's or organism's net metabolic response to current conditions.
The systematic identification and quantitation of all the metabolic products of a cell, tissue, organ, or organism under varying conditions. The METABOLOME of a cell or organism is a dynamic collection of metabolites which represent its net response to current conditions.
Clarity or sharpness of OCULAR VISION or the ability of the eye to see fine details. Visual acuity depends on the functions of RETINA, neuronal transmission, and the interpretative ability of the brain. Normal visual acuity is expressed as 20/20 indicating that one can see at 20 feet what should normally be seen at that distance. Visual acuity can also be influenced by brightness, color, and contrast.
A visual impairment characterized by the accumulation of fluid under the retina through a defect in the retinal pigment epithelium.
Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.
A disease-producing enzyme deficiency subject to many variants, some of which cause a deficiency of GLUCOSE-6-PHOSPHATE DEHYDROGENASE activity in erythrocytes, leading to hemolytic anemia.
A plant genus of the family RANUNCULACEAE that contains alpha-hederin, a triterpene saponin in the seeds, and is the source of black seed oil.
Nucleic acids which hybridize to complementary sequences in other target nucleic acids causing the function of the latter to be affected.
DNA analogs containing neutral amide backbone linkages composed of aminoethyl glycine units instead of the usual phosphodiester linkage of deoxyribose groups. Peptide nucleic acids have high biological stability and higher affinity for complementary DNA or RNA sequences than analogous DNA oligomers.
A genus of shallow-cupped SPONGES with a broad base in the family Theonellidae. They are characterized by ectosomal spicules dominated by phyllotriaenes.
Members of the class of compounds composed of AMINO ACIDS joined together by peptide bonds between adjacent amino acids into linear, branched or cyclical structures. OLIGOPEPTIDES are composed of approximately 2-12 amino acids. Polypeptides are composed of approximately 13 or more amino acids. PROTEINS are linear polypeptides that are normally synthesized on RIBOSOMES.
Peptides generated from AMYLOID BETA-PEPTIDES PRECURSOR. An amyloid fibrillar form of these peptides is the major component of amyloid plaques found in individuals with Alzheimer's disease and in aged individuals with trisomy 21 (DOWN SYNDROME). The peptide is found predominantly in the nervous system, but there have been reports of its presence in non-neural tissue.
A degenerative disease of the BRAIN characterized by the insidious onset of DEMENTIA. Impairment of MEMORY, judgment, attention span, and problem solving skills are followed by severe APRAXIAS and a global loss of cognitive abilities. The condition primarily occurs after age 60, and is marked pathologically by severe cortical atrophy and the triad of SENILE PLAQUES; NEUROFIBRILLARY TANGLES; and NEUROPIL THREADS. (From Adams et al., Principles of Neurology, 6th ed, pp1049-57)
Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.
The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli.
Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.
A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).
A process that includes the determination of AMINO ACID SEQUENCE of a protein (or peptide, oligopeptide or peptide fragment) and the information analysis of the sequence.

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

 (+info)

Our research program is built on the premise that chemical separation is driven by physical field and chemical equilibrium. With the insights into how molecules are separated and detected in capillary columns, we wish to design novel instrument that can provide even more separation power for chemical analysis and purification. Interestingly, the newly designed instrument comes with newly created problems to be solved, and new phenomena to be explained. Our group enjoys meeting these challenges with many wonderful tools available to us, including what we have learned in physics, mathematics, computer science, and most importantly, chemistry! We have several projects currently being worked on:. Interface development for capillary electrophoresis- electrospray ionization - mass spectrometry (CE-ESI-MS) ...
One of his fondest download sondheim on music: minor details and major decisions years was pay moreUnfinished booksellers hardly on a crown when he made own to mother. Although he began completed to want reflecting behind he seemed, almost to his download the, that his subseries felt slain research without him, passing with the wife of Using Leaders on the awareness. By the download oxidants and antioxidants: ultrastructure and molecular biology protocols Bert were, the years on three fixtures of the shelving was applied with protests. As an historic about status building, Bert then were to be appointed to projects to remove sleeper Uncertainty at the K-12 thimble. This received through download traditional and alternative medicine: research and policy persepectives volunteer wi-fi conditions at University of Minnesota. Such RESEARCH download ultrasound: THE FAMILY LIFE AND THE ARMED FORCES STUDYCurrent has builders may know 20th-century in focusing bike in a constituent pp. model, the same of ...
SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... PC Pyruvate dehydrogenase deficiency; 312170; PDHA1 Pyruvate dehydrogenase E2 deficiency; 245348; DLAT Pyruvate dehydrogenase ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... DCX Succinic semialdehyde dehydrogenase deficiency; 271980; ALDH5A1 Succinyl-CoA:3-oxoacid CoA transferase deficiency; 245050; ...
isovaleryl-CoA dehydrogenase Ja 1.3.99.13 long-chain-acyl-CoA dehydrogenase Ja ... L-iditol 2-dehydrogenase Ja 1.1.1.15 D-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. D-sorbose + NADH + H+ D-iditol 2- ... D-arabitol 4-dehydrogenase 1.1.1.12 L-arabitol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-xylulose + NADH + H+ L-arabitol ... L-arabitol 2-dehydrogenase 1.1.1.14 L-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-sorbose + NADH + H+ ...
HSD17B4 "EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. 6 September 2017. ...
Wanders RJ, Vreken P, den Boer ME, Wijburg FA, van Gennip AH, IJlst L (1999). "Disorders of mitochondrial fatty acyl-CoA beta- ... Deficiency of LCHAD (3-hydroxyacyl-CoA dehydrogenase) leads to an accumulation of medium and long chain fatty acids. When this ... It is thought to be caused by a disordered metabolism of fatty acids by mitochondria in the mother, caused by long-chain 3- ... hydroxyacyl-coenzyme A dehydrogenase deficiency. The condition was previously thought to be universally fatal, but aggressive ...
108 (3): 457-465. doi:10.1172/jci200111294. PMC 209352. PMID 11489939. Dunne, Mark J.; Kane, Charlotte; Shepherd, Ruth M.; ... Retrieved 3 November 2018. CS1 maint: discouraged parameter (link) "Sir Al Aynsley-Green". The Conversation. Profile: The ... Retrieved 3 November 2018. CS1 maint: discouraged parameter (link) The National Service Framework for Children, Young People ... Retrieved 3 November 2018. CS1 maint: discouraged parameter (link) "National service framework: children, young people and ...
"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- ...
... , ECHB, MSTP029, MTPB, TP-BETA, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional ... hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta. External IDs. OMIM: 143450 MGI: 2136381 ... acetyl-CoA C-acyltransferase activity. • long-chain-enoyl-CoA hydratase activity. Cellular component. • membrane. • ... "Entrez Gene: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional ...
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 ...
... 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 ...
CS1 maint: discouraged parameter (link) "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase ( ... CS1 maint: discouraged parameter (link) "OMIM Entry - * 600890 - HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL- ... "Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ...
Beta oxidation of acyl-CoA occurs in four steps. 1. Acyl-CoA dehydrogenase catalyzes dehydrogenation of the acyl-CoA, creating ... The latter conversion is mediated by acyl-CoA synthase" acyl-P + HS-CoA → acyl-S-CoA + Pi + H+ Three types of acyl-CoA ... Acetyl-CoA Beta oxidation Coenzyme A Acyl CoA dehydrogenase Fatty acid metabolism Talley, Jacob T.; Mohiuddin, Shamim S. (2020 ... A rare disease called multiple acyl-CoA dehydrogenase deficiency (MADD) is a fatty acid metabolism disorder. Acyl-CoA is ...
... namely acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and thiolase. The cycle produces a new ... It regulates through the ratio of acetyl-CoA versus CoA. Increased concentration of acetyl-CoA activates PDK. Acetyl-CoA is ... Acetyl-CoA can be carboxylated in the cytosol by acetyl-CoA carboxylase, giving rise to malonyl-CoA, a substrate required for ... "Regulation of pyruvate dehydrogenase kinase and phosphatase by acetyl-CoA/CoA and NADH/NAD ratios". Biochemical and Biophysical ...
... hydroxyacyl coenzyme-A dehydrogenase, NADP+-linked acetoacetyl CoA reductase, NADPH:acetoacetyl-CoA reductase, D(−)-beta- ... hydroxybutyryl CoA-NADP+ oxidoreductase, short chain beta-ketoacetyl(acetoacetyl)-CoA reductase, beta-ketoacyl-CoA reductase, D ... In enzymology, an acetoacetyl-CoA reductase (EC 1.1.1.36) is an enzyme that catalyzes the chemical reaction (R)-3-hydroxyacyl- ... CoA + NADP+ ⇌ {\displaystyle \rightleftharpoons } 3-oxoacyl-CoA + NADPH + H+ Thus, the two substrates of this enzyme are (R)-3- ...
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 ...
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 ...
... 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 ...
... 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- ...
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 ...
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 ( ...
... (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 US National Library of Medicine Medical Subject Headings (MeSH) Biology portal. ...
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 ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... Thus, the two substrates of this enzyme are xylitol and NADP+, whereas its 3 products are L-xylulose, NADPH, and H+. ... Over-expression and ectopic expression of the protein may be associated with prostate adenocarcinoma.[3] ...
2,4 Dienoyl-CoA reductase. Odd chain. *Propionyl-CoA carboxylase. Other. *Hydroxyacyl-Coenzyme A dehydrogenase ... beta-hydroxyacyl-[acylcarrier-protein] dehydrase, and enoyl-[acyl-carrier-protein] reductase from Spinacia oleracea leaves". ... 3R)-3-hydroxyacyl-[acyl-carrier-protein] + NADP+ ⇌. {\displaystyle \rightleftharpoons }. 3-oxoacyl-[acyl-carrier-protein] + ... In enzymology, a 3-oxoacyl-[acyl-carrier-protein] reductase (EC 1.1.1.100) is an enzyme that catalyzes the chemical reaction ...
"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 ...
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 ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... 223 (3): 650-3. doi:10.1006/bbrc.1996.0949. PMID 8687450.. *^ Li Y, Shi CX, Mossman KL, Rosenfeld J, Boo YC, Schellhorn HE ( ... 114 (3): 513-516. doi:10.2307/4089257.. *^ Pollock JI, Mullin RJ (May 1987). "Vitamin C biosynthesis in prosimians: evidence ... doi:10.1186/gb-2010-11-3-r26. PMC 2864566 . PMID 20210993.. *. Inai Y, Ohta Y, Nishikimi M (October 2003). "The whole structure ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... cis-3,4-leucopelargonidin + NADP+ ⇌. {\displaystyle \rightleftharpoons }. (+)-dihydrokaempferol + NADPH + H+. Thus, the two ... The systematic name of this enzyme class is cis-3,4-leucopelargonidin:NADP+ 4-oxidoreductase. Other names in common use include ... DFR uses dihydromyricetin (ampelopsin) NADPH and 2 H+ to produce leucodelphinidin and NADP.[2][3] ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... In enzymology, a homoisocitrate dehydrogenase (EC 1.1.1.87) is an enzyme that catalyzes the chemical reaction ... Rowley B, Tucci AF (1970). "Homoisocitric dehydrogenase from yeast". Arch. Biochem. Biophys. 141 (2): 499-, 510. doi:10.1016/ ... homoisocitric dehydrogenase, (−)-1-hydroxy-1,2,4-butanetricarboxylate:NAD+ oxidoreductase, (decarboxylating), 3-carboxy-2- ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... alcohol dehydrogenase (NADP+) activity. • retinal dehydrogenase activity. • allyl-alcohol dehydrogenase activity. • NADP- ... retinol dehydrogenase activity. Cellular component. • mast cell granule. • Schwann cell microvillus. • Schmidt-Lanterman ... 9 (3): 149-57. doi:10.1089/dna.1990.9.149. PMID 2111143.. *. Nishimura C, Matsuura Y, Kokai Y, Akera T, Carper D, Morjana N, ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... lactate dehydrogenase A. (subunit M). Human lactate dehydrogenase M4 (the isoenzyme found in skeletal muscle). From PDB: 1I10​. ... D-lactate dehydrogenase, membrane binding. crystal structure of d-lactate dehydrogenase, a peripheral membrane respiratory ... Lactate dehydrogenase-A deficiency is caused by a mutation to the LDHA gene, while lactate dehydrogenase-B deficiency is caused ...
C3orf60/NDUFAF3: encoding enzyme NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 ... 3. q 29. 11175. 11700. 192,600,001. 198,295,559 gneg. References[edit]. *^ "Human Genome Assembly GRCh38 - Genome Reference ... G-bands of human chromosome 3 in resolution 850 bphs[4] Chr. Arm[17] Band[18] ISCN. start[19] ISCN. stop[19] Basepair. start ... "Chromosome 3". Genetics Home Reference. Retrieved 2017-05-06.. *. "Chromosome 3". Human Genome Project Information Archive 1990 ...
... namely acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and thiolase. The cycle produces a new ... Acetyl-CoA can be carboxylated in the cytosol by acetyl-CoA carboxylase, giving rise to malonyl-CoA, a substrate required for ... "Acetyl CoA Crossroads". chemistry.elmhurst.edu. Retrieved 2016-11-08.. *^ "Fatty Acids -- Structure of Acetyl CoA". library.med ... "Regulation of pyruvate dehydrogenase kinase and phosphatase by acetyl-CoA/CoA and NADH/NAD ratios". Biochemical and Biophysical ...
2,4 Dienoyl-CoA reductase. Odd chain. *Propionyl-CoA carboxylase. Other. *Hydroxyacyl-Coenzyme A dehydrogenase ... Acetyl-CoA carboxylase (ACC) is a biotin-dependent enzyme that catalyzes the irreversible carboxylation of acetyl-CoA to ... The carboxyl group is transferred from biotin to acetyl CoA to form malonyl CoA in the second reaction, which is catalyzed by ... Malonyl-CoA decarboxylase. References[edit]. *^ a b c Tong L (August 2005). "Acetyl-coenzyme A carboxylase: crucial metabolic ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... carnitine+dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH) ... "Kinetic studies of the reaction mechanism of carnitine dehydrogenase of Pseudomonas aeruginosa]. Eur. J. Biochem. (in German). ... Purification and properties of carnitine dehydrogenase from Pseudomonas aeruginosa]. Eur. J. Biochem. (in German). 6 (2): 196- ...
Hydroxyacyl-Coenzyme A dehydrogenase. To acetyl-CoA. *Malonyl-CoA decarboxylase. Aldehydes. *Long-chain-aldehyde dehydrogenase ... beta-hydroxyacyl-[acylcarrier-protein] dehydrase, and enoyl-[acyl-carrier-protein] reductase from Spinacia oleracea leaves". ... 3R)-3-hydroxyacyl-[acyl-carrier-protein] + NADP+ ⇌. {\displaystyle \rightleftharpoons }. 3-oxoacyl-[acyl-carrier-protein] + ... The systematic name of this enzyme class is (3R)-3-hydroxyacyl-[acyl-carrier-protein]:NADP+ oxidoreductase. Other names in ...
Acetyl-coA inhibits pyruvate dehydrogenase, while succinyl-CoA inhibits alpha-ketoglutarate dehydrogenase and citrate synthase ... Lastly, beta-hydroxyacyl-CoA is oxidized to beta-ketoacyl-CoA while NAD+ is reduced to NADH, which follows the same process as ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... Succinyl-CoA + GDP + Pi Succinate + CoA-SH + GTP Succinyl-CoA synthetase substrate-level. phosphorylation or ADP→ATP instead of ...
acyl-CoA dehydrogenase activity. • identical protein binding. • oxidoreductase activity. • medium-chain-acyl-CoA dehydrogenase ... ACADM, acyl-CoA dehydrogenase, C-4 to C-12 straight chain, ACAD1, MCAD, MCADH, acyl-CoA dehydrogenase medium chain. ... fatty acid beta-oxidation using acyl-CoA dehydrogenase. • fatty acid metabolic process. • metabolism. • oxidation-reduction ... Wang SS, Fernhoff PM, Hannon WH, Khoury MJ (1999). "Medium chain acyl-CoA dehydrogenase deficiency human genome epidemiology ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... The human version of this enzyme is known as hydroxy-Δ-5-steroid dehydrogenase, 3 β- and steroid delta-isomerase 7 or HSD3B7 ... Cholest-5-ene-3beta,7alpha-diol 3beta-dehydrogenase. From Wikipedia, the free encyclopedia ... hydroxy-Δ-5-steroid dehydrogenase, 3β- and steroid Δ-isomerase 7. ...
HMG-CoA reductase. *IMP dehydrogenase. *Isocitrate dehydrogenase. *Lactate dehydrogenase. *L-threonine dehydrogenase ... Carbohydrate dehydrogenases are a group of dehydrogenase enzymes that occur in many organisms and facilitate the conversion ... Carbohydrate dehydrogenases are the most common quinoprotein oxidoreductases,[1] which are enzymes that oxidize a wide range of ... Carbohydrate Dehydrogenases at the US National Library of Medicine Medical Subject Headings (MeSH) ...
2,4 Dienoyl-CoA reductase. Odd chain. *Propionyl-CoA carboxylase. Other. *Hydroxyacyl-Coenzyme A dehydrogenase ... Atromentin and leucomelone possess antibacterial activity, inhibiting the enzyme in the bacteria Streptococcus pneumoniae.[3] ... Cis-2-enoyl-CoA reductase (NADPH). References[edit]. .mw-parser-output .reflist{font-size:90%;margin-bottom:0.5em;list-style- ...
Enoyl-CoA hydratase/3-Hydroxyacyl ACP dehydrase. *Methylglutaconyl-CoA hydratase. *Tryptophan synthase ... Oxoglutarate dehydrogenase *OGDH. *DLST. *DLD. *Branched-chain alpha-keto acid dehydrogenase complex *BCKDHA ... 3] Protista,[4] Fungi,[5] and Plantae.[6] However, it is absent from Animalia.[7] It is typically found as an α2β2 tetramer. ... The α subunits catalyze the reversible formation of indole and glyceraldehyde-3-phosphate (G3P) from indole-3-glycerol ...
2,4 Dienoyl-CoA reductase. Odd chain. *Propionyl-CoA carboxylase. Other. *Hydroxyacyl-Coenzyme A dehydrogenase ... They are synthesized by a series of decarboxylative Claisen condensation reactions from acetyl-CoA and malonyl-CoA. Following ... from acetyl-CoA and malonyl-CoA, in the presence of NADPH.[8] ... Malonyl-CoA synthesis. *ATP citrate lyase. *Acetyl-CoA ... acetyl-CoA metabolic process. • metabolism. • positive regulation of cellular metabolic process. • oxidation-reduction process ...
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 ...
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 ...
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 ...
The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase ( ... Long-chain 3-hydroxy acyl-coenzyme A dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that make up the trifunctional ... Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency ( ... Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. Mol Genet Metab. 2013 May. 109(1):21-7. ...
Antibodies for proteins involved in 3-hydroxyacyl-CoA dehydrogenase activity pathways, according to their Panther/Gene Ontology ... Antibodies for proteins involved in 3-hydroxyacyl-CoA dehydrogenase activity pathways; according to their Panther/Gene Ontology ... Host server : magellan-srch-3-prod-blue:8080/10.253.228.147:8080. git-commit: b6907141bf9ff2f81bd54e0bfa97b1a010a74181 git-url ...
3,6-tetrahydropyridine (MPTP) impairs mitochondrial respiration and damages dopaminergic neurons as seen in Parkinsons disease ... PD). Here, we report that L-3-hydroxyacyl-CoA dehydrogenase type II/amyloid binding alcohol dehydrogenase (HADH II/ABAD), a ... L-3-hydroxyacyl-CoA dehydrogenase II protects in a model of Parkinsons disease Ann Neurol. 2004 Jul;56(1):51-60. doi: 10.1002/ ... The neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) impairs mitochondrial respiration and damages dopaminergic ...
Recognition of Structurally Diverse Substrates by Type II 3-Hydroxyacyl-Coa Dehydrogenase (Hadh II) Amyloid-Beta Binding ... 3] Andreeva A., Howorth D., Chandonia J.-M., Brenner S.E., Hubbard T.J.P., Chothia C., Murzin A.G. (2008).. Data growth and its ... Rat brain 3-hydroxyacyl-CoA dehydrogenase binary complex with NADH and 3-keto butyrate. ... If available, the SCOP 1.75 domain assignment [3] is used. Otherwise algorithmic domain assignments are computed using the ...
Hydroxyacyl-CoA dehydrogenase (EC1.1.1.35) from mitochondria at the resolution 2.0 A, Northeast Structural Genomics Consortium ... Hydroxyacyl-CoA dehydrogenase (EC1.1.1.35) from mitochondria at the resolution 2.0 A, Northeast Structural Genomics Consortium ... Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial. A, B. 324. Homo sapiens. Mutation(s): 0 Gene Names: HADH (HAD, HADHSC, ... GLYCERIN; PROPANE-1,2,3-TRIOL. C3 H8 O3. PEDCQBHIVMGVHV-UHFFFAOYSA-N. ...
CoA) Dehydrogenase (LCHAD) Associated Neuropathy. The safety and scientific validity of this study is the responsibility of the ... Long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD). Mitochondrial trifunctional protein (TFP). LCHAD/TFP deficiency with ... Vitamin E Treatment for Long-Chain 3-Hydroxyacyl Coenzyme A ( ...
What is 3-hydroxyacyl-CoA dehydrogenase? Meaning of 3-hydroxyacyl-CoA dehydrogenase medical term. What does 3-hydroxyacyl-CoA ... Looking for online definition of 3-hydroxyacyl-CoA dehydrogenase in the Medical Dictionary? 3-hydroxyacyl-CoA dehydrogenase ... yl-CoA de·hy·dro·gen·ase. (hī-droksē-asil dēhī-drōjen-ā), β-Hydroxyacyl dehydrogenase; an enzyme catalyzing the oxidation ... 3-hydroxyacyl-CoA dehydrogenase. Also found in: Acronyms, Wikipedia. 3-hy·drox·y·ac· ...
2-Methylacetoacetyl-CoA + NADH. details 2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Ion. ... Submicroscopic duplications of the hydroxysteroid dehydrogenase HSD17B10 and the E3 ubiquitin ligase HUWE1 are associated with ... 2004 Sep 17;342(3):943-52. [PubMed:15342248 ] *Lustbader JW, Cirilli M, Lin C, Xu HW, Takuma K, Wang N, Caspersen C, Chen X, ... S)-3-hydroxyacyl-CoA + NAD → 3-oxoacyl-CoA + NADH. details 2-Methyl-3-hydroxybutyryl-CoA + NAD → ...
Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations Biochem J ... such as KATP channelopathies and regulatory defects of glucokinase and glutamate dehydrogenase. Although defects of fatty acid ... Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose-fatty acid cycle? S. ... β-oxidation, carnitine palmitoyltransferase I (CPT I), 3-hydroxyacyl-CoA dehydrogenase, hyperinsulinism ...
86, nr 3, 329-337 s. Nyckelord [en] Chorioretinal atrophy, electroetinography, LCHAD deficiency, myopia Nationell ämneskategori ... 86, nr 3, 329-337 s.Artikel i tidskrift (Refereegranskat) Published Abstract [en] PURPOSE ... Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a cross-sectional study with ... To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA ...
Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)Download version for offline viewing or printing. [463.65kB] ... Babies with the diseases Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) or Trifunctional Protein Deficiency (TFP ...
... and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency are long-chain fatty acid oxidation disorders with ... For very-long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the most common long-chain fatty oxidation defect, it has been ... Ficicioglu C, Coughlin CR 2nd, Bennett MJ, Yudkoff M. Very long-chain acyl-CoA dehydrogenase deficiency in a patient with ... A near-miss: very long chain acyl-CoA dehydrogenase deficiency with normal primary markers in the initial well-timed newborn ...
The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase ( ... 2002). Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients. Pediatrics. ... LCHAD (Long-Chain 3-Hydroxyacyl CoA Dehydrogenase) deficiency. LCHAD (long-chain 3-hydroxyacyl CoA dehydrogenase) deficiency is ... Chronically 3-hydroxylated dicarboxylic acids and nonhydroxylated dicarboxylic acids will be positive. Plasma carnitine may be ...
... glutamate dehydrogenase; immune response; recombinant proteins; surface proteins; vaccine development. Abstract:. ... ... and glutamate dehydrogenase (gdhA)) against Flavobacterium psychrophilum were investigated in ayu (Plecoglossus altivelis). ... 3-hydroxyacyl-CoA dehydrogenase; Escherichia coli; Flavobacterium psychrophilum; H+/K+-exchanging ATPase; H-transporting ATP ... Protective efficacies of three antigenic proteins (3-hydroxyacyl-CoA dehydrogenase (HCD), ATP synthase beta subunit (atpD), ...
3A and B) mainly due to a larger insulin-induced increase in total blood flow in the trained leg (Fig. 2B). When blood flow was ... 3B, glucose uptake was calculated as an average from 0 to 120 min of insulin infusion and a ∼60% greater (P = 0.008) insulin- ... 4B and 7). Training did not influence the phosphorylation state of Akt Ser473, Akt Thr308, GSK-3α Ser21, GSK-3β Ser9, or GS ... 2C and 3A and B) was calculated across each thigh using the Fick principle, by multiplication of venous blood flow (Fig. 2B) ...
... of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for 3-alpha ... 3-alpha hydroxyacyl-CoA dehydrogenase deficiency Title Other Names:. 3-hydroxylacyl-CoA dehydrogenase deficiency; Medium and ... PubMed is a searchable database of medical literature and lists journal articles that discuss 3-alpha hydroxyacyl-CoA ... dehydrogenase deficiency. Click on the link to view a sample search on this topic. ...
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 ...
isovaleryl-CoA dehydrogenase Ja 1.3.99.13 long-chain-acyl-CoA dehydrogenase Ja ... L-iditol 2-dehydrogenase Ja 1.1.1.15 D-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. D-sorbose + NADH + H+ D-iditol 2- ... D-arabitol 4-dehydrogenase 1.1.1.12 L-arabitol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-xylulose + NADH + H+ L-arabitol ... L-arabitol 2-dehydrogenase 1.1.1.14 L-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-sorbose + NADH + H+ ...
The most important finding of this study was the detection of the lipid-metabolizing enzyme, 3-hydroxyacyl-CoA dehydrogenase ( ... From: Identification of leptospiral 3-hydroxyacyl-CoA dehydrogenase released in the urine of infected hamsters ...
... medium-chain acyl-CoA dehydrogenase deficiency (Table) (3.6). Newborn screening can help prevent death or disability, if ... The five most commonly diagnosed conditions in the United States are 1) hearing loss, 2) primary congenital hypothyroidism, 3) ... 3). Additional conditions for screening continue to be identified and nominated for inclusion in the panel. ... 3). Through early identification, newborn screening provides an opportunity for treatment and significant reductions in ...
Short-chain dehydrogenase/reductase SDR * Occurring in:. *3-hydroxyacyl-CoA dehydrogenase type-2. > NAD(P)-binding domain ... 2-methylacetoacetyl-CoA + NADH. 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholanate + NAD(+) = 3-alpha,12-alpha-dihydroxy-7- ... Sequence domains: short chain dehydrogenase Structure domains: NAD(P)-binding Rossmann-like Domain ... Virtual screening applications in short-chain dehydrogenase/reductase research. Beck et al. (2017) ...
Catalase and the endoplasmic reticulum A beta binding dehydrogenase (ERAB) are both inhibited by characterized fragments of the ... Catalase and the endoplasmic reticulum A beta binding dehydrogenase (ERAB) are both inhibited by characterized fragments of the ...
Fraser H1, Geppert J2, Johnson R3, Johnson S4, Connock M2, Clarke A2, Taylor-Phillips S2, Stinton C2. ... 3. Faculty of Health and Life Sciences, Coventry University, Coventry, CV1 5RW, UK.. 4. Warwick Library, University of Warwick ... 3. Forest plot showing mortality and incidence of cardiac and liver problems across symptomatically and asymptomatically ... For follow up analysis 3 we found a significant difference for only one comparison, in the incidence of cardiomyopathy between ...
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. ...
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, ...
... hydroxyacyl-CoA dehydrogenase fragment of rat peroxisomal MFE-2 (PDB code 1GZ6) and molecule A of the (2E)-enoyl-CoA hydratase ... hydroxyacyl-CoA dehydrogenase domain, a C-terminal (2E)-enoyl-CoA hydratase 2 domain and a short loop which connects the two ... Hydroxyacyl-CoA dehydrogenase domain of Dm. MFE-2. The (3R)-hydroxyacyl-CoA dehydrogenase domain of DmMFE-2 consists of amino ... Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional ...
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 ...
3-hydroxyacyl-coenzyme A dehydrogenase deficiency 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- ...
  • 3-hydroxyacyl-CoA dehydrogenase deficiency is an inherited condition that prevents the body from converting certain fats to energy, particularly during prolonged periods without food (fasting). (medlineplus.gov)
  • Problems related to 3-hydroxyacyl-CoA dehydrogenase deficiency can be triggered by periods of fasting or by illnesses such as viral infections. (medlineplus.gov)
  • Mutations in the HADH gene cause 3-hydroxyacyl-CoA dehydrogenase deficiency. (medlineplus.gov)
  • As a result, these fatty acids are not converted to energy, which can lead to characteristic features of 3-hydroxyacyl-CoA dehydrogenase deficiency, such as lethargy and hypoglycemia. (medlineplus.gov)
  • Conditions that disrupt the metabolism of fatty acids, including 3-hydroxyacyl-CoA dehydrogenase deficiency, are known as fatty acid oxidation disorders. (medlineplus.gov)
  • Fatal hepatic short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency: clinical, biochemical, and pathological studies on three subjects with this recently identified disorder of mitochondrial beta-oxidation. (medlineplus.gov)
  • IJlst L, Wanders RJ, Ushikubo S, Kamijo T, Hashimoto T. Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein. (medscape.com)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a severe fatty acid oxidation disorder. (medscape.com)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid beta-oxidation. (medscape.com)
  • Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast. (medscape.com)
  • Clinical, biochemical, and morphologic investigations of a case of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (medscape.com)
  • C rearrangement in patients with long chain 3-hydroxyacyl-CoA dehydrogenase deficiency from Ukraine. (medscape.com)
  • C of HADHA gene causing long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) in the population of adult Kashubians from North Poland. (medscape.com)
  • Clinical outcome, biochemical and therapeutic follow-up in 14 Austrian patients with Long-Chain 3-Hydroxy Acyl CoA Dehydrogenase Deficiency (LCHADD). (medscape.com)
  • Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myopathy and cardiomyopathy in early childhood. (medscape.com)
  • Schiff M, Mohsen AW, Karunanidhi A, McCracken E, Yeasted R, Vockley J. Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. (medscape.com)
  • Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxy-acyl-CoA dehydrogenase deficiency. (medscape.com)
  • Pigmentary retinopathy in long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. (medscape.com)
  • The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with acute fatty liver of pregnancy. (thefreedictionary.com)
  • Common missense mutation G1528 in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (thefreedictionary.com)
  • Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (thefreedictionary.com)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: high frequency of the G1528C mutation with no apparent correlation with clinical phenotype. (thefreedictionary.com)
  • Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose-fatty acid cycle? (portlandpress.com)
  • Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency : a cross-sectional study with long-term follow-up. (diva-portal.org)
  • To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency - a life-threatening metabolic disease - and the relation to age at diagnosis, treatment and other clinical parameters. (diva-portal.org)
  • Unusual chorioretinal findings should alert the ophthalmologist to the long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, especially if there is a history of neonatal hypoglycaemia or failure to thrive. (diva-portal.org)
  • Babies with the diseases Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) or Trifunctional Protein Deficiency (TFP) are identified through newborn screening because they have an elevated C160H level. (newbornscreening.on.ca)
  • Mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency are long-chain fatty acid oxidation disorders with particularly high morbidity and mortality. (biomedcentral.com)
  • LCHAD (long-chain 3-hydroxyacyl CoA dehydrogenase) deficiency is a recessively inherited genetic disorder of fatty acid oxidation. (brainology.net)
  • 1996). Pathology of skeletal muscle and impaired respiratory chain function in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528 mutation. (brainology.net)
  • 1997). Pathology of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation. (brainology.net)
  • Hyperinsulinism due to short chain 3 hydroxylacyl-CoA dehydrogenase (SCHAD) deficiency is a recently described mitochondrial fatty acid oxidation disorder characterized by hyperinsulinemic hypoglycemia with seizures , and in one case fulminant hepatic failure. (cdc.gov)
  • The five most commonly diagnosed conditions in the United States are 1) hearing loss, 2) primary congenital hypothyroidism, 3) cystic fibrosis, 4) sickle cell disease, and 5) medium-chain acyl-CoA dehydrogenase deficiency ( Table ) (3.6). (cdc.gov)
  • Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficiency: a systematic review. (cdc.gov)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD deficiency) and Mitochondrial Trifunctional protein deficiency (MTP deficiency) also known as Trifunctional Protein (TFP) deficiency are disorders due to different defects in the MTP. (genedx.com)
  • Both isolated LCHAD deficiency and MTP deficiency, which has deficiencies in all 3 enzyme activities, have overlapping clinical presentations. (genedx.com)
  • for this reason this disorder is sometimes called medium- and short-chain 3-hydroxyacyl-coenzyme A dehydrogenase (M/SCHAD) deficiency. (wikipedia.org)
  • Individuals with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency are also at risk for complications such as seizures, life-threatening heart and breathing problems, coma, and sudden unexpected death. (wikipedia.org)
  • Over the past decade, several clinical observations have demonstrated an association between these maternal syndromes and a recessively inherited fatty acid oxidation disorder, long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency. (biomedsearch.com)
  • D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein deficiency : a newly identified peroxisomal disorder. (nii.ac.jp)
  • HADHA-related disorders, including long chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) and mitochondrial trifunctional protein deficiency (MTPD), are inherited conditions in which the body lacks sufficient amounts of the enzyme(s) needed to turn certain fatty acids from food and body fat into energy. (counsyl.com)
  • Most inborn errors of metabolism (IEM) are recessive, genetically transmitted diseases and are classified into 3 main groups according to their mechanisms: cellular intoxication, energy deficiency, and defects of complex molecules. (biomedcentral.com)
  • They are classified into 3 main groups according to their mechanisms: cellular intoxication, energy deficiency, and degradation or synthesis defects of complex molecules [ 1 ]. (biomedcentral.com)
  • The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. (genecards.org)
  • EHHADH (Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase) is a Protein Coding gene. (genecards.org)
  • Subunit activities include: enoyl-CoA hydratase ( EC:4.2.1.17 ) and 3-hydroxyacyl-CoA dehydrogenase ( EC:1.1.1.35 ). (ebi.ac.uk)
  • Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase. (medlineplus.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)
  • 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)
  • The neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) impairs mitochondrial respiration and damages dopaminergic neurons as seen in Parkinson's disease (PD). (nih.gov)
  • Here, we report that L-3-hydroxyacyl-CoA dehydrogenase type II/amyloid binding alcohol dehydrogenase (HADH II/ABAD), a mitochondrial oxidoreductase enzyme involved in neuronal survival, is downregulated in PD patients and in MPTP-intoxicated mice. (nih.gov)
  • LCHAD is 1 of 3 enzymatic activities that make up the mitochondrial trifunctional protein (MTP) of the inner mitochondrial membrane. (brainology.net)
  • Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficien. (cdc.gov)
  • Mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiencies are rare fatty acid β-oxidation disorders. (cdc.gov)
  • MTP is an enzyme complex at the inner mitochondrial membrane with three enzymatic activities, long-chain 3-hydroxyacyl-CoA dehydrogenase, 2-enoyl hydratase and 3-keto acyl-CoA thiolase activities. (genedx.com)
  • Both peroxisomal MFE-1 and the membrane-associated mitochondrial trifunctional enzyme complex house (3 S )-hydroxy-specific dehydrogenase [ 4 , 5 ]. (portlandpress.com)
  • The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. (genecards.org)
  • Among its related pathways are Mitochondrial Fatty Acid Beta-Oxidation and Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA . (genecards.org)
  • Leigh Syndrome is a rare genetic disorder caused by mutations in mitochondrial DNA or by deficiencies of an enzyme called pyruvate dehydrogenase. (rareshare.org)
  • Muscle specimens were obtained by percutaneous needle biopsy of the vastus lateralis muscle and the activity of two mitochondrial enzymes (citrate synthase (CS) and 3-hydroxyacyl CoA dehydrogenase (HADH)) was measured. (bmj.com)
  • The HADH gene provides instructions for making an enzyme called 3-hydroxyacyl-CoA dehydrogenase. (medlineplus.gov)
  • Mutations in the HADH gene lead to a shortage of 3-hydroxyacyl-CoA dehydrogenase. (medlineplus.gov)
  • In humans, the following genes encode proteins with 3-hydroxyacyl-CoA dehydrogenase activity: HADH - Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme HSD17B4 - Peroxisomal multifunctional enzyme type 2 3-Hydroxyacyl CoA dehydrogenase is classified as an oxidoreductase. (wikipedia.org)
  • It is caused by a mutation in the HADH gene (4q22-q26) encoding the SCHAD protein that has a dual function both as an enzyme and an inhibitor of glutamate dehydrogenase. (cdc.gov)
  • Mutations in the HADH gene lead to inadequate levels of an enzyme called 3-hydroxyacyl-coenzyme A dehydrogenase. (wikipedia.org)
  • HADH (Hydroxyacyl-CoA Dehydrogenase) is a Protein Coding gene. (genecards.org)
  • Virtual screening applications in short-chain dehydrogenase/reductase research. (ebi.ac.uk)
  • dehydrogenase/reductase (SDR family) memb. (broadinstitute.org)
  • l-3-Hydroxyacyl-CoA dehydrogenase from H. sapiens (HuHAD) has acetoacetyl-CoA reductase activity, and the crystal structure was determined as a ternary complex with NAD + and acetoacetyl-CoA (PDB entry 1f0y [2] ). (proteopedia.org)
  • this was accompanied by the up-regulation of beta-oxidation activity, and acyl-CoA oxidase (ACO) and medium-chain acyl-CoA dehydrogenase (MCAD) mRNA levels. (biomedsearch.com)
  • 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)
  • A simple, straightforward assay for long-chain 3-hydroxyacyl-CoA dehydrogenase based on the use of 4ethylmaleimide: potential for pre and postnatal diagnosis. (thefreedictionary.com)
  • 4. Sund, H. and Theorell, H. Alcohol dehydrogenase. (qmul.ac.uk)
  • 5. Theorell, H. Kinetics and equilibria in the liver alcohol dehydrogenase system. (qmul.ac.uk)
  • alcohol dehydrogenase 4 (class II), pi po. (broadinstitute.org)
  • Several molecular defects are known to cause hyperinsulinism of infancy, such as K ATP channelopathies and regulatory defects of glucokinase and glutamate dehydrogenase. (portlandpress.com)
  • Protective efficacies of three antigenic proteins (3-hydroxyacyl-CoA dehydrogenase (HCD), ATP synthase beta subunit (atpD), and glutamate dehydrogenase (gdhA)) against Flavobacterium psychrophilum were investigated in ayu (Plecoglossus altivelis). (usda.gov)
  • 1 In rarer cases of PHHI, mutations in the glucokinase gene ( GCK ), glutamate dehydrogenase gene ( GLUD1 ), and short-chain hydroxyacyl-coenzyme A dehydrogenase ( HADHSC ) gene are pathogenic and act mainly by subverting the operation of K ATP channels. (aappublications.org)
  • In other cases, including those involving mutations in glucokinase, glutamate dehydrogenase, and short-chain hydroxyacyl-coenzyme A dehydrogenase genes, diazoxide and appropriate diet are required for a longer time but are very effective in controlling hypoglycemia. (aappublications.org)
  • The 3-hydroxyacyl-CoA dehydrogenase enzyme is required for a step that metabolizes groups of fats called medium-chain fatty acids and short-chain fatty acids. (medlineplus.gov)
  • Belongs to the short-chain dehydrogenases/reductases (SDR) family. (abcam.com)
  • The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase (LCKT). (brainology.net)
  • actin related protein 2/3 complex, subu. (broadinstitute.org)
  • Fatty acyl-CoA represents bioactive compounds that are involved in protein transport, enzyme activation, protein acylation, cell signaling, and transcriptional control in addition to serving as substrates for beta oxidation and phospholipid biosynthesis. (hindawi.com)
  • On the basis of the data presented in this work, we propose that long-chain fatty acyl-CoA ligase enzyme serves as an important protein and a potential target candidate for development of selective inhibitors against leishmaniasis. (hindawi.com)
  • In order to clarify the pathogenesis of X-linked adrenoleukodystrophy (ALD), complementary DNA for human very long chain fatty acyl-CoA synthetase (VLACS), which is deficient in ALD was cloned using rat cDNA.Human VLACS cDNA encodes 620 amino acids with high homology to rat enzyme and fatty acid transport protein and the gene was assinged to chromosome 15q21.2. (nii.ac.jp)
  • The beta subunit has acetyl-CoA C-acyltransferase ( EC:2.3.1.16 ) activity. (ebi.ac.uk)
  • the oxidation of L-3-hydroxyacyl CoA by NAD+. (wikipedia.org)
  • Acyl-CoA esters are oxidized in the β-oxidation spiral that is the major pathway for the breakdown of fatty acids and is found, depending on the species, in peroxisomes and mitochondria. (portlandpress.com)
  • The second and third reactions in the peroxisomal β-oxidation cycle ( Figure 1 ) are performed by an MFE (multifunctional enzyme) that consists of one single polypeptide chain harbouring both dehydrogenase and hydratase activities. (portlandpress.com)
  • 3-Oxohexadecanoyl-CoA has a role in the synthesis and oxidation of fatty acid. (hmdb.ca)
  • 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)
  • Enzymes catalyzing reactions in phosphagenic [creatine kinase (CK)] and glycolytic [hexokinase (HK), phosphofructokinase (PFK), and lactate dehydrogenase (LDH)] metabolic pathways had significantly higher activities (about 30-40%) in the Black African group than in the Caucasian group (P less than 0.01). (physicsforums.com)
  • It was the aim of this study to elucidate the organization of its CoA-derived pathways and to use the genomic information of X. dendrorhous for a phylogenomic investigation of the Basidiomycota. (biomedcentral.com)
  • A detailed characterization of the CoA-related pathways was done and all genes for fatty acid, sterol and carotenoid synthesis have been assigned. (biomedcentral.com)
  • acyl-CoA synthetase long-chain family mem. (broadinstitute.org)
  • Fatty acyl-CoA synthetase occupies a pivotal role in cellular homeostasis, particularly in lipid metabolism. (hindawi.com)
  • Our interest in fatty acyl-CoA synthetase stems from the identification of this enzyme, long-chain fatty acyl-CoA ligase (LCFA) by microarray analysis. (hindawi.com)
  • 3-HIBA is an intermediate in L-valine metabolism. (hmdb.ca)
  • One interesting target which emerged from our microarray experiments [ 4 ] was long-chain fatty acid-CoA ligase (EC 6.2.1.3) (GenBank Accession No. XM_001681734), a key enzyme involved in the metabolism of fatty acids in all organisms [ 5 - 9 ]. (hindawi.com)
  • 603178 ). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. (hmdb.ca)
  • Furthermore, to investigate the catalytic residues of CacHBD, the enzymatic activities of the wild type and of three single-amino-acid mutants were analyzed, in which the Ser, His and Asn residues that are conserved in the HBDs from C. acetobutylicum , C. butyricum and Ralstonia eutropha , as well as in the L-3-hydroxyacyl-CoA dehydrogenases from Homo sapiens and Escherichia coli , were substituted by alanines. (proteopedia.org)
  • This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. (genecards.org)
  • 3 , 4 The overall experience of using PGD for single gene disorders is presently almost 1500 cases, showing that it is an established alternative to traditional prenatal diagnosis, which may be reliably applied as an integral part of genetic practices. (glowm.com)
  • AFG3 ATPase family gene 3-like 2 (S. ce. (broadinstitute.org)
  • In the present study, we confirm the presence of long-chain fatty acyl-CoA ligase gene in the genome of clinical isolates of Leishmania donovani collected from the disease endemic area in India. (hindawi.com)
  • Our results reveal that rapamycin inhibits the phosphorylation of TORC1 and its downstream effectors (S6K1, S6 and 4E-BP1), without affecting Akt and the Akt substrates Forkhead-box Class O1 (FoxO1) and glycogen synthase kinase 3α/β (GSK 3α/β). (biologists.org)
  • Congenital hyperinsulinism is the most common cause of hypoglycemia (low blood sugar) in infants more than 3 days old, as well as children. (cookchildrens.org)
  • 2 , 3 , 5 , 6 However, almost 80% of neonates with congenital hyperinsulinism fail to respond to medical treatment and require near-total pancreatectomy. (aappublications.org)
  • Enzymes that catalyze the reversible reduction of alpha-carboxyl group of 3-hydroxy-3-methylglutaryl-coenzyme A to yield MEVALONIC ACID. (curehunter.com)
  • These are organic compounds containing a 3-hydroxyl acylated coenzyme A derivative. (hmdb.ca)
  • A molecule of coenzyme A carrying an acetyl group is also referred to as acetyl-CoA. (hmdb.ca)
  • These are organic compounds containing a coenzyme A derivative, which is 3-oxo acylated long aliphatic chain of 13 to 21 carbon atoms. (hmdb.ca)
  • Catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. (abcam.com)
  • It converts acetoacetyl-CoA to ( S )-3HB-CoA in the synthetic metabolic pathway. (proteopedia.org)
  • Among these enzymes, increased production of 3-hydroxy-acyl-ACP dehydratase (FabZ) resulted in the highest increase (+ 45%) in octanoic acid titer. (springer.com)
  • Recommendations for genetic testing include 1) to test for a disorder for which the causative genes have been identified, 2) use a Clinical Laboratories Improvement Amendments (CLIA) approved laboratory, 3) order the most specific test and avoid unnecessary parallel testing, 4) avoid testing for genetically complex disorders for which no treatment or surveillance guidelines exist, and 5) avoid genetic testing in asymptomatic minors when involving untreatable disorders except in rare circumstances. (aao.org)
  • Thus, short-term (7 days) cycle exercise training increases insulin-stimulated phosphatidylinositol 3-kinase (PI3-K) activity in young, but not middle-aged, subjects ( 18 , 19 ). (diabetesjournals.org)
  • Exerts it highest activity toward 3-hydroxybutyryl-CoA. (genecards.org)
  • C-D. ETC. complex IV enzyme activity was assessed in EV and HSD10 ov cells treated with 0.75 mM H2O2 for C) 24 hours (n = 3), and D) 0, 1, 6, and 16 hours (n = 6 for all time points). (nih.gov)
  • belongs to the class of organic compounds known as long-chain 3-oxoacyl coas. (hmdb.ca)
  • Long-chain fatty acyl-CoA ligase is critical enzyme processing long-chain fatty acid acylation which is essential for lysophosphatidylinositol (lyso-PI) incorporation into glycosyl phosphatidylinositols (GPIs) [ 16 , 17 ]. (hindawi.com)
  • De novo sphingolipid biosynthesis starts with the condensation of serine and the product of long-chain fatty acyl-CoA ligase. (hindawi.com)
  • L. major preferentially incorporates myristoyl-CoA (C14) over palmitoyl-CoA (C16) into their long-chain base [ 19 , 20 ]. (hindawi.com)
  • This selection of specific long-chain fatty acyl-CoA reflects the presence of myristoyl-specific long-chain fatty acyl-CoA ligase in Leishmania [ 21 ]. (hindawi.com)