Cholestadiene derivatives containing a hydroxy group anywhere in the molecule.
Detailed account or statement or formal record of data resulting from empirical inquiry.
Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.
A loose confederation of computer communication networks around the world. The networks that make up the Internet are connected through several backbone networks. The Internet grew out of the US Government ARPAnet project and was designed to facilitate information exchange.
The portion of an interactive computer program that issues messages to and receives commands from a user.
Sequential operating programs and data which instruct the functioning of a digital computer.
Linear POLYPEPTIDES that are synthesized on RIBOSOMES and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of AMINO ACIDS determines the shape the polypeptide will take, during PROTEIN FOLDING, and the function of the protein.
A class in the phylum CNIDARIA, comprised mostly of corals and anemones. All members occur only as polyps; the medusa stage is completely absent.
Twenty-carbon compounds derived from MEVALONIC ACID or deoxyxylulose phosphate.
A plant genus of the family POACEAE. The seed is one of the millets used in EDIBLE GRAIN. It contains vitexin. The common name of buffelgrass is also used for CENCHRUS.
A plant genus in the CHENOPODIACEAE family.
A plant genus of the family POACEAE. The seed is one of the EDIBLE GRAINS used in millet cereals and in feed for birds and livestock (ANIMAL FEED). It contains diosgenin (SAPONINS).

Bile acid synthesis in the Smith-Lemli-Opitz syndrome: effects of dehydrocholesterols on cholesterol 7alpha-hydroxylase and 27-hydroxylase activities in rat liver. (1/52)

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)

Nuclear and cytoplasmic maturation of mouse oocytes after treatment with synthetic meiosis-activating sterol in vitro. (2/52)

Synthetically produced meiosis-activating sterol, a sterol originally derived from follicular fluid (FF-MAS), induces meiotic maturation of mouse oocytes in vitro. We therefore compared FF-MAS-induced maturation of naked mouse oocytes arrested in prophase I by either hypoxanthine (Hx) or forskolin (Fo) with spontaneous maturation of naked oocytes. FF-MAS-treated oocytes overcame the meiotic block by Hx or Fo, although germinal vesicle breakdown was delayed by 11 h and 7 h, respectively. We also investigated the influence of FF-MAS on chromosome, microtubule, and ultrastructural dynamics in Hx-cultured oocytes by immunocytochemistry and electron microscopy. Similarly to spontaneously matured oocytes, chromosomes became aligned, a barrel-shaped spindle formed, and overall organelle distribution was normal in FF-MAS-matured oocytes. The number of small cytoplasmic asters was elevated in FF-MAS-treated oocytes. Although the number of cortical granules (CGs) was similar to that in spontaneously matured oocytes, the overall distance between CGs and oolemma was increased in the FF-MAS group. These observations suggest that the initiation of meiotic maturation in FF-MAS-treated oocytes in the presence of high cAMP levels leads to a delayed but otherwise normal nuclear maturation. FF-MAS appears to improve oocyte quality by supporting microtubule assembly and by delaying CG release, which is known to contribute to reduced fertilization.  (+info)

Diagnosis of Smith-Lemli-Opitz syndrome from stored filter paper blood specimens. (3/52)

BACKGROUND: Smith-Lemli-Opitz (SLO) syndrome is a recessively inheritable metabolic disease with deficiency of cholesterol and accumulation of dehydrocholesterols, caused by a defect in the last step of cholesterol biosynthesis. Biochemical methods for identification of affected individuals, even prenatally, have been developed. Reliable genetic counselling is now possible. AIM: To find a method of proving or disproving whether a child in whom SLO syndrome had been suspected but not confirmed during lifetime had in fact died of the SLO syndrome. METHODS: Lipid extracts of stored filter paper blood specimens collected at the national neonatal metabolic screening were used. The ratio of dehydrocholesterols to cholesterol was measured by combined gas chromatography-mass spectrometry. RESULTS: The ratio of 8-dehydrocholesterol to cholesterol in stored filter paper specimens clearly distinguished affected infants from normal infants. SLO syndrome was thus proven in two children who had died more than seven years earlier. CONCLUSION: It is possible to diagnose SLO syndrome from dried paper specimens, even when the samples were collected more than a decade ago. Genetic counselling is available for families of affected children who died before the discovery of the defect in cholesterol synthesis.  (+info)

Simvastatin. A new therapeutic approach for Smith-Lemli-Opitz syndrome. (4/52)

The Smith-Lemli-Opitz syndrome (SLOS) is caused by deficient Delta(7)-dehydrocholesterol reductase, which catalyzes the final step of the cholesterol biosynthetic pathway, resulting in low cholesterol and high concentrations of its direct precursors 7-dehydrocholesterol (7DHC) and 8DHC. We hypothesized that i) 7DHC and 8DHC accumulation contributes to the poor outcome of SLOS patients and ii) blood exchange transfusions with hydroxymethylglutaryl (HMG)-CoA reductase inhibition would improve the precursor-to-cholesterol ratio and may improve the clinical outcome of SLO patients. First, an in vitro study was performed to study sterol exchange between plasma and erythrocyte membranes. Second, several exchange transfusions were carried out in vivo in two SLOS patients. Third, simvastatin was given for 23 and 14 months to two patients. The in vitro results illustrated rapid sterol exchange between plasma and erythrocyte membranes. The effect of exchange transfusion was impressive and prompt but the effect on plasma sterol levels lasted only for 3 days. In contrast, simvastatin treatment for several months demonstrated a lasting improvement of the precursor-to-cholesterol ratio in plasma, erythrocyte membranes, and cerebrospinal fluid (CSF). Plasma precursor concentrations decreased to 28 and 33% of the initial level, respectively, whereas the cholesterol concentration normalized by a more than twofold increase. During the follow-up period all morphometric parameters improved. The therapy was well tolerated and no unwanted clinical side effects occurred. This is the first study in which the blood cholesterol level in SLOS patients is normalized with a simultaneous significant decrease in precursor levels. There was a lasting biochemical improvement with encouraging clinical improvement. Statin therapy is a promising novel approach in SLOS that deserves further studies in larger series of patients.  (+info)

Biosynthesis of sterols by a yeast homogenate. Incorporation of mevalonic acid into cholesta-5,7,24-trien-3beta-ol and 5alpha-cholesta-7,24-dien-3beta-ol. (5/52)

Incubation of (3RS,2R)-[2-14C,2-3H]mevalonic acid and (3RS, 2S)-[2-14C,2-3H]mevalonic acid with mechanically disrupted yeast cells resulted in C27-metabolites. Two (14C5, 3H4)-metabolites, cholesta-5,7,24-trien-3beta-ol and 5 alpha-cholesta-7,24-dien-3beta-ol, were isolated and characterized. The impairment of the 24-methyl transferase system was confirmed by the lack of incorporation of 14C into the sterol fraction on incubation of S-adenosyl-L-[methyl-14C]methionine with the yeast homogenate. The results indicate that interference with the (C-24)-alkylating system did not prevent the transformation of lanosterol to the cholesta-5,7,24-trien-3beta-ol and to 5 alpha-cholesta-7,24-dien-3beta-ol. It can therefore be inferred that transformations of the nucleus and of the side chain can function independently. However our results do not provide a definition of the actual sequence of the metabolic events between lanosterol and ergosterol.  (+info)

Regulation of spontaneous and induced resumption of meiosis in mouse oocytes by different intracellular pathways. (6/52)

The mitogen-activated protein kinase-dependent and the cAMP-protein kinase A-dependent signal transduction pathways were studied in cultured mouse oocytes during induced and spontaneous meiotic maturation. The role of the mitogen-activated protein kinase pathway was assessed using PD98059, which specifically inhibits mitogen-activated protein kinase 1 and 2 (that is, MEK1 and MEK2), which activates mitogen-activated protein kinase. The cAMP-dependent protein kinase was studied by treating oocytes with the protein kinase A inhibitor rp-cAMP. Inhibition of the mitogen-activated protein kinase pathway by PD98059 (25 micromol l(-1)) selectively inhibited the stimulatory effect on meiotic maturation by FSH and meiosis-activating sterol (that is, 4,4-dimethyl-5alpha-cholest-8,14, 24-triene-3beta-ol) in the presence of 4 mmol hypoxanthine l(-1), whereas spontaneous maturation in the absence of hypoxanthine was unaffected. This finding indicates that different signal transduction mechanisms are involved in induced and spontaneous maturation. The protein kinase A inhibitor rp-cAMP induced meiotic maturation in the presence of 4 mmol hypoxanthine l(-1), an effect that was additive to the maturation-promoting effect of FSH and meiosis-activating sterol, indicating that induced maturation also uses the cAMP-protein kinase A-dependent signal transduction pathway. In conclusion, induced and spontaneous maturation of mouse oocytes appear to use different signal transduction pathways.  (+info)

Oxysterols in the circulation of patients with the Smith-Lemli-Opitz syndrome: abnormal levels of 24S- and 27-hydroxycholesterol. (7/52)

Infants with the cholesterol synthesis defect Smith- Lemli-Opitz syndrome (SLO) have reduced activity of the enzyme 7-dehydrocholesterol-7-reductase and accumulate 7-dehydrocholesterol, with the highest concentration in the brain. As a result of the generally reduced content of cholesterol, plasma levels of oxysterols would be expected to be reduced. 24S-hydroxycholesterol is almost exclusively formed in the brain, whereas 27-hydroxycholesterol is mainly formed from extrahepatic and extracerebral cholesterol. In accordance with the expectations, sterol-correlated plasma levels of 24S-hydroxycholesterol were reduced in infants with SLO (by about 50%). In contrast, the sterol-correlated levels of 27-hydroxycholesterol in the circulation were markedly increased. No side-chain oxidized metabolites of 7-dehydrocholesterol were detected in the circulation. Recombinant human CYP27 had markedly lower 27-hydroxylase activity toward 7-dehydrocholesterol than towards cholesterol. HEK293 cells expressing 24S-hydroxylase active toward cholesterol had no significant activity towards 7-dehydrocholesterol. The plasma levels of 3 beta,7 alpha-dihydroxy-5-cholestenoic in the patients acid were reduced, suggesting a generally reduced metabolism of 27-oxygenated steroids. It is concluded that the accumulation of 7-dehydrocholesterol in the brains of patients with SLO reduces formation of 24S-hydroxycholesterol. The condition is associated with markedly increased circulating levels of 27-hydroxycholesterol, most probably due to reduced metabolism of this oxysterol. We discuss the possibility that the circulating levels of 24S-hydroxycholesterol may be used as a marker for the severity of the disease.--Bjorkhem, I., L. Starck, U. Andersson, D. Lutjohann, S. von Bahr, I. Pikuleva, A. Babiker, and U. Diczfaulsy. Oxysterols in the circulation of patients with the Smith-Lemli-Opitz syndrome: abnormal levels of 24S- and 27-hydroxycholesterol. J. Lipid Res. 2001. 42: 366--371.  (+info)

Progestins block cholesterol synthesis to produce meiosis-activating sterols. (8/52)

The resumption of meiosis is regulated by meiosis-preventing and meiosis-activating substances in testes and ovaries. Certain C29 precursors of cholesterol are present at elevated levels in gonadal tissue, but the mechanism by which these meiosis-activating sterols (MAS) accumulate has remained an unresolved question. Here we report that progestins alter cholesterol synthesis in HepG2 cells and rat testes to increase levels of major MAS (FF-MAS and T-MAS). These C29 sterols accumulated as a result of inhibition of Delta24-reduction and 4alpha-demethylation. Progesterone, pregnenolone, and 17alpha-OH-pregnenolone were potent inhibitors of Delta24-reduction in an in vitro cell assay and led to the accumulation of desmosterol, a Delta5,24 sterol precursor of cholesterol. A markedly different effect was observed for 17alpha-OH-progesterone, which caused the accumulation of sterols associated with inhibition of 4alpha-demethylation. The flux of 13C-acetate into lathosterol and cholesterol was decreased by progestins as measured by isotopomer spectral analysis, whereas newly synthesized MAS accumulated. The combined evidence that MAS concentrations can be regulated by physiological levels of progestins and their specific combination provides a plausible explanation for the elevated concentration of MAS in gonads and suggests a new role for progestins in fertility.  (+info)

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Reactome is pathway database which provides intuitive bioinformatics tools for the visualisation, interpretation and analysis of pathway knowledge.
Scientifically reviewed pathway providing a current overview of Sterol Biosynthesis in A. thaliana Interactive web-based biological databases and softwares for life-scientists and researchers. - Scientifically reviewed pathway providing a current overview of Sterol Biosynthesis in A. thaliana
Read chapter Status Report on the Alteration of Fatty Acid and Sterol Composition in Lipids in Meat, Milk, and Eggs: Fat Content and Composition of Animal...
Ojserkis, Bennett E., The effect of sterol composition on the activities of membrane-bound enzymes in mouse L cells. (1974). Summer and Academic Year Student Reports. 609 ...
Subcutaneously administered anti-IL-6 receptor antibody for treatment of systemic sclerosis | Ophthalmic composition comprising a prostaglandin | Methods and compositions for stimulating neurogenesis and inhibiting neuronal degeneration | Dihydro-benzo-oxazine and dihydro-pyrido-oxazine derivatives | Compounds and methods of use thereof for treating neurodegenerative disorders |
A new acceptor-donor-acceptor-structured nonfullerene acceptor ITCC (3,9-bis(4-(1,1-dicyanomethylene)-3-methylene-2-oxo-cyclopenta[b]thiophen)-5,5,11,11-tetrakis(4-hexylphenyl)-dithieno[2,3-d′:2,3-d′]-s-indaceno[1,2-b:5,6-b′]-dithiophene) is ...
This thesis examines the gene promoter activity and morphological characteristics of mutants of HYDRA 1 (HYD 1) and HYDRA2 I FACKEL (HYD2 I FK) (Mayer et al. 1991, Topping et al. 1997) from Arabidopsis. These loci are unique, and encode components of the sterol biosynthesis pathway (Schrick et al. 2000, Souter et at. 2002). Various patterning processes are disrupted in hydra mutants (Topping et at. 1997), and bulk sterol profiles are altered (Schrick et at. 2000, Souter et al. 2002). The mutants show heightened responses to auxin, and their phenotype is partly ameliorated by inhibition of ethylene signalling (Souter et al. 2002, 2004, He et al. 2003). Although much previous attention has been given to the analysis of their phenotype, the precise basis of the pleiotropic defects seen in the hydra mutants have not been attributed to any single phenomenon.This thesis examines the hydra mutants body patterning and morphology, and aims to test the hypothesis that hydra mutants are defective in ...
3-(6-((2-methylene-3-(((octadecylamino)carbonyl)oxy)propoxy)carbonyl)hexyl)thiazolium: RN refers to Br salt; RN & structure given in first source; PAF antagonist
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ID 2: 485. Toxin: n. Trivial name: 2,​5-​Piperazinedione, 3-​[[2-​(1,​1-​dimethyl-​2-​propenyl)​-​6-​(3-​methyl-​2-​butenyl)​-​1H-​indol-​3-​yl]​methylene]​-​6-​methylene-​, (3Z)​- (9CI); 2,​5-​Piperazinedione, 3-​[[2-​(1,​1-​dimethyl-​2-​propenyl)​-​6-​(3-​methyl-​2-​butenyl)​-​1H-​indol-​3-​yl]​methylene]​-​6-​methylene-​, (Z)​-; Alkaloid E 8, from Aspergillus amstelodami; Cryptoechinulin A; Cryptoechinuline A; Neoechinulin C; Neoechinuline C. Systematic name: 2,​5-​Piperazinedione, 3-​[[2-​(1,​1-​dimethyl-​2-​propen-​1-​yl)​-​6-​(3-​methyl-​2-​buten-​1-​yl)​-​1H-​indol-​3-​yl]​methylene]​-​6-​methylene-​, (3Z)​-. Molecular formulae: C24H27N3O2. Molecular weight: 389.49. Chemical abstract number: 55179-54-9. Chemical type: alkaloid. Literature reference:. ...
Pederin,(1S)-2,6-Anhydro-3,5,7-trideoxy-1-C-[[(2S)-hydroxy[(2R,5R,6R)-tetrahydro-2-methoxy-5,6-dimethyl-4-methylene-2H-pyran-2-yl]acetyl]amino]-5,5-dimethyl-1,8,9-tri-O-methyl-D-manno-nonitol,N-[[6-(2,3-dimethoxypropyl)tetrahydro-4-hydroxy-5,5-dimethyl-2H-pyran-2-yl]methoxymethyl]tetrahydro-2-methoxy-5,6-dimethyl-4-methylene-2H-pyran-2-glycolamide,pederine,paederine,Pseudopederin,Pseudopederin,N-[[6-(2,3-Dimethoxypropyl)tetrahydro-4-hydroxy-5,5-dimethyl-2H-pyran-2-yl]methoxymethyl]tetrahydro-alpha,2-dihydroxy-5,6-dimethyl-4-methylene-2H-pyran-2-acetamide,psi-paederine,psi-pederine,pseudopaederine
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0078] Since the presence of mature DC and CD8+ cells in the tumors positively influence the outcome of lung cancer patients, the inventors stratified the patients into 4 groups according to the high or low density of each marker (DC-LampHi/CD8Hi, DC-LampHi/CD8Lo, DC-LampLo/CD8Hi, and DC-LampLo/CD8Lo). The inventors observed that the group of patients with DC-LampHi tumors regardless of the density of CD8S cells had the lowest risk of death (P=3,4×10-07, median OS were 92 months for DC-LampHi/CD8SHi patients and 100 months for DC-LampHi/CD8SLo patients), as was observed for DC-LampHi patients. Interestingly, only the DC-LampHi patients present an improved survival as compared to the whole cohort. In contrast, patients with a low density of both dendritic and CD8S cells were at highest risk of death (median OS was 22 months) as compared to each immune marker alone (mean OS DC-LampLo=36 months, mean OS CD8SLo=40 months). Patients with DC-LampLo /CD8SHi tumors were at an intermediate risk of death ...
A new acceptor-donor-acceptor-structured nonfullerene acceptor ITCC (3,9-bis(4-(1,1-dicyanomethylene)-3-methylene-2-oxo-cyclopenta[b]thiophen)-5,5,11,11-tetrakis(4-hexylphenyl)-dithieno[2,3-d′:2,3-d′]-s-indaceno[1,2-b:5,6-b′]-dithiophene) is ...
Define Smith-Lemli-Opitz syndrome. Smith-Lemli-Opitz syndrome synonyms, Smith-Lemli-Opitz syndrome pronunciation, Smith-Lemli-Opitz syndrome translation, English dictionary definition of Smith-Lemli-Opitz syndrome. n. 1. A group of symptoms that collectively indicate or characterize a disease, disorder, or other condition considered abnormal. 2. a. A complex of...
Smith-Lemli-Opitz syndrome (SLOS) is a severe autosomal recessive disorder resulting from defects in the cholesterol synthesising enzyme 7-dehydrocholesterol reductase (Δ7-sterol reductase, DHCR7, EC 1.3.1.21) leading to a build-up of the cholesterol precursor 7-dehydrocholesterol (7-DHC) in tissues and blood plasma. Although the underling enzyme deficiency associated with SLOS is clear there are likely to be multiple mechanisms responsible for SLOS pathology. In an effort to learn more of the aetiology of SLOS we have analysed plasma from SLOS patients to search for metabolites derived from 7-DHC which may be responsible for some of the pathology. We have identified a novel hydroxy-8-dehydrocholesterol, which is either 24- or 25-hydroxy-8-dehydrocholesterol and also the known metabolites 26-hydroxy-8-dehydrocholesterol, 4-hydroxy-7-dehydrocholesterol, 3β,5α-dihydroxycholest-7-en-6-one and 7α,8α-epoxycholesterol. None of these metabolites are detected in control plasma at quantifiable ...
Smith-Lemli-Opitz syndrome (SLOS, RSH, OMIM #270400) is an autosomal recessive, multiple malformation, mental retardation syndrome due to an inborn error of cholesterol biosynthesis. Specifically, these patients have a deficiency of 3 beta-hydroxysterol Delta 7-reductase activity due to mutation of the 3 beta-hydroxysterol delta 7-reductase gene (DHCR7). This enzymatic deficiency impairs the conversion of 7-dehydrocholesterol (7-DHC) to cholesterol in the last step of cholesterol biosynthesis via the Kandutch-Russel biosynthetic pathway. The clinical manifestations of SLOS are extremely variable and the phenotypic spectrum is broad. At the severe end of the spectrum SLOS is a lethal disorder with multiple major congenital anomalies, and in mild cases SLOS combines minor physical stigmata with behavioral and learning disabilities. Based on clinical studies, the incidence of SLOS is on the order of 1/10,000 to 1/60,000. Molecular studies have shown a carrier frequency of about 1% for the most ...
Smith-Lemli-Opitz Syndrome (SLOS) is an autosomal recessive disorder caused by a metabolic error in the final step of cholesterol biosynthesis, leading to cholesterol deficiency and accumulation of the cholesterol precursor, 7-dehydrocholesterol.Patients with SLOS display complex medical problems including growth failure, intellectual disability, behavioral disorders, progressive retinal dystrophy, hearing loss and photosensitivity. Dr Elias was one of the original geneticists who discovered the cause of this disorder in 1994, and ever since has been treating SLOS patients with cholesterol supplementation. Since 2008, a second medication called AquADEKS, a mixture of vitamins and other compounds with antioxidant properties was added to the treatment regimen. The purpose of the AquADEKS is to allow treatment with antioxidant medications in an effort to prevent retinal degeneration, hearing and skin problems associated with SLOS.. This protocol has been approved by the Colorado Multiple ...
This abstract was presented today at the Association for Research in Vision and Opthalmology (ARVO) meetings in Seattle, Washington by Steven J. Fliesler, Christopher C. Goulah, W. Drew Ferrell, Robert E. Marc and Bryan W. Jones.. Purpose: Smith-Lemli-Opitz syndrome (SLOS) is a developmental disorder involving defective cholesterol biosynthesis. Prior studies using a rat model of SLOS have documented progressive retinal dysfunction and degeneration, apparently involving caspase-3-independent cell death of photoreceptors. Retinal remodeling has been documented in human retinal degenerations and a myriad of animal models of retinal disease (Jpn J Ophthalmol. 56(4):289, 2012). Here, we examined retinal degeneration and remodeling in the SLOS rat model vs. age-matched control rats.. Methods: A pharmacologically-induced rat model of SLOS was generated by treating Sprague-Dawley rats with AY9944 (Arch. Ophthalmol. 122:1190, 2004). At 81 days postnatal (P81), eyes from AY9944-treated and control rats ...
We investigated the enzyme defect in late cholesterol biosynthesis in the Smith-Lemli-Opitz syndrome, a recessively inherited developmental disorder characterized by facial dysmorphism, mental retardation, and multiple organ congenital anomalies. Reduced plasma and tissue cholesterol with increased 7-dehydrocholesterol concentrations are biochemical features diagnostic of the inherited enzyme defect. Using isotope incorporation assays, we measured the transformation of the precursors, [3 alpha- 3H]lathosterol and [1,2-3H]7-dehydrocholesterol into cholesterol by liver microsomes from seven controls and four Smith-Lemli-Opitz homozygous subjects. The introduction of the double bond in lathosterol at C-5[6] to form 7-dehydrocholesterol that is catalyzed by lathosterol-5-dehydrogenase was equally rapid in controls and homozygotes liver microsomes (120 +/- 8 vs 100 +/- 7 pmol/mg protein per min, P = NS). In distinction, the reduction of the double bond at C-7 [8] in 7-dehydrocholesterol to yield ...
Emyr Lloyd-Evans lab, Cardiff University. Lysosome cell biology and lysosomal storage diseases. Including Niemann-Pick type C (NPC) and Batten disease (NCLs).
Caspeta L, Chen Y, Ghiaci P, Feizi A, Buskov S, Hallström BM, Petranovic D, Nielsen J Science 346 (6205) 75-78 [2014-10-03; online 2014-10-02] Ethanol production for use as a biofuel is mainly achieved through simultaneous saccharification and fermentation by yeast. Operating at ≥40°C would be beneficial in terms of increasing efficiency of the process and reducing costs, but yeast does not grow efficiently at those temperatures. We used adaptive laboratory evolution to select yeast strains with improved growth and ethanol production at ≥40°C. Sequencing of the whole genome, genome-wide gene expression, and metabolic-flux analyses revealed a change in sterol composition, from ergosterol to fecosterol, caused by mutations in the C-5 sterol desaturase gene, and increased expression of genes involved in sterol biosynthesis. Additionally, large chromosome III rearrangements and mutations in genes associated with DNA damage and respiration were found, but contributed less to the thermotolerant ...
soy derived sterylglucoside mixture: a soy-bean derived mixture; contains 49.9% beta-sitosterol monoglucoside, 29.1% campesterol, 13.8% stigmasterol, and 7.2% brassicasterol; a potentially effective absorption enhancer for the nasal absorption of insulin
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Affiliation:山口大学,大学院・医学系研究科,元教授, Research Field:Pediatrics,Fundamental nursing,Clinical nursing,Human genetics,基礎・地域看護学, Keywords:PCR,遺伝看護,遺伝看護学,先天異常,出生前診断,アポトーシス,遺伝看護教育,神経可塑性,7-dehydrocholesterol,コレステロール代謝異常症, # of Research Projects:23, # of Research Products:45
When cursor points to a box further details will be displayed in a tooltip window. If you click on the box it will change to the appropriate reaction scheme or enzyme specification.. Part of cholesterol and related sterol biosynthesis. ...
Looking for online definition of Smith-Lemli-Opitz syndrome in the Medical Dictionary? Smith-Lemli-Opitz syndrome explanation free. What is Smith-Lemli-Opitz syndrome? Meaning of Smith-Lemli-Opitz syndrome medical term. What does Smith-Lemli-Opitz syndrome mean?
Smith-Lemli-Opitz syndrome (SLOS, OMIM 270400) is an autosomal recessive disorder of cholesterol biosynthesis resulting from deficient 3β-hydroxysterol Δ7-reductase (DHCR7) activity.1,2 Patients with SLOS have a characteristic facial phenotype, various degrees of cleft palate and of syndactyly of toes 2 and 3, failure to thrive, behavioural problems, and mental retardation in addition to variable combinations of external and internal malformations.3-5 The spectrum of severity extends from prenatal death with holoprosencephaly or other lethal malformations to minimally physically affected patients with normal intelligence or minimal intellectual impairment. Most patients with SLOS have abnormally low levels of plasma cholesterol and all have raised levels of its immediate precursor, 7-dehydrocholesterol (7DHC).2. The DHCR7 gene has been mapped to chromosome 11q13, spans approximately 14 kb, and encodes a protein of 475 amino acid residues.6 To date, 85 different DHCR7 mutations have been ...
Smith-Lemli-Opitz syndrome: Deficient Delta 7-reductase activity in cultured skin fibroblasts and chorionic villus fibroblasts and its application to pre- and postnatal ...
This gene encodes an enzyme that removes the C(7-8) double bond in the B ring of sterols and catalyzes the conversion of 7-dehydrocholesterol to cholesterol. This gene is ubiquitously expressed and its transmembrane protein localizes to the endoplasmic reticulum membrane and nuclear outer membrane. Mutations in this gene cause Smith-Lemli-Opitz syndrome (SLOS); a syndrome that is metabolically characterized by reduced serum cholesterol levels and elevated serum 7-dehydrocholesterol levels and phenotypically characterized by cognitive disability, facial dysmorphism, syndactyly of second and third toes, and holoprosencephaly in severe cases to minimal physical abnormalities and near-normal intelligence in mild cases. Alternative splicing results in multiple transcript variants that encode the same protein.[provided by RefSeq, Aug 2009 ...
This gene encodes an enzyme that removes the C(7-8) double bond in the B ring of sterols and catalyzes the conversion of 7-dehydrocholesterol to cholesterol. This gene is ubiquitously expressed and its transmembrane protein localizes to the endoplasmic reticulum membrane and nuclear outer membrane. Mutations in this gene cause Smith-Lemli-Opitz syndrome (SLOS); a syndrome that is metabolically characterized by reduced serum cholesterol levels and elevated serum 7-dehydrocholesterol levels and phenotypically characterized by mental retardation, facial dysmorphism, syndactyly of second and third toes, and holoprosencephaly in severe cases to minimal physical abnormalities and near-normal intelligence in mild cases. Alternative splicing results in multiple transcript variants that encode the same protein.[provided by RefSeq, Aug 2009 ...
Meiosis-activating sterols (MAS) are substrates of SC4MOL and NSDHL in the cholesterol pathway and are important for normal organismal development. Oncogenic transformation by epidermal growth factor receptor (EGFR) or RAS increases the demand for cholesterol, suggesting a possibility for metabolic interference. To test this idea in vivo, we ablated Nsdhl in adult keratinocytes expressing KRAS(G12D). Strikingly, Nsdhl inactivation antagonized the growth of skin tumors while having little effect on normal skin. Loss of Nsdhl induced the expression of ATP-binding cassette (ABC) transporters ABCA1 and ABCG1, reduced the expression of low-density lipoprotein receptor (LDLR), decreased intracellular cholesterol, and was dependent on the liver X receptor (LXR) α. Importantly, EGFR signaling opposed LXRα effects on cholesterol homeostasis, whereas an EGFR inhibitor synergized with LXRα agonists in killing cancer cells. Inhibition of SC4MOL or NSDHL, or activation of LXRα by sterol metabolites, can ...
Corn oil (CO) and Sterculic foetida oil (SFO) fed rats were injected with [9, 10-methylene-¹⁴C]sterculic acid. Less than 1% of the label was expired as carbon dioxide. The majority of the label was excreted in the urine as short-chain dicarboxylic acids with an intact cyclopropane ring. The major metabolites for both CO and SFO fed rats were cis-3, 4-methylene adipic acid and cis-3, 4- methylene suberic acid. Sterculic acid must undergo β- and [Greek w]-oxidation to form these urinary metabolites, α-oxidation played a minor role in the formation of cis- and trans-3, 4-methylene pimelic acid. Rats on the SFO diet could metabolize sterculic acid faster than fats on the CO diet. However, both CO and SFO fed rats produced the same urinary metabolites. CO fed rats incorporated more label from sterculic acid into protein and acid soluble liver fractions than SFO fed rats. Less than 0.01% of the label from either group was found in liver lipid sterol or glycerol fractions. There was a tendency for ...
Minor aberrant pathways of cholesterol biosynthesis normally produce only trace levels of abnormal sterol metabolites but may assume major importance when an essential biosynthetic step is blocked. Cholesta-5,8-dien-3beta-ol, its Delta(5,7) isomer, and other noncholesterol sterols accumulate in subjects with the Smith-Lemli-Opitz syndrome (SLOS), a severe developmental disorder caused by a ...
To evaluate whether dietary intake of plant sterols might be beneficial for the prevention of AD, we analyzed the effect of plant sterols and cholesterol on the molecular mechanisms of APP processing.. We found that stigmasterol was the only plant sterol that significantly decreased Aβ levels. Compared with stigmasterol, β-sitosterol and cholesterol significantly increased Aβ levels, whereas campesterol and brassicasterol showed minor or no effect on Aβ secretion. Interestingly, the Aβ-reducing plant sterol stigmasterol is structurally the most distinctive from cholesterol, having an additional double-bond at C22-C23 and an ethyl-group at C24. The analysis of the molecular mechanisms revealed that stigmasterol directly inhibited β-secretase activity and further reduced β-secretase cleavage of APP by decreasing BACE1 internalization from the plasma membrane to the endosomal compartments, known to have an optimal acidic pH for BACE1 cleavage of APP (Vassar et al., 1999; Huse et al., 2000). ...
Lanosterol 14α-demethylase (or CYP51A1) is a cytochrome P450 enzyme that is involved in the conversion of lanosterol to 4,4-dimethylcholesta-8(9),14,24-trien-3β-ol. The cytochrome P450 isoenzymes are a conserved group of proteins that serve as key players in the metabolism of organic substances and the biosynthesis of important steroids, lipids, and vitamins in eukaryotes. As a member of this family, lanosterol 14α-demethylase is responsible for an essential step in the biosynthesis of sterols. In particular, this protein catalyzes the removal of the C-14α-methyl group from lanosterol (Lepesheva et al.). This demethylation step is regarded as the initial checkpoint in the transformation of lanosterol to other sterols that are widely used within the cell (Lepesheva et al.). Although lanosterol 14α-demethylase is present in a wide variety of organisms, this enzyme is studied primarily in the context of fungi, where it plays an essential role in mediating membrane permeability. In fungi, CYP51 ...
Smith-Lemli-Opitz Syndrome (SLOS) is a genetic disorder (autosomal recessive) caused by an abnormality in the production of cholesterol. The disorder can occur in both a mild or severe form. SLOS is associated with multiple birth defects and mental retardation. Some of the birth defects include; abnormal facial features, poor muscle tone, poor growth, shortened life span, and abnormalities of the heart, lungs, brain, gastrointestinal tract, limbs, genitalia, and kidneys. There is no known cure for SLOS but recently patients have been treated with increased amounts of cholesterol in their diet. The cholesterol in a persons diet is unable to correct the abnormalities in the patients organs, but researchers hope it will improve growth failure and mental retardation. This study was developed to answer questions about the causes and complications of SLOS, as well as the effectiveness of cholesterol treatment. The study will enroll patients diagnosed with SLOS, and their mothers. The objectives ...
Taton, M.; Benveniste, P.; Rahier, A., 1989: Microsomal delta 8,14-sterol delta 14-reductase in higher plants. Characterization and inhibition by analogues of a presumptive carbocationic intermediate of the reduction reaction
Sigma-Aldrich offers abstracts and full-text articles by [Raku Shinkyo, Libin Xu, Keri A Tallman, Qian Cheng, Ned A Porter, F Peter Guengerich].
Synthesis of 17b-hydroxy-androst-4-eno[3,2-c]pyrazole 3, 17b-hydroxy-androst-4-eno[3,2-c]-1 H-5 -methylthio-pyrazole 4, 17b-hydroxy-androst-4-eno[2,3-d]isoxazole 5, 17b-hydroxy-androst-4-eno[2,3-d] -1 H-3 -methylthio isoxazole 6, 17b-hydroxy-androst-4-eno[3,2-d]-2 -amino-6 -ethoxypyrimidine 7, 17b,2 -dihydroxy-androst-4-eno[3,2-d]- 6 -ethoxypyrimidine 8, and 17b-hydroxy-androst-4-eno[3,2-d]-2 -thiolo-6 -ethoxypyrimidine 9, 17b-hydroxy-4-androsteno[3,2-d]-2 -amino-6 -ethoxypyrimidine 10, 17b,2 -dihydroxy-4-androsteno[3,2-d]-6 ethoxypyrimidine 11, 17b-hydroxy-4-androsteno[3,2-d]-2 -thiolo-6 -ethoxypyrimidine 12, from 2-ethoxymethylene-4-androsten-3-one 1 and 2-bis (methylthio) methylene-4-androsten-3-one 2 are reported ...
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This work extends previous research regarding the role of (esterified) steryl glucosides ¿(E)SG- in olive oil. In line with previous research and to contribute to a comprehensive olive oil chemical characterization, we have determined the profile and content of these compounds in different mono-variety virgin olive oils using cholesterol ß-D-glucoside (ChSG) as single internal standard. To do this, we have collected 22 types of olive cultivars, extracted the oils by the Abencor® method and applied a formerly developed SPE protocol. We have also determined the corresponding response factors with respect to the internal standard. Additionally, we have analyzed some of the purity and quality parameters included in the Regulations with the aim of understanding their relationship with those sterol derivatives. Results show the feasibility of using ChSG as internal standard, shortening the global time of analysis. They also confirm the suitability of the limits previously established for the ...
The present invention is directed towards the hemisulfate salt of 5,10-methylene-(6R)-tetrahydrofolic acid, preferably in substantially crystalline form, as well as pharmaceutical compositions and uses thereof in therapy, preferably chemotherapy.
Ergosterol is the major sterol found in the membranes of Chlamydomonas reinhardtii. While past studies have identified some ergosterol mutants in C. reinhardtii, very little is known about sterol biosynthesis pathways in this species. With the elucidation of the Chlamydomonas genome, bioinformatics analysis has allowed us to determine potential genes involved in ergosterol biosynthesis. With this knowledge, a working model of the pathway was designed for future analysis. Several of the ergosterol biosynthetic genes were analyzed in respect to their role and involvement in flagellar regeneration. These genes were upregulated during the regrowth of the flagella. Also Chlamydomonas strains lacking flagella were analyzed by Q-RT PCR to determine what role ergosterol biosynthetic genes played in the absence of their flagella. Finally, one of the genes with homology to the yeast sterol C-5 desaturase, ERG3, was chosen for further analysis. To test whether ERG3 of C. reinhardtii had a similar function, yeast
Medical definition of zymosterol: a crystalline unsaturated sterol C27H43OH occurring with ergosterol in yeast fat, resembling ergosterol chemically, and yielding cholestanol on hydrogenation.
Magnesium compounds of tetradentate amino-bis(phenolato) ligands, Mg[L1] (1) and Mg[L2] (2) (where [L1] = 2-pyridyl-N,N-bis(2-methylene-4-methoxy-6-tert-butylphenolato), and [L2] = dimethylaminoethylamino-N,N-bis(2-methylene-4-methyl-6-tert-butylphenolato)) were prepared. The proligands, H2[L1] and H2[L2] we Earth Abundant Element Compounds in Homogeneous Catalysis
A process for the preparation of 6-chloro-.alpha.-methylcarbazole-2-acetic acid from 6-chloro-.alpha.-hydroxy-.alpha.-methylcarbazole-2-acetic acid and/or 6-chloro-.alpha.-methylene-2-carboxylic acid, is described. The preparation of intermediates, such as, 6-chloro-.alpha.-hydroxy-.alpha.-methylcarbazole-2-acetic acid and lower alkyl carbazole-2-oxalate, inter alia, is also described.
The use of behavioral changes as an indicator of teratogenic potential is evaluated. Testing procedures involving central nervous system activity or task specific responses are discussed along the effects of drug metabolism on behavioral response. Preliminary results of a behavioral teratology study of methylene- chloride (75092) in Long-Evans-rats are included. The author concludes that behaviora
You are viewing an interactive 3D depiction of the molecule (6alpha,17alpha)-19-norpregna-1(10),2,4-trien-20-yne-3,6,17-triol (C20H24O3) from the PQR.
The attempted Baylis-Hillman reactions of N-tosyl aldimines and aryl aldehydes with 3-methylpenta-3,4-dien-2-one gave the corresponding Baylis-Hillman adducts 3 and 6 in moderate to good yields in the presence of DMAP in DMSO, respectively. In the case of the aza-Baylis-Hillman reactions of N-tosyl aldimines
Here you can find all of the regulations and regulatory lists in which this substance appears, according to the data available to ECHA. This substance has been found in the following regulatory activities (directly, or inheriting the regulatory context of a parent substance):. ...
... cholestadienols MeSH D04.808.247.222.222.347.200 - dehydrocholesterols MeSH D04.808.247.222.222.347.231 - desmosterol MeSH ...
The PDB archive contains information about experimentally-determined structures of proteins, nucleic acids, and complex assemblies. As a member of the wwPDB, the RCSB PDB curates and annotates PDB data according to agreed upon standards. The RCSB PDB also provides a variety of tools and resources. Users can perform simple and advanced searches based on annotations relating to sequence, structure and function. These molecules are visualized, downloaded, and analyzed by users who range from students to specialized scientists.
glaucasterol: from soft coral Sarcophyton glaucum; natural C27 sterol with cyclopropane ring in side chain; structure given in first source
Cholestadienols コレスタジエノール Sterols ステロール DNA Primers プライマー-DNA ...
... cholestadienols MeSH D04.808.247.222.222.347.200 - dehydrocholesterols MeSH D04.808.247.222.222.347.231 - desmosterol MeSH ...
cholestadienols*euonymus*cinnamomum aromaticum*cuscuta*neurogranin*cholestenes*human umbilical vein endothelial cells*2 ...
Cholestadienols D4.808.247.222.222.347 D4.210.500.247.222.222.347 Cholestanes D4.808.247 D4.210.500.247 Cholestanetriol 26- ...
Animals , Apoptosis/drug effects , Bile/chemistry , Cholestadienes/toxicity , Cholestadienols/toxicity , Cholestanes/toxicity ...
Humans , Male , Female , Risk Factors , Sex Factors , Case-Control Studies , Smoking , Cholestadienols/blood , Body Mass Index ...
Cholestadienols [D04.808.247.222.222.347]. *Lanosterol [D04.808.247.222.222.347.557]. *Sterols [D04.808.247.808] ...
cholestadienols*tomatine*volatile fatty acids*xanthomatosis*dimyristoylphosphatidylcholine*ketocholesterols*azetidines* ...

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