An NAPH-dependent cytochrome P450 enzyme that catalyzes the oxidation of the side chain of sterol intermediates such as the 27-hydroxylation of 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol.
Oxidases that specifically introduce DIOXYGEN-derived oxygen atoms into a variety of organic molecules.
Widely distributed enzymes that carry out oxidation-reduction reactions in which one atom of the oxygen molecule is incorporated into the organic substrate; the other oxygen atom is reduced and combined with hydrogen ions to form water. They are also known as monooxygenases or hydroxylases. These reactions require two substrates as reductants for each of the two oxygen atoms. There are different classes of monooxygenases depending on the type of hydrogen-providing cosubstrate (COENZYMES) required in the mixed-function oxidation.
An NADPH-dependent flavin monooxygenase that plays a key role in the catabolism of TRYPTOPHAN by catalyzing the HYDROXYLATION of KYNURENINE to 3-hydroxykynurenine. It was formerly characterized as EC and EC
A family of gram-negative, aerobic bacteria utilizing only one-carbon organic compounds and isolated from in soil and water.
A species of METHYLOCOCCUS which forms capsules and is capable of autotrophic carbon dioxide fixation. (From Bergey's Manual of Determinative Bacteriology, 9th ed)
A superfamily of hundreds of closely related HEMEPROTEINS found throughout the phylogenetic spectrum, from animals, plants, fungi, to bacteria. They include numerous complex monooxygenases (MIXED FUNCTION OXYGENASES). In animals, these P-450 enzymes serve two major functions: (1) biosynthesis of steroids, fatty acids, and bile acids; (2) metabolism of endogenous and a wide variety of exogenous substrates, such as toxins and drugs (BIOTRANSFORMATION). They are classified, according to their sequence similarities rather than functions, into CYP gene families (>40% homology) and subfamilies (>59% homology). For example, enzymes from the CYP1, CYP2, and CYP3 gene families are responsible for most drug metabolism.
A species of METHYLOSINUS which is capable of degrading trichloroethylene and other organic pollutants.
A soluble cytochrome P-450 enzyme that catalyzes camphor monooxygenation in the presence of putidaredoxin, putidaredoxin reductase, and molecular oxygen. This enzyme, encoded by the CAMC gene also known as CYP101, has been crystallized from bacteria and the structure is well defined. Under anaerobic conditions, this enzyme reduces the polyhalogenated compounds bound at the camphor-binding site.
A genus of gram-negative, ellipsoidal or rod-shaped bacteria whose major source of energy and reducing power is from the oxidation of ammonia to nitrite. Its species occur in soils, oceans, lakes, rivers, and sewage disposal systems.
A condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)
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 P450 oxidoreductase that catalyzes the hydroxylation of the terminal carbon of linear hydrocarbons such as octane and FATTY ACIDS in the omega position. The enzyme may also play a role in the oxidation of a variety of structurally unrelated compounds such as XENOBIOTICS, and STEROIDS.
A genus of gram-negative, aerobic, rod-shaped bacteria widely distributed in nature. Some species are pathogenic for humans, animals, and plants.
Elimination of ENVIRONMENTAL POLLUTANTS; PESTICIDES and other waste using living organisms, usually involving intervention of environmental or sanitation engineers.
A benzyl-indazole having analgesic, antipyretic, and anti-inflammatory effects. It is used to reduce post-surgical and post-traumatic pain and edema and to promote healing. It is also used topically in treatment of RHEUMATIC DISEASES and INFLAMMATION of the mouth and throat.
The simplest saturated hydrocarbon. It is a colorless, flammable gas, slightly soluble in water. It is one of the chief constituents of natural gas and is formed in the decomposition of organic matter. (Grant & Hackh's Chemical Dictionary, 5th ed)
An enzyme that catalyzes the conversion of L-tyrosine, tetrahydrobiopterin, and oxygen to 3,4-dihydroxy-L-phenylalanine, dihydrobiopterin, and water. EC
The second enzyme in the committed pathway for CHOLESTEROL biosynthesis, this enzyme catalyzes the first oxygenation step in the biosynthesis of STEROLS and is thought to be a rate limiting enzyme in this pathway. Specifically, this enzyme catalyzes the conversion of SQUALENE to (S)-squalene-2,3-epoxide.
Placing of a hydroxyl group on a compound in a position where one did not exist before. (Stedman, 26th ed)
An enzyme that utilizes NADH or NADPH to reduce FLAVINS. It is involved in a number of biological processes that require reduced flavin for their functions such as bacterial bioluminescence. Formerly listed as EC and EC
A flavoprotein that catalyzes the reduction of heme-thiolate-dependent monooxygenases and is part of the microsomal hydroxylating system. EC
These enzymes catalyze the elimination of ammonia from amidines with the formation of a double bond. EC 4.3.2.
Systems of enzymes which function sequentially by catalyzing consecutive reactions linked by common metabolic intermediates. They may involve simply a transfer of water molecules or hydrogen atoms and may be associated with large supramolecular structures such as MITOCHONDRIA or RIBOSOMES.
Derivatives of the dimethylisoalloxazine (7,8-dimethylbenzo[g]pteridine-2,4(3H,10H)-dione) skeleton. Flavin derivatives serve an electron transfer function as ENZYME COFACTORS in FLAVOPROTEINS.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
The class of all enzymes catalyzing oxidoreduction reactions. The substrate that is oxidized is regarded as a hydrogen donor. The systematic name is based on donor:acceptor oxidoreductase. The recommended name will be dehydrogenase, wherever this is possible; as an alternative, reductase can be used. Oxidase is only used in cases where O2 is the acceptor. (Enzyme Nomenclature, 1992, p9)
A bacterial genus of the order ACTINOMYCETALES.
An antiseptic and disinfectant aromatic alcohol.
A highly volatile inhalation anesthetic used mainly in short surgical procedures where light anesthesia with good analgesia is required. It is also used as an industrial solvent. Prolonged exposure to high concentrations of the vapor can lead to cardiotoxicity and neurological impairment.
A widely used industrial solvent.
Closed vesicles of fragmented endoplasmic reticulum created when liver cells or tissue are disrupted by homogenization. They may be smooth or rough.
A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.
A family of gram-negative methanotrophs in the order Rhizobiales, distantly related to the nitrogen-fixing and phototrophic bacteria.
A large group of aerobic bacteria which show up as pink (negative) when treated by the gram-staining method. This is because the cell walls of gram-negative bacteria are low in peptidoglycan and thus have low affinity for violet stain and high affinity for the pink dye safranine.
A family of aerobic gram-negative rods that are nitrogen fixers. They are highly viscous, and appear as a semitransparent slime in giant colonies.
The facilitation of a chemical reaction by material (catalyst) that is not consumed by the reaction.
A species of gram-negative, aerobic bacteria isolated from soil and water as well as clinical specimens. Occasionally it is an opportunistic pathogen.
The generic name for the group of aliphatic hydrocarbons Cn-H2n+2. They are denoted by the suffix -ane. (Grant & Hackh's Chemical Dictionary, 5th ed)
Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)
Drug metabolizing enzymes which oxidize methyl ethers. Usually found in liver microsomes.
A drug-metabolizing enzyme found in the hepatic, placental and intestinal microsomes that metabolizes 7-alkoxycoumarin to 7-hydroxycoumarin. The enzyme is cytochrome P-450- dependent.
A species of gram-negative bacteria in the genus PSEUDOMONAS, which is found in SOIL and WATER.
Unsaturated hydrocarbons of the type Cn-H2n, indicated by the suffix -ene. (Grant & Hackh's Chemical Dictionary, 5th ed, p408)
Phenols substituted with one or more chlorine atoms in any position.
The rate dynamics in chemical or physical systems.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
Toxic chlorinated unsaturated hydrocarbons. Include both the 1,1- and 1,2-dichloro isomers. Both isomers are toxic, but 1,1-dichloroethylene is the more potent CNS depressant and hepatotoxin. It is used in the manufacture of thermoplastic polymers.
A species of gram-negative bacteria in the genus PSEUDOMONAS, containing multiple genomovars. It is distinguishable from other pseudomonad species by its ability to use MALTOSE and STARCH as sole carbon and energy sources. It can degrade ENVIRONMENTAL POLLUTANTS and has been used as a model organism to study denitrification.
The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.
A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. Note that the aqueous form of ammonia is referred to as AMMONIUM HYDROXIDE.
A colorless liquid used as a solvent and an antiseptic. It is one of the ketone bodies produced during ketoacidosis.
A heavy metal trace element with the atomic symbol Cu, atomic number 29, and atomic weight 63.55.
8-Hydroxyquinolinols chlorinated on the number 5 and/or 7 carbon atom(s). They are antibacterial, antiprotozoal, and antidiarrheal, especially in amebiasis, and have also been used as antiseborrheics. The compounds are mostly used topically, but have been used also as animal feed additives. They may cause optic and other neuropathies and are most frequently administered in combination with other agents.
An element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
An enzyme that catalyzes the hydroxylation of TRYPTOPHAN to 5-HYDROXYTRYPTOPHAN in the presence of NADPH and molecular oxygen. It is important in the biosynthesis of SEROTONIN.
A set of genes descended by duplication and variation from some ancestral gene. Such genes may be clustered together on the same chromosome or dispersed on different chromosomes. Examples of multigene families include those that encode the hemoglobins, immunoglobulins, histocompatibility antigens, actins, tubulins, keratins, collagens, heat shock proteins, salivary glue proteins, chorion proteins, cuticle proteins, yolk proteins, and phaseolins, as well as histones, ribosomal RNA, and transfer RNA genes. The latter three are examples of reiterated genes, where hundreds of identical genes are present in a tandem array. (King & Stanfield, A Dictionary of Genetics, 4th ed)
Organic compounds that include a cyclic ether with three ring atoms in their structure. They are commonly used as precursors for POLYMERS such as EPOXY RESINS.
Compounds in which one or more of the three hydroxyl groups of glycerol are in ethereal linkage with a saturated or unsaturated aliphatic alcohol; one or two of the hydroxyl groups of glycerol may be esterified. These compounds have been found in various animal tissue.
A coenzyme for a number of oxidative enzymes including NADH DEHYDROGENASE. It is the principal form in which RIBOFLAVIN is found in cells and tissues.
A flavoprotein that catalyzes the synthesis of protocatechuic acid from 4-hydroxybenzoate in the presence of molecular oxygen. EC
An enzyme that catalyzes the oxidation of BENZOATE to 4-hydroxybenzoate. It requires IRON and tetrahydropteridine.
A bicyclic monoterpene ketone found widely in plants, especially CINNAMOMUM CAMPHORA. It is used topically as a skin antipruritic and as an anti-infective agent.
The functional hereditary units of BACTERIA.
Organic esters or salts of sulfonic acid derivatives containing an aliphatic hydrocarbon radical.
Artifactual vesicles formed from the endoplasmic reticulum when cells are disrupted. They are isolated by differential centrifugation and are composed of three structural features: rough vesicles, smooth vesicles, and ribosomes. Numerous enzyme activities are associated with the microsomal fraction. (Glick, Glossary of Biochemistry and Molecular Biology, 1990; from Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)
The chemical alteration of an exogenous substance by or in a biological system. The alteration may inactivate the compound or it may result in the production of an active metabolite of an inactive parent compound. The alterations may be divided into METABOLIC DETOXICATION, PHASE I and METABOLIC DETOXICATION, PHASE II.
A second-line antitubercular agent that inhibits mycolic acid synthesis.
A genus in the family BURKHOLDERIACEAE, comprised of many species. They are associated with a variety of infections including MENINGITIS; PERITONITIS; and URINARY TRACT INFECTIONS.
A colorless, toxic liquid with a strong aromatic odor. It is used to make rubbers, polymers and copolymers, and polystyrene plastics.
Indolesulfonic acid used as a dye in renal function testing for the detection of nitrates and chlorates, and in the testing of milk.
An antineoplastic agent that is a derivative of progesterone and used to treat advanced breast cancer.
The art or process of comparing photometrically the relative intensities of the light in different parts of the spectrum.
A thioureylene antithyroid agent that inhibits the formation of thyroid hormones by interfering with the incorporation of iodine into tyrosyl residues of thyroglobulin. This is done by interfering with the oxidation of iodide ion and iodotyrosyl groups through inhibition of the peroxidase enzyme.
An increase in the rate of synthesis of an enzyme due to the presence of an inducer which acts to derepress the gene responsible for enzyme synthesis.
A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
Hydrocarbon compounds with one or more of the hydrogens replaced by CHLORINE.
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.
A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
Proteins found in any species of bacterium.
A barbituric acid derivative that acts as a nonselective central nervous system depressant. It potentiates GAMMA-AMINOBUTYRIC ACID action on GABA-A RECEPTORS, and modulates chloride currents through receptor channels. It also inhibits glutamate induced depolarizations.
A multistage process that includes cloning, physical mapping, subcloning, determination of the DNA SEQUENCE, and information analysis.
A trihydroxybenzene or dihydroxy phenol that can be prepared by heating GALLIC ACID.
A genus of gram-negative rods which form exospores and are obligate methanotrophs.
Derivatives and polymers of styrene. They are used in the manufacturing of synthetic rubber, plastics, and resins. Some of the polymers form the skeletal structures for ion exchange resin beads.
An enzyme of the oxidoreductase class that catalyzes the formation of L-TYROSINE, dihydrobiopterin, and water from L-PHENYLALANINE, tetrahydrobiopterin, and oxygen. Deficiency of this enzyme may cause PHENYLKETONURIAS and PHENYLKETONURIA, MATERNAL. EC
A large group of cytochrome P-450 (heme-thiolate) monooxygenases that complex with NAD(P)H-FLAVIN OXIDOREDUCTASE in numerous mixed-function oxidations of aromatic compounds. They catalyze hydroxylation of a broad spectrum of substrates and are important in the metabolism of steroids, drugs, and toxins such as PHENOBARBITAL, carcinogens, and insecticides.
A family of anaerobic METHANOCOCCALES whose organisms are motile by means of flagella. These methanogens use carbon dioxide as an electron acceptor.
A genus of gram-negative, aerobic, spherical cells usually occurring in pairs. The resting stage is considered a cyst. (From Bergey's Manual of Determinative Bacteriology, 9th ed)
Proteins prepared by recombinant DNA technology.
An inhibitor of drug metabolism and CYTOCHROME P-450 ENZYME SYSTEM activity.
A group of 1,2-benzenediols that contain the general formula R-C6H5O2.
The relationships of groups of organisms as reflected by their genetic makeup.
Compounds based on ANTHRACENES which contain two KETONES in any position. Substitutions can be in any position except on the ketone groups.
A carcinogen that is often used in experimental cancer studies.
Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.
Deoxyribonucleic acid that makes up the genetic material of bacteria.
Non-heme iron-containing enzymes that incorporate two atoms of OXYGEN into the substrate. They are important in biosynthesis of FLAVONOIDS; GIBBERELLINS; and HYOSCYAMINE; and for degradation of AROMATIC HYDROCARBONS.
Theoretical representations that simulate the behavior or activity of chemical processes or phenomena; includes the use of mathematical equations, computers, and other electronic equipment.
Cytochromes of the b group that are found bound to cytoplasmic side of ENDOPLASMIC RETICULUM. They serve as electron carrier proteins for a variety of membrane-bound OXYGENASES. They are reduced by the enzyme CYTOCHROME-B(5) REDUCTASE.
The parts of a macromolecule that directly participate in its specific combination with another molecule.

An endocytic pathway essential for renal uptake and activation of the steroid 25-(OH) vitamin D3. (1/263)

Steroid hormones may enter cells by diffusion through the plasma membrane. However, we demonstrate here that some steroid hormones are taken up by receptor-mediated endocytosis of steroid-carrier complexes. We show that 25-(OH) vitamin D3 in complex with its plasma carrier, the vitamin D-binding protein, is filtered through the glomerulus and reabsorbed in the proximal tubules by the endocytic receptor megalin. Endocytosis is required to preserve 25-(OH) vitamin D3 and to deliver to the cells the precursor for generation of 1,25-(OH)2 vitamin D3, a regulator of the calcium metabolism. Megalin-/- mice are unable to retrieve the steroid from the glomerular filtrate and develop vitamin D deficiency and bone disease.  (+info)

Antilithiasic effect of beta-cyclodextrin in LPN hamster: comparison with cholestyramine. (2/263)

Beta-Cyclodextrin (BCD), a cyclic oligosaccharide that binds cholesterol and bile acids in vitro, has been previously shown to be an effective plasma cholesterol lowering agent in hamsters and domestic pigs. This study examined the effects of BCD as compared with cholestyramine on cholesterol and bile acid metabolism in the LPN hamster model model for cholesterol gallstones. The incidence of cholesterol gallstones was 65% in LPN hamsters fed the lithogenic diet, but decreased linearly with increasing amounts of BCD in the diet to be nil at a dose of 10% BCD. In gallbladder bile, cholesterol, phospholipid and chenodeoxycholate concentrations, hydrophobic and lithogenic indices were all significantly decreased by 10% BCD. Increases in bile acid synthesis (+110%), sterol 27-hydroxylase activity (+106%), and biliary cholate secretion (+140%) were also observed, whereas the biliary secretion of chenodeoxycholate decreased (-43%). The fecal output of chenodeoxycholate and cholate (plus derivatives) was increased by +147 and +64%, respectively, suggesting that BCD reduced the chenodeoxycholate intestinal absorption preferentially. Dietary cholestyramine decreased biliary bile acid concentration and secretion, but dramatically increased the fecal excretion of chenodeoxycholate and cholate plus their derivatives (+328 and +1940%, respectively). In contrast to BCD, the resin increased the lithogenic index in bile, induced black gallstones in 34% of hamsters, and stimulated markedly the activities of HMG-CoA reductase (+670%), sterol 27-hydroxylase (+310%), and cholesterol 7alpha-hydroxylase (+390%). Thus, beta-cyclodextrin (BCD) prevented cholesterol gallstone formation by decreasing specifically the reabsorption of chenodeoxycholate, stimulating its biosynthesis and favoring its fecal elimination. BCD had a milder effect on lipid metabolism than cholestyramine and does not predispose animals to black gallstones as cholestyramine does in this animal model.  (+info)

Molecular cloning, characterization, and promoter analysis of the human 25-hydroxyvitamin D3-1alpha-hydroxylase gene. (3/263)

The human 25-hydroxyvitamin D3-1alpha-hydroxylase (1alpha-OHase) gene has been cloned. It contained nine exons and eight introns spanning approximately 6.5 kb and a 1.4-kb 5'-flanking region. The 5'-flanking region contains consensus or highly conserved sequences for TATA, Pu, and CCAAT boxes, four cAMP response elements, two activator protein-1 (AP-1) response elements, two AP-2 response elements, three specific protein-1 (Sp1) response elements, and four NF-kappaB binding sites, but no vitamin D response element. By using luciferase reporter gene constructs of truncated forms of the 1alpha-OHase promoter transfected into a modified pig kidney cell line, AOK-B50, we identified regulatory regions of the 1.4-kb 1alpha-OHase promoter for parathyroid hormone 1-34 [PTH(1-34)], forskolin, and 1,25-hydroxyvitamin D3 [1,25(OH)2D3]. The 1.4-kb 1alpha-OHase promoter (AN1) modestly (1.7-fold) induced luciferase activity, whereas 1,100- (AN2), 827- (AN3), 672- (AN4), 463-(AN5), and 363-bp (AN6)-truncated promoters greatly stimulated luciferase activity by 494-fold, 18.4-fold, 55.3-fold, 643-fold, and 56.4-fold, respectively. PTH(1-34) and forskolin stimulated the activity of all constructs to varying degrees with significantly greater responsiveness for both compounds on AN2 and AN5. 1,25(OH)2D3 suppressed PTH(1-34)-induced activity on AN2 and AN5 constructs by 58% and 52%, respectively, but had no effect on the other constructs. These studies characterize the regulatory regions of the human 1alpha-OHase gene and provide insight into the physiologic basis for regulation of the expression of this gene by PTH and 1,25(OH)2D3.  (+info)

Lipoprotein cholesterol uptake mediates up-regulation of bile-acid synthesis by increasing cholesterol 7alpha-hydroxylase but not sterol 27-hydroxylase gene expression in cultured rat hepatocytes. (4/263)

Lipoproteins may supply substrate for the formation of bile acids, and the amount of hepatic cholesterol can regulate bile-acid synthesis and increase cholesterol 7alpha-hydroxylase expression. However, the effect of lipoprotein cholesterol on sterol 27-hydroxylase expression and the role of different lipoproteins in regulating both enzymes are not well established. We studied the effect of different rabbit lipoproteins on cholesterol 7alpha-hydroxylase and sterol 27-hydroxylase in cultured rat hepatocytes. beta-Migrating very-low-density lipoprotein (betaVLDL) and intermediate-density lipoprotein (IDL) caused a significant increase in the intracellular cholesteryl ester content of cells (2. 3- and 2-fold, respectively) at a concentration of 200 microgram of cholesterol/ml, whereas high-density lipoprotein (HDL, 50% v/v), containing no apolipoprotein E (apo E), showed no effect after a 24-h incubation. betaVLDL and IDL increased bile-acid synthesis (1. 9- and 1.6-fold, respectively) by up-regulation of cholesterol 7alpha-hydroxylase activity (1.7- and 1.5-fold, respectively). Dose- and time-dependent changes in cholesterol 7alpha-hydroxylase mRNA levels and gene expression underlie the increase in enzyme activity. Incubation of cells with HDL showed no effect. Sterol 27-hydroxylase gene expression was not affected by any of the lipoproteins added. Transient-expression experiments in hepatocytes, transfected with a promoter-reporter construct containing the proximal 348 nucleotides of the rat cholesterol 7alpha-hydroxylase promoter, showed an enhanced gene transcription (2-fold) with betaVLDL, indicating that a sequence important for a cholesterol-induced transcriptional response is located in this part of the cholesterol 7alpha-hydroxylase gene. The extent of stimulation of cholesterol 7alpha-hydroxylase is associated with the apo E content of the lipoprotein particle, which is important in the uptake of lipoprotein cholesterol. We conclude that physiological concentrations of cholesterol in apo E-containing lipoproteins increase bile-acid synthesis by stimulating cholesterol 7alpha-hydroxylase gene transcription, whereas HDL has no effect and sterol 27-hydroxylase is not affected.  (+info)

Mutations producing premature termination of translation and an amino acid substitution in the sterol 27-hydroxylase gene cause cerebrotendinous xanthomatosis associated with parkinsonism. (5/263)

OBJECTIVES: Mutational analysis of the sterol 27-hydroxylase (CYP27) gene was performed on three patients from two Japanese families who had cerebrotendinous xanthomatosis (CTX) associated with parkinsonism. METHODS: Clinical evaluations, brain MRI studies, and laboratory analyses were completed on the three patients. The CYP27 gene was analysed for mutations by PCR amplification of gene segments followed by direct sequencing. RESULTS: Two different, homozygous mutations were identified in these families. One is a novel transition, substituting T for G at Glu162 (GAG) resulting in a stop codon (TAG). The other is also a transition, substituting T for C at Arg441 (CGG) resulting in Trp (TGG). The second is located in two amino acids ahead of the heme ligand binding site (Cys443) of the protein likely rendering it non-functional. It is the most common CTX mutation in Japanese patients. CONCLUSIONS: CTX with parkinsonism is caused by mutations with a severe impact on enzyme function. The two mutations described here are likely to cause loss of function because they are chain terminating or affect an essential site in the protein.  (+info)

Elimination of cholesterol as cholestenoic acid in human lung by sterol 27-hydroxylase: evidence that most of this steroid in the circulation is of pulmonary origin. (6/263)

Human alveolar macrophages have exceptionally high capacity to convert cholesterol into 27-hydroxycholesterol and cholestenoic acid by the sterol 27-hydroxylase mechanism. It is shown here that the human lung has a higher content of 27-hydroxycholesterol relative to cholesterol than any other organ. In order to evaluate the importance of the sterol 27-hydroxylase mechanism for cholesterol homeostasis in the lung, the production of cholestenoic acid by human lung was investigated. Removal of one lung reduced the level of cholestenoic acid in the circulation by 48 +/- 4% (P < 0.005). The levels of cholestenoic acid in the pulmonary artery and in the pulmonary vein showed significant differences (P < 0.002) with higher levels in the pulmonary vein (108 +/- 16 and 104 +/- 16 ng/mL, respectively). This corresponds to a net flux of cholestenoic acid from the lung of about 14 mg/day, which is more than 80% of the reported removal of this oxysterol and its metabolites from the circulation by the liver per day. Bypassing the lung for 60 min led to a reduction in circulating cholestenoic acid (30%) that fits with a pulmonary origin when taking into account the half-life of cholestenoic acid. The level of circulating cholestenoic acid was found to be less in patients with different lung diseases. It is evident that most of the cholestenoic acid in the circulation is of pulmonary origin. The present results suggest that the sterol 27-hydroxylase in the lung is responsible for at least half of the total flux of 27-oxygenated cholesterol metabolites to the liver and that this enzyme system may be of importance for cholesterol homeostasis in the lung.  (+info)

Bile acid synthesis in the Smith-Lemli-Opitz syndrome: effects of dehydrocholesterols on cholesterol 7alpha-hydroxylase and 27-hydroxylase activities in rat liver. (7/263)

The Smith-Lemli-Opitz syndrome (SLOS) is a congenital birth defect syndrome caused by a deficiency of 3beta-hydroxysterol Delta(7)-reductase, the final enzyme in the cholesterol biosynthetic pathway. The patients have reduced plasma and tissue cholesterol concentrations with the accumulation of 7-dehydrocholesterol and 8-dehydrocholesterol. Bile acid synthesis is reduced and unnatural cholenoic and cholestenoic acids have been identified in some SLOS patients. To explore the mechanism of the abnormal bile acid production, the activities of key enzymes in classic and alternative bile acid biosynthetic pathways (microsomal cholesterol 7alpha-hydroxylase and mitochondrial sterol 27-hydroxylase) were measured in liver biopsy specimens from two mildly affected SLOS patients. The effects of 7- and 8-dehydrocholesterols on these two enzyme activities were studied by using liver from SLOS model rats that were treated with the Delta(7)-reductase inhibitor (BM15.766) for 4 months and were comparable with more severe SLOS phenotype in plasma and hepatic sterol compositions. In the SLOS patients, cholesterol 7alpha-hydroxylase and sterol 27-hydroxylase were not defective. In BM15.766-treated rats, both enzyme activities were lower than those in control rats and they were competitively inhibited by 7- and 8-dehydrocholesterols. Rat microsomal cholesterol 7alpha-hydroxylase did not transform 7-dehydrocholesterol or 8-dehydrocholesterol into 7alpha-hydroxylated sterols. In contrast, rat mitochondrial sterol 27-hydroxylase catalyzed 27-hydroxylation of 7- and 8-dehydrocholesterols, which were partially converted to 3beta-hydroxycholestadienoic acids. Addition of microsomes to the mitochondrial 27-hydroxylase assay mixture reduced 27-hydroxydehydrocholesterol concentrations, which suggested that 27-hydroxydehydrocholesterols were further metabolized by microsomal enzymes. These results suggest that reduced normal bile acid production is characteristic of severe SLOS phenotype and is caused not only by depletion of hepatic cholesterol but also by competitive inhibition of cholesterol 7alpha-hydroxylase and sterol 27-hydroxylase activities by accumulated 7- and 8-dehydrocholesterols. Unnatural bile acids are synthesized mainly by the alternative pathway via mitochondrial sterol 27-hydroxylase in SLOS.  (+info)

Spinal xanthomatosis: a variant of cerebrotendinous xanthomatosis. (8/263)

We describe seven Dutch patients from six families with a slowly progressive, mainly spinal cord syndrome that remained for many years the sole expression of cerebrotendinous xanthomatosis (CTX). MRI demonstrated white matter abnormalities in the lateral and dorsal columns of the spinal cord. Post-mortem examination of one of the patients showed extensive myelin loss in these columns. An array of genotypes was found in these patients. We conclude that 'spinal xanthomatosis' is a clinical and radiological separate entity of CTX that should be included in the differential diagnosis of 'chronic myelopathy'.  (+info)

The mitochondrial sterol 27-hydroxylase (CYP27A1) is a multifunctional cytochrome P450 enzyme that catalyses important hydroxylations in the biosynthesis of bile acids and bioactivation of vitamin D3. Previous results [Babiker, Andersson, Lund, Xiu, Deeb, Reshef, Leitersdorf, Diczfalusy and Björkhem (1997) J. Biol. Chem. 272, 26253-26261] suggest that CYP27A1 plays an important role in cholesterol homoeostasis and affects atherogenesis. In the present study, the regulation of the human CYP27A1 gene by growth hormone (GH), insulin-like growth factor-1 (IGF-1), dexamethasone, thyroid hormones and PMA was studied. HepG2 cells were transfected transiently with luciferase reporter gene constructs containing DNA fragments flanking the 5′-region of the human CYP27A1 gene. GH, IGF-1 and dexamethasone increased the promoter activity by 2-3-fold, whereas thyroxine (T4) and PMA repressed the activity significantly when measured with luciferase activity expressed in the cells. The endogenous CYP27A1 ...
We investigated the effect of increasing dietary cholesterol on bile acid pool sizes and the regulation of the two bile acid synthetic pathways (classic, via cholesterol 7α-hydroxylase, and alternative, via sterol 27-hydroxylase) in New Zealand white rabbits fed 3 g cholesterol/per day for up to 15 days. Feeding cholesterol for one day increased hepatic cholesterol 75% and cholesterol 7α-hydroxylase activity 1.6 times without significant change of bile acid pool size or sterol 27-hydroxylase activity. After three days of cholesterol feeding, the bile acid pool size increased 83% (P < 0.01), and further feeding produced 10%-20% increments, whereas cholesterol 7α-hydroxylase activity declined progressively to 60% below baseline. In contrast, sterol 27-hydroxylase activity rose 58% after three days of cholesterol feeding and remained elevated with continued intake. Bile drainage depleted the bile acid pool and stimulated downregulated cholesterol 7α-hydroxylase activity but did not affect ...
The oxysterol profile of the retina suggests that all known pathways of cholesterol elimination in extraocular organs are operative in the retina and that they likely vary depending on specific cell type. However, overall oxidation to 5-cholestenoic acid appears to be the predominant mechanism for c …
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This gene encodes a small iron-sulfur protein that transfers electrons from NADPH through ferredoxin reductase to mitochondrial cytochrome P450, involved in steroid, vitamin D, and bile acid metabolism. Pseudogenes of this functional gene are found on chromosomes 20 and 21. [provided by RefSeq, Aug 2011 ...
Cerebrotendinous xanthomatosis (CTX) is a rare, disabling genetic disorder in which cholestanol and cholesterol accumulate in the nervous system and other tissues. It has an autosomal recessive mode...
Get natural cures for Cerebrotendinous Xanthomatosis that can make a difference in your life or the life of someone you love with alternative treatments.
The biosynthesis of steroid hormones is an integral component of insect growth, development and reproduction. Although there is an abundance of biochemical data implicating both microsomal and mitochondrial cytochrome P450s in steroid metabolism, molecular genetic information on mitochondrial P450s is almost entirely limited to vertebrate sequences. In the current study, a degenerate polymerase chain reaction (PCR) primer was targeted to the highly conserved region of P450 genes that encodes the heme-binding decapeptide. Using a 5 rapid amplification of cDNA ends (RACE) approach, seven novel cytochrome P450 genes were isolated from Drosophila acanthoptera, including one sequence (CYP12B1) with high regional homology to vertebrate mitochondrial P450s. Sequence analysis of the conceptual translation of the full length gene, obtained by 5RACE, revealed an amphipathic NH2-terminus rich in basic and hydrophilic amino acids, a characteristic feature of mitochondrial P450s that distinguishes them ...
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The IUPHAR/BPS Guide to Pharmacology. 7α-hydroxycholesterol ligand page. Quantitative data and detailed annnotation of the targets of licensed and experimental drugs.
Seven patients with cerebrotendinous xanthomatosis (CTX) were studied by electrophysiological techniques. The percentages of abnormalities detected in nerve conduction studies and electroencephalograms were 28.6% (two patients) and 100%, respectively. All patients showed prolonged central conduction times in short latency somatosensory evoked potentials (SSEPs) by tibial nerve stimulation but normal SSEPs by median nerve stimulation. Brain stem auditory evoked potentials and visual evoked potentials were abnormal in three (42.9%) and four patients (57.1%), respectively. These electrophysiological parameters were correlated with the ratio of serum cholestanol to cholesterol concentration. The results of SSEPs suggest that the polyneuropathy in CTX is caused by distal axonopathy affecting longer axons before shorter axons (central-peripheral distal axonopathy).. ...
Cerebrotendineous xanthomatosis (or cerebrotendinous xanthomatosis, with one fewer e, or Van Bogaert-Scherer-Epstein syndrome, or cerebrotendinous cholesterosis) is a form of xanthomatosis associated with the CYP27A1 gene on chromosome 2. An inherited disorder associated with the deposition of a form of cholesterol in the brain and other tissues and with elevated levels of cholesterol in plasma but with normal total cholesterol level; it is characterized by progressive cerebellar ataxia beginning after puberty and by juvenile cataracts, and tendineous or tuberous xanthomas. ...
27-Hydroxycholesterol (27-HC), also known as (25R)-cholest-5-ene-3β,26-diol or by its conventional name 26-hydroxycholesterol, is an oxygenated derivative of cholesterol and a major oxysterol in circulation (PMID: 7749852 ). 27-Hydroxycholesterol is the product of the enzyme sterol 27-hydroxylase. The enzyme is critical for the degradation of the steroid side-chain and a genetic deficiency of the enzyme leads to reduced formation of bile acids in humans. There is a correlation between 27-hydroxycholesterol and cholesterol in the circulation, and females have lower levels of 27-hydroxycholesterol than males. A strong correlation is observed between circulating levels of 27-hydroxycholesterol and cholesterol, in both healthy subjects and subjects with hypercholesterolemia and documented atherosclerosis. 27-Hydroxycholesterol is metabolized by an oxysterol 7alpha-hydroxylase in the liver. Changes in the activity of this enzyme may lead to the accumulation of 27-hydroxycholesterol in the ...
Looking for normal cholesteremic xanthomatosis? Find out information about normal cholesteremic xanthomatosis. A childhood syndrome characterized by exopthalmos, diabetes insipidus, and softened or punched-out areas in the bones Explanation of normal cholesteremic xanthomatosis
proteopedia link. proteopedia link. Israel Hanukoglu, Ph.D. Professor of Biochemistry and Molecular Biology Faculty of Natural Sciences, Ariel University, Ariel, Israel Fields of expertise: Epithelial sodium channel, steroidogenic enzymes, keratin and intermediate filament structure, mitochondrial cytochromes P450 Personal web site: ...
Aldosterone Synthase: A mitochondrial cytochrome P450 enzyme that catalyzes the 18-hydroxylation of steroids in the presence of molecular oxygen and NADPH-specific flavoprotein. This enzyme, encoded by CYP11B2 gene, is important in the conversion of CORTICOSTERONE to 18-hydroxycorticosterone and the subsequent conversion to ALDOSTERONE.
Eruptive xanthomatosis is a skin condition that causes small yellow-red bumps to appear on the body. It can occur in people who have very high blood fats (lipids). These patients also frequently have diabetes.
Catalyzes the first step in the oxidation of the side chain of sterol intermediates; the 27-hydroxylation of 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol. Has also a vitamin D3-25-hydroxylase activity.
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The cholestanol content of a cataractous lens nucleus from a patient with cerebrotendinous xanthomatosis (CTX) was quantified by gas chromatography-mass spectrometry and found to be 0.27 micrograms per mg freeze-dried lens tissue. The cholestanol-cholesterol ratio of 1.7% in the lens nucleus was similar to that in the serum of the CTX patient. The cholestanol content and cholestanol-cholesterol ratio in the CTX lens were approximately four-fold and six-fold greater respectively than the mean levels found in three senile cataractous lens nuclei analysed simultaneously for comparative purposes. ...
The research team, which included scientists from the University of Medicine and Dentistry of New Jersey and Stanford University, noted after vitamin D binds to the receptor, also blocks a protein called NFAT needed to turn on the gene. Without the gene on, levels of IL-17 fall. Vitamin D also does another job by turning on a gene that produces suppressive T cells to fight destructive IL-17-production that occurs elsewhere.. According to the authors, in mouse models with EAE, vitamin D, 1,25-dihydroxyvitamin D3 [1,25(OH)2D3] diminishes paralysis and progression of the disease and reduces IL-17A-secreting CD4+ T cells in the periphery and central nervous system (CNS).. Identification of the vitamin D pathway could lead to new treatments not only for multiple sclerosis, but for other autoimmune diseases include type 1 diabetes, rheumatoid arthritis and skin disorders. The authors concluded, Our results describe novel mechanisms and new concepts with regard to vitamin D and the immune system ...
Egg-shell calcium (Ca) is one of the effective Ca sources for bone metabolism. In the present study, we investigated whether egg-shell Ca had similar effects compared with calcium carbonate (CaCO3) when vitamin D3 (1alpha(OH)D3) treatment was given to an osteoporotic rat model. In both 1alpha(OH)D3- …
The origin, development, and characteristics of two types of testicular macrophage have been described. To elucidate the nature of these immune cells, the researchers used a novel cell tracing method. Their findings are of fundamental importance. They may help understand certain kinds of infertility in men and find new treatments for them. Read more ...
Encyclopedia article on this inherited disorder associated with the deposition of a form of cholesterol (cholestanol) in the brain and other tissues. ...
The body does make its own vitamin D-3. Vitamin D-3, also called cholecalciferol, is made in the skin of human beings from a compound called...
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Bile acid synthesis defects may manifest in the neonatal period or later. This panel is specifically designed to cover the genes which lead to neonatal or infantile onset of symptoms. The Jaundice NGS Panel is an option for those cases which present with jaundice and an unclear cause.
Biology of Reproduction contains original scientific research on a broad range of topics in the field of reproductive biology, as well as minireviews.
TY - JOUR. T1 - Using components of the vitamin D pathway to prevent and treat colon cancer. AU - Stubbins, Renee E.. AU - Hakeem, Aisha. AU - Núñez, Nomelí P.. PY - 2012/12. Y1 - 2012/12. N2 - The objective of this review was to analyze the components of vitamin D and their potential usefulness in preventing and treating colorectal cancer. The active form of vitamin D, 1α,25(OH2)D3, targets the wnt/β-catenin pathway by upregulating key tumor suppressor genes such as E-cadherin, which promotes an epithelial phenotype, but this is only possible when the vitamin D receptor (VDR) is present. Colorectal cell lines have shown that VDR expression levels decrease in the later stages of colon cancer. In colorectal cancers with low VDR expression, treatments to increase VDR expression could target alterations at the genomic and epigenomic levels by modulating transcription factors such as SNAIL1 and by utilizing histone deacetyltransferase inhibitors, respectively. Finally, epidemiological studies ...
TY - JOUR. T1 - Cloning and regulation of cholesterol 7α-hydroxylase, the rate-limiting enzyme in bile acid biosynthesis. AU - Jelinek, D. F.. AU - Andersson, S.. AU - Slaughter, C. A.. AU - Russell, D. W.. N1 - Copyright: Copyright 2007 Elsevier B.V., All rights reserved.. PY - 1990. Y1 - 1990. N2 - The rate-limiting step in bile acid biosynthesis is catalyzed by the microsomal cytochrome P-450 cholesterol 7α-hydroxylase (7α-hydroxylase). The expression of this enzyme is subject to feedback regulation by sterols and is thought to be coordinately regulated with enzymes in the cholesterol supply pathways, including the low density lipoprotein receptor and 3-hydroxy-3-methylglutaryl-coenzyme A reductase and synthase. Here we report the purification of rat 7α-hydroxylase and the determination of a partial amino acid sequence. Oligonucleotides derived from peptide sequence were used to clone a full-length cDNA encoding 7α-hydroxylase. DNA sequence analysis of the cDNA revealed a 7α-hydroxylase ...
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27-Hydroxylase deficiency (cerebrotendinous xanthomatosis (CTX)) is a rare familial sterol storage disease with accumulation of cholestanol and cholesterol in most tissues, and in particular in xanthomas, bile, and brain. Clinically, this disorder is characterized by dementia, spinal cord paresis, cerebellar ataxia, tuberous and tendon xanthomas, early atherosclerosis, and cataracts.. More than 20 different mutations have been defined in the sterol 27-hydroxylase gene of CTX patients. The defect leads to a block in bile acid biosynthesis, with accumulation of substrates for the mitochondrial 27-hydroxylase such as 5β-cholestane-3α,7α,12α-triol and 7α-hydroxy-4-cholestene-3-one. The former metabolite is metabolized into 5β-cholestane-3α,7α,12α,25-tetrol, 5β-cholestane-3α,7α,12α,23-tetrol, and 5β-cholestane-3α,7α,12α,24,25-pentol.. These bile alcohols are excreted in gram amounts in bile and feces. At least part of the excess cholestanol in patients with CTX is formed from the ...
SNP in the vitamin D receptor (VDR) gene is associated with risk of lower respiratory infections. The influence of genetic variation in the vitamin D pathway resulting in susceptibility to upper respiratory infections (URI) has not been investigated. We evaluated the influence of thirty-three SNP in eleven vitamin D pathway genes (DBP, DHCR7, RXRA, CYP2R1, CYP27B1, CYP24A1, CYP3A4, CYP27A1, LRP2, CUBN and VDR) resulting in URI risk in 725 adults in London, UK, using an additive model with adjustment for potential confounders and correction for multiple comparisons. Significant associations in this cohort were investigated in a validation cohort of 737 children in Manchester, UK. In all, three SNP in VDR (rs4334089, rs11568820 and rs7970314) and one SNP in CYP3A4 (rs2740574) were associated with risk of URI in the discovery cohort after adjusting for potential confounders and correcting for multiple comparisons (adjusted incidence rate ratio per additional minor allele ≥1·15, Pfor trend ...
7 alpha,26-Dihydroxy-4-cholesten-3-one is involved in primary bile acid biosynthesis. 7 alpha,26-Dihydroxy-4-cholesten-3-one is produced from 7 alpha,27-Dihydroxycholesterol through the action of HSD3B7 (EC: 7 alpha,26-Dihydroxy-4-cholesten-3-one can then be converted to 7 alpha-Hydroxy-3-oxo-4-cholestenoate by CYP27A (EC: ...
Information on Congenital bile acid synthesis defect, type 1, which may include symptoms, causes, inheritance, treatments, orphan drugs, associated orgs, and other relevant data.
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SWISS-MODEL Template Library (SMTL) entry for 3k9y.1. Crystal structure of rat mitochondrial P450 24A1 S57D in complex with CYMAL-5
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Common name: S-(L-glutamyl)-[peptidyl-carrier protein]-3-hydroxylase (3-hydroxylating, erythro-hydroxy-L-glutamyl-[peptidyl-carrier-protein]-forming ...
Cholesterol is among one of the most decorated molecules in biology today, the study of which produced not only a number of Nobel Prizes but also key findings that influence our study of physiology and medicine today. It is the single starting substrate of the steroidogenic pathway, the synthesis of steroid hormones such as progesterone, testosterone, and corticosterone, which control a variety of essential physiological and metabolic functions including salt and water balance, spermatogenesis, follicular development and maintenance of pregnancy. Steroidogenesis is a unique process in which all of the steroid hormones are derived from a single substrate, cholesterol, by the CYP11A1 enzyme located on the matrix side of the inner mitochondrial membrane. Steroidogenesis is rapidly triggered in response to induction by tropic hormones such as ACTH or LH binding to their respective G-protein coupled receptors on the plasma membrane, triggering a rise in intracellular cAMP. This process, however, ...
Looking for online definition of 17alpha-hydroxylase in the Medical Dictionary? 17alpha-hydroxylase explanation free. What is 17alpha-hydroxylase? Meaning of 17alpha-hydroxylase medical term. What does 17alpha-hydroxylase mean?
Create a standard curve by reducing the data using computer software capable of generating a four parameter logistic (4-PL) curve-fit. As an alternative, construct a standard curve by plotting the mean absorbance for each standard on the x-axis against the concentration on the y-axis and draw a best fit curve through the points on the graph. The data may be linearized by plotting the log of the SREBF1 concentrations versus the log of the O.D. and the best fit line can be determined by regression analysis. This procedure will produce an adequate but less precise fit of the data ...
pan-frying with olive oil, pan-frying with soya oil, and roasting. Roasting did not modify the fat content from that of raw samples. Frying increased the fat content 2-fold, with no difference between samples fried with different oils. Total cholesterol oxidized products (COPs) were 0.74, 2.98, 3.35, and 7.38 μg/g fat in raw, fried with olive oil, fried with soya oil, and roasted salmon, respectively, which represent 0.01, 0.08, 0.09, and 0.15% of cholesterol. A significant correlation (r = 0.902, ≤ 0.01) was found between acidity index and total COPs. The most abundant COPs were 7-ketocholesterol, which appeared in all the samples, and cholestanetriol (one of the most citotoxic COP), which appeared only in cooked samples (1.05−1.33 μg/g fat). All cooked samples supplied more ω-6 polyunsaturated fatty acids (PUFAs) than raw samples and showed higher ω-6/ω-3 ratios. Roasted salmon showed the lowest ω-3 content and the highest PUFAs/(SFAs)-C18:0 and MUFAs+PUFAs/(SFAs-C18:0) ratios. ...
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Low levels of vitamin D-3 affect more than half the population and about 40 percent of the population has low levels of B-12. Not getting enough of these vital nutrients can have devastating effects ...
The typical daily dosage of vitamin D-3 from food intake alone is 204 to 288 international units depending on gender and life stage, according to the National Institutes of Health. The recommended...
7 alpha-hydroxy-4-cholesten-3-one 12-alpha-hydroxylase7-alpha-hydroxy-4-cholesten-3-one 12-alpha-hydroxylase7-alpha-hydroxycholest-4-en-3-one 12-alpha-hydroxylaseCYPVIIIB1cytochrome P450 8B1cytochrome P450, subfamily VIIIB (sterol 12-alpha- ...
The price per single test (either LPN1 or LPN2) on a single sample is $65; however, the price for both tests on a single sample requested at the same time is $100. The expected turnaround time is 3 - 4 weeks. If a sample has already been submitted for the LPN1 test (August 2014 and earlier), and the result has been reported, the LPN2 test can be performed on this same sample for $40 USD provided that an updated health profile is provided. For instructions on submitting a request for this reduced fee testing option, please contact [email protected] Requests sent to the VDL will be charged at $65 ...
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... calcidiol 1-monooxygenase EC trans-cinnamate 2-monooxygenase EC cholestanetriol 26-monooxygenase EC ... alpha-pinene monooxygenase EC 1,8-cineole 2-endo-monooxygenase EC 1,8-cineole 2-exo-monooxygenase EC ... camphor 5-monooxygenase EC camphor 1,2-monooxygenase EC alkane 1-monooxygenase EC steroid 11b- ... menthol monooxygenase EC (S)-limonene 3-monooxygenase EC (S)-limonene 6-monooxygenase EC (S ...
In enzymology, a cholestanetriol 26-monooxygenase (EC is an enzyme that catalyzes the chemical reaction 5beta- ... cholestanetriol 26-hydroxylase, sterol 27-hydroxylase, sterol 26-hydroxylase, cholesterol 27-hydroxylase, CYP27A, CYP27A1, and ... Isolation of a cytochrome P-450 from rabbit liver mitochondria catalyzing 26-hydroxylation of C27-steroids". J. Biol. Chem. 259 ... The systematic name of this enzyme class is 5beta-cholestane-3alpha,7alpha,12alpha-triol,NADPH:oxygen oxidoreductase (26- ...
In enzymology, a cholestanetriol 26-monooxygenase (EC is an enzyme that catalyzes the chemical reaction 5beta- ... cholestanetriol 26-hydroxylase, sterol 27-hydroxylase, sterol 26-hydroxylase, cholesterol 27-hydroxylase, CYP27A, CYP27A1, and ... Isolation of a cytochrome P-450 from rabbit liver mitochondria catalyzing 26-hydroxylation of C27-steroids". J. Biol. Chem. 259 ... The systematic name of this enzyme class is 5beta-cholestane-3alpha,7alpha,12alpha-triol,NADPH:oxygen oxidoreductase (26- ...
Cholestanetriol 26-Monooxygenase * Chromatography, High Pressure Liquid * Clone Cells * Gene Expression Regulation, Enzymologic ...
Cholestanetriol 26-Monooxygenase * Cholesterol Side-Chain Cleavage Enzyme Grant support * AG024336/AG/NIA NIH HHS/United States ...
... cholestanetriol 26-monooxygenase; cyt; Cytochrome P-450C27/25; Cytochrome P450 27; cytochrome P450, family 27, subfamily A, ... Protein Aliases: 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol 26-hydroxylase; 5-beta-cholestane-3-alpha, 7-alpha, 12- ... alpha-triol 27-hydroxylase; 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 26-hydroxylase; 5-beta-cholestane-3-alpha,7-alpha, ... Sterol 26-hydroxylase, mitochondrial; Sterol 27-hydroxylase; Vitamin D(3) 25-hydroxylase ...
Involved in monooxygenase activity. Specific Function. Catalyzes the first step in the oxidation of the side chain of sterol ... cholestanetriol 26-monooxygenase activity. cholesterol 26-hydroxylase activity. vitamin D3 25-hydroxylase activity. ... 7 alpha,26-Dihydroxy-4-cholesten-3-one + Oxygen + NADPH + Hydrogen Ion → 7alpha-Hydroxy-3-oxo-4-cholestenoate + NADP + Water. ... Showing Protein Sterol 26-hydroxylase, mitochondrial (HMDBP01037). IdentificationBiological propertiesGene propertiesProtein ...
Common name: cholestanetriol 26-monooxygenase. Reaction: 5β-cholestane-3α,7α,12α-triol + NADPH + H+ + O2 = (25R)-5β-cholestane- ... Other name(s): 24-hydroxycholesterol 7α-monooxygenase; CYP39A1; CYP39A1 oxysterol 7α-hydroxylase. Systematic name: (24R)- ... Other name(s): cholesterol 24-monooxygenase; CYP46; CYP46A1; cholesterol 24S-hydroxylase; cytochrome P450 46A1. Systematic name ... Other name(s): 25-hydroxycholesterol 7α-monooxygenase; CYP7B1; CYP7B1 oxysterol 7α-hydroxylase. Systematic name: cholest-5-ene- ...
cholestanetriol 26-monooxygenase [EC:]. K08762 diazepam-binding inhibitor (GABA receptor modulator, acyl-CoA-binding ... cholesterol 7alpha-monooxygenase [EC:]. K07431 sterol 12-alpha-hydroxylase [EC:]. ...
keywords = "25-Hydroxyvitamin D3 1-alpha-Hydroxylase, Administration, Oral, Adult, Biopsy, Cholecalciferol, Cholestanetriol 26- ... Monooxygenase, Female, Humans, Male, Middle Aged, Psoriasis, RNA, Messenger, Real-Time Polymerase Chain Reaction, Skin, ...
... calcidiol 1-monooxygenase EC trans-cinnamate 2-monooxygenase EC cholestanetriol 26-monooxygenase EC ... alpha-pinene monooxygenase EC 1,8-cineole 2-endo-monooxygenase EC 1,8-cineole 2-exo-monooxygenase EC ... camphor 5-monooxygenase EC camphor 1,2-monooxygenase EC alkane 1-monooxygenase EC steroid 11b- ... menthol monooxygenase EC (S)-limonene 3-monooxygenase EC (S)-limonene 6-monooxygenase EC (S ...
enzyme was re - named into 27-monooxygenase because it hydroxylates the methyl group in position 27 [7]) [7] 5b-cholestane-3a, ... Cholestanetriol 26-monooxygenase Reaction type oxidation redox reaction reduction Natural substrates and products S ... S.; Hoshita, N.; Okuda, K.: Enzymatic characteristics of CO-sen - sitive 26-hydroxylase system for 5b-cholestane-3a,7a,12a- ...
Cholestanetriol 26-Monooxygenase / genetics* Actions. * Search in PubMed * Search in MeSH * Add to Search ...
Cholestanetriol 26-Monooxygenase / genetics* Actions. * Search in PubMed * Search in MeSH * Add to Search ...
Cholestanetriol 26-monooxygenase (substance). Code System Preferred Concept Name. Cholestanetriol 26-monooxygenase (substance) ...
The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of ... cholestanetriol 26-monooxygenase , cytochrome P-450C27/25 , cytochrome P450 27 , cytochrome P450, subfamily XXVIIA (steroid 27- ... sterol 26-hydroxylase, mitochondrial (CYP27A1) antibody * cytochrome P450, family 27, subfamily A, polypeptide 1 (cyp27a1) ... 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol 26-hydroxylase , 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol 27- ...
The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of ... cholestanetriol 26-monooxygenase,cytochrome P-450C27/25,cytochrome P450, subfamily XXVIIA (steroid 27-hydroxylase, cerebrot ... 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol 26-hydroxylase,5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol 27- ...
Cholestanetriol 26-Monooxygenase. D10 - Lipids. Isobutyric Acids. Isobutyrates. D12 - Amino Acids, Peptides, and Proteins. ...
Cholestanetriol 26-Monooxygenase. Cholesterol. Cholesterol Side-Chain Cleavage Enzyme. Chromans. Chromatin. Chromatin ...
Define Cholerine. Cholerine synonyms, Cholerine pronunciation, Cholerine translation, English dictionary definition of Cholerine. n. 1. The precursory symptoms of cholera
Cholestanetriol 26-Monooxygenase/antagonists & inhibitors. *Cholestanetriol 26-Monooxygenase/metabolism. *Disease Models, ...
TY - JOUR. T1 - Mutations in the bile acid biosynthetic enzyme sterol 27-hydroxylase underlie cerebrotendinous xanthomatosis. AU - Cali, James J.. AU - Hsieh, Chih Lin. AU - Francke, Uta. AU - Russell, David W.. PY - 1991/4/25. Y1 - 1991/4/25. N2 - The sterol storage disorder cerebrotendinous xanthomatosis (CTX) is characterized by abnormal deposition of cholesterol and cholestanol in multiple tissues. Deposition in the central nervous system leads to neurological dysfunction marked by dementia, spinal cord paresis, and cerebellar ataxia. Deposition in other tissues causes tendon xanthomas, premature atherosclerosis, and cataracts. In two unrelated patients with CTX, we have identified different point mutations in the gene (CYP27) encoding sterol 27-hydroxylase, a key enzyme in the bile acid biosynthesis pathway. Transfection of mutant cDNAs into cultured cells results in the synthesis of immunoreactive sterol 27-hydroxylase protein with greatly diminished enzyme activity. We have localized the ...
Rafelski, J., Greiner, W. & Fulcher, L. P., Jan 1 1973, In : Il Nuovo Cimento B Series 11. 13, 1, p. 135-160 26 p.. Research ...
Cholestanetriol 26-Monooxygenase * ATP Binding Cassette Transporter, Subfamily B, Member 11 * constitutive androstane receptor ...
Graham, A., Borthwick, F. & Taylor, J. M. W., 2014, Cholesterol Transporters of the START Domain Protein Family in Health and Disease. Clark, B. & Stocco, D. (eds.). Springer, p. 99-117 19 p.. Research output: Chapter in Book/Report/Conference proceeding › Chapter (peer-reviewed) ...
Cholestanetriol 26-Monooxygenase. Grant support. *P30 DK034987/DK/NIDDK NIH HHS/United States ...
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The activity is part of EC, cholestanetriol 26-monooxygenase (EC created 1976, deleted 2012)]. EC ... In plants, this reaction is catalysed by EC (choline monooxygenase), whereas in animals and many bacteria it is ... EC Deleted entry: 3α,7α,12α-trihydroxycholestan-26-al 26-oxidoreductase. ...
Camphor 5-Monooxygenase D8.244.453.85 D8.244.453.12 D8.811.682.690.708.170.85 D8.811.682.690.708.170.12 D12.776.422.220.453.85 ... Cholestanetriol 26-Monooxygenase D8.244.453.499.750 D8.811.682.690.708.170.493.750 D12.776.422.220.453.499.750 Cholestanol ... G2.111.26 G2.149.115.26 G12.122.100 G12.425.143.100 Aging G7.700.320.124 G7.345.124 Agrin D12.776.641.50 D12.776.631.50 Air ...
Cholestanetriol 26-Monooxygenase D8.811.682.690.708.783.150 Cholesterol D10.851.208 Cholesterol 7-alpha-Hydroxylase D8.811. ...
D12.776.543.585.450.437.249 Cholestanetriol 26-Monooxygenase D12.776.422.220.453.915.250 D12.776.422.220.453.915.150 ...
Adult, Body Mass Index, Cholestanetriol 26-Monooxygenase, Cytochrome P450 Family 2, Female, Genetic Predisposition to Disease, ...
  • The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. (
  • Okuda, K.: Isolation of rat liver mitochondrial ferredoxin and its reductase active in the 5b- cholestane -3a,7a,12a-triol 26 hydroxylase. (
  • The systematic name of this enzyme class is 5beta-cholestane-3alpha,7alpha,12alpha-triol,NADPH:oxygen oxidoreductase (26-hydroxylating). (
  • Okuda, K.: Enzymatic characteristics of CO-sen - sitive 26-hydroxylase system for 5b- cholestane -3a,7a,12a-triol in rat-liver mitochondria and its intramitochondrial localization. (