A cholesterol derivative found in human feces, gallstones, eggs, and other biological matter.
An autosomal recessive lipid storage disorder due to mutation of the gene CYP27A1 encoding a CHOLESTANETRIOL 26-MONOOXYGENASE. It is characterized by large deposits of CHOLESTEROL and CHOLESTANOL in various tissues resulting in xanthomatous swelling of tendons, early CATARACT, and progressive neurological symptoms.
A condition marked by the development of widespread xanthomas, yellow tumor-like structures filled with lipid deposits. Xanthomas can be found in a variety of tissues including the SKIN; TENDONS; joints of KNEES and ELBOWS. Xanthomatosis is associated with disturbance of LIPID METABOLISM and formation of FOAM CELLS.
Cholestanes substituted in any position with one or more hydroxy groups. They are found in feces and bile. In contrast to bile acids and salts, they are not reabsorbed.
A family of sterols commonly found in plants and plant oils. Alpha-, beta-, and gamma-isomers have been characterized.
Derivatives of the saturated steroid cholestane with methyl groups at C-18 and C-19 and an iso-octyl side chain at C-17.
Steroids with a hydroxyl group at C-3 and most of the skeleton of cholestane. Additional carbon atoms may be present in the side chain. (IUPAC Steroid Nomenclature, 1987)
A procedure consisting of the SURGICAL ANASTOMOSIS of the proximal part of the JEJUNUM to the distal portion of the ILEUM, so as to bypass the nutrient-absorptive segment of the SMALL INTESTINE. Due to the severe malnutrition and life-threatening metabolic complications, this method is no longer used to treat MORBID OBESITY.
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.
CHOLESTENES with one or more double bonds and substituted by any number of keto groups.
A class of organic compounds known as STEROLS or STEROIDS derived from plants.
The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils.
A strongly basic anion exchange resin whose main constituent is polystyrene trimethylbenzylammonium Cl(-) anion.
An intermediate in the synthesis of cholesterol.
Steroid acids and salts. The primary bile acids are derived from cholesterol in the liver and usually conjugated with glycine or taurine. The secondary bile acids are further modified by bacteria in the intestine. They play an important role in the digestion and absorption of fat. They have also been used pharmacologically, especially in the treatment of gallstones.
Abnormal passage in any organ of the biliary tract or between biliary organs and other organs.
A membrane-bound cytochrome P450 enzyme that catalyzes the 7-alpha-hydroxylation of CHOLESTEROL in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP7, converts cholesterol to 7-alpha-hydroxycholesterol which is the first and rate-limiting step in the synthesis of BILE ACIDS.
Cytochrome P-450 monooxygenases (MIXED FUNCTION OXYGENASES) that are important in steroid biosynthesis and metabolism.
Steroids with methyl groups at C-10 and C-13 and a branched 8-carbon chain at C-17. Members include compounds with any degree of unsaturation; however, CHOLESTADIENES is available for derivatives containing two double bonds.
Acquired or inborn metabolic diseases that produce brain dysfunction or damage. These include primary (i.e., disorders intrinsic to the brain) and secondary (i.e., extracranial) metabolic conditions that adversely affect cerebral function.
Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM.
Fibrous bands or cords of CONNECTIVE TISSUE at the ends of SKELETAL MUSCLE FIBERS that serve to attach the MUSCLES to bones and other structures.
Unstable isotopes of carbon that decay or disintegrate emitting radiation. C atoms with atomic weights 10, 11, and 14-16 are radioactive carbon isotopes.
Fractionation of a vaporized sample as a consequence of partition between a mobile gaseous phase and a stationary phase held in a column. Two types are gas-solid chromatography, where the fixed phase is a solid, and gas-liquid, in which the stationary phase is a nonvolatile liquid supported on an inert solid matrix.
An oxidoreductase that catalyzes the conversion of 3-oxo-delta4 steroids into their corresponding 5alpha form. It plays an important role in the conversion of TESTOSTERONE into DIHYDROTESTOSTERONE and PROGESTERONE into DIHYDROPROGESTERONE.
A major primary bile acid produced in the liver and usually conjugated with glycine or taurine. It facilitates fat absorption and cholesterol excretion.
A microanalytical technique combining mass spectrometry and gas chromatography for the qualitative as well as quantitative determinations of compounds.
Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.
Cholesterol which is substituted by a hydroxy group in any position.

Serum sterols during stanol ester feeding in a mildly hypercholesterolemic population. (1/103)

We investigated the changes of cholesterol and non-cholesterol sterol metabolism during plant stanol ester margarine feeding in 153 hypercholesterolemic subjects. Rapeseed oil (canola oil) margarine without (n = 51) and with (n = 102) stanol (2 or 3 g/day) ester was used for 1 year. Serum sterols were analyzed with gas-liquid chromatography. The latter showed a small increase in sitostanol peak during stanol ester margarine eating. Cholestanol, campesterol, and sitosterol proportions to cholesterol were significantly reduced by 5-39% (P < 0.05 or less for all) by stanol esters; the higher their baseline proportions the higher were their reductions. The precursor sterol proportions were significantly increased by 10- 46%, and their high baseline levels predicted low reduction of serum cholesterol. The decrease of the scheduled stanol dose from 3 to 2 g/day after 6-month feeding increased serum cholesterol by 5% (P < 0. 001) and serum plant sterol proportions by 8-13% (P < 0.001), but had no consistent effect on precursor sterols. In twelve subjects, the 12-month level of LDL cholesterol exceeded that of baseline; the non-cholesterol sterol proportions suggested that stimulated synthesis with relatively weak absorption inhibition contributed to the non-responsiveness of these subjects. In conclusion, plant stanol ester feeding lowers serum cholesterol in about 88% of subjects, decreases the non-cholesterol sterols that reflect cholesterol absorption, increases the sterols that reflect cholesterol synthesis, but also slightly increases serum plant stanols. Low synthesis and high absorption efficiency of cholesterol results in the greatest benefit from stanol ester consumption.  (+info)

Highly simplified method for gas-liquid chromatographic quantitation of bile acids and sterols in human stool. (2/103)

A simple method for the gas-liquid chromatographic quantitation of human fecal bile acids and sterols is described where bile acids are subjected to n-butyl ester derivatization, without prior isolation from the stool, followed by trimethylsilylation of the sterols and bile acids. Under these conditions, bile acid derivatives are well resolved from each other and from the trimethylsilyl ether derivatives of fecal sterols and no overlap occurs. The method was shown to be highly reproducible and recoveries were similar to those obtained with other methods used for fecal bile acid analysis. Application of the method for bile acid and sterol analysis in human stool is described.  (+info)

Correlation of neomycin, faecal neutral and acid sterols with colon carcinogenesis in rats. (3/103)

High fat diets have been implicated in incidence of colon cancer both in epidemiological and animal studies. Present investigation deals with the incidence, location and numbers of large and small bowel tumours induced by 1,2-dimethyl hydrazine (DMH) in rats fed high fat diets and neomycin. Neomycin was used to modify the faecal sterol metabolism and the relationship of the high fat diet and faecal neutral and acid sterols to the large bowel tumorigenesis was evaluated. DMH administered rats were fed with (a) 20% safflower oil; (b) 20% safflower oil and neomycin; (c) 20% safflower oil, cholesterol and cholic acid; and (d) 20% safflower oil, cholesterol, cholic acid and neomycin. Neomycin was found to be associated with both increase and decrease of tumour numbers. The faecal sterols lithocholic and deoxycholic acids were found to have no participation, while cholesterol and cholic acid were found to decrease with increase in tumour numbers. However, faecal coprostanol has been found to have a significant positive correlation with tumorigenesis in all dietary groups. Therefore coprostanol might possibly be associated with colon carcinogenesis in DMH-fed rats and cholesterol metabolism in gut appears to be related to the development of tumours.  (+info)

Condensed complexes of cholesterol and phospholipids. (4/103)

Mixtures of dihydrocholesterol and phospholipids form immiscible liquids in monolayer membranes at the air-water interface under specified conditions of temperature and 2-dimensional pressure. In recent work it has been discovered that a number of these mixtures exhibit two upper miscibility critical points. Pairs of upper critical points can be accounted for by a theoretical model that implies the cooperative formation of molecular complexes of dihydrocholesterol and phospholipid molecules. These complexes are calculated to be present in the membranes both above and below the critical points. Below the critical points the complexes form a separate phase, whereas above the critical points the complexes are completely miscible with the other lipid components. The cooperativity of complex formation prompts the use of the terminology condensed complex.  (+info)

Electric field effect on cholesterol-phospholipid complexes. (5/103)

Monolayer mixtures of dihydrocholesterol and phospholipids at the air-water interface are used to model membranes containing cholesterol and phospholipids. Specific, stoichiometric interactions between cholesterol and some but not all phospholipids have been proposed to lead to the formation of condensed complexes. It is reported here that an externally applied electric field of the appropriate sign can destabilize these complexes, resulting in their dissociation. This is demonstrated through the application of an electric field gradient that leads to phase separations in otherwise homogeneous monolayers. This is observed only when the monolayer composition is close to the stoichiometry of the complex. The electric field effect is analyzed with the same mean field thermodynamic model as that used previously to account for pairs of upper miscibility critical points in these mixtures. The concentrations of dihydrocholesterol, phospholipid, and complex vary strongly and sometimes discontinuously in the monolayer membrane in the field gradient. The model is an approximation to a two-dimensional liquid in which molecules freely exchange between free and complexed form so that the chemical potentials are constant throughout the membrane. The calculations are illustrated for a complex of about 15 molecules, composed of 5 cholesterol molecules and 10 phospholipid molecules.  (+info)

Cholestanol induces apoptosis of corneal endothelial and lens epithelial cells. (6/103)

PURPOSE: To determine whether cholestanol induces cornea endothelial and lens epithelial cell death in vitro. METHODS: Cornea endothelial and lens epithelial cells were cultured in minimum essential media with 10% fetal bovine serum containing 10 microg/ml cholesterol in ethanol, 10 microg/ml cholestanol in ethanol, or 1% ethanol. These cells, stained using the terminal deoxynucleotidyl transferase (TdT) dUTP nick-end labeling (TUNEL) method, were analyzed by laser cytometer. The activities of ICE and CPP32 proteases in cells were also measured. RESULTS: Both cornea endothelial and lens epithelial cells cultured with 10 microg/ml cholestanol showed a significant loss of viability. The nuclei of these cells cultured with 10 microg/ml cholestanol were more frequently stained than those exposed to 10 microg/ml cholesterol or 1% ethanol. Quantitative analysis of apoptotic DNA fragmentation confirmed that the cholestanol induced apoptosis of these cells in a time-dependent manner. The activities of interleukin-1beta-converting enzyme (ICE) and CPP32 proteases for cells cultured with 10 microg/ml cholestanol were significantly higher than those observed in control cells. CONCLUSIONS: In vitro, cholestanol was taken up by corneal endothelial cells and lens epithelial cells, an event that led to apoptosis of these cells.  (+info)

Noncholesterol sterols and cholesterol lowering by long-term simvastatin treatment in coronary patients: relation to basal serum cholestanol. (7/103)

Coronary patients with low baseline ratios of serum cholestanol and plant sterols to cholesterol (indicating low cholesterol absorption) but not those with high ratios (high absorption) experienced reduced recurrences of coronary events during simvastatin treatment in the Scandinavian Simvastatin Survival Study. Thus, in the present study, serum cholesterol, its precursor sterols (reflecting cholesterol synthesis), plant sterols (campesterol and sitosterol), and cholestanol were measured before and during a 5-year period of placebo treatment (n=433) and simvastatin treatment (n=434) in patients from a subgroup of the Scandinavian Simvastatin Survival Study to determine whether changes in cholesterol synthesis and serum levels were related to cholesterol absorption. Serum cholesterol level was unchanged, the ratios of cholesterol precursor sterols to cholesterol were decreased, and the ratios of plant sterols to cholesterol were increased in relation to increasing baseline ratios of cholestanol quartiles. The latter predicted 5-year ratios and simvastatin-induced reductions of the precursor sterols, with the lowering of the ratios (cholesterol synthesis reduction) being almost twice higher in the lowest versus the highest quartile. The ratios of plant sterols, especially campesterol, to cholesterol were markedly increased during simvastatin treatment, mostly in subjects with the highest baseline cholestanol quartiles. Simvastatin reduced serum cholesterol more (P=0.003) in the lowest versus the highest cholestanol quartile during the 5-year treatment period. The results show for the first time that baseline cholesterol metabolism, measured by serum noncholesterol sterols, predicts the effectiveness of simvastatin in reducing cholesterol synthesis and serum levels of cholesterol. The drug suppresses the synthesis of cholesterol markedly more effectively in subjects with high than with low baseline synthesis but reduces respective serum cholesterol levels less markedly than synthesis. Subjects with high cholesterol absorption and low synthesis may need a combination therapy to lower more effectively their serum cholesterol levels and prevent an increase in the levels of plant sterols.  (+info)

Condensed complexes, rafts, and the chemical activity of cholesterol in membranes. (8/103)

Epifluorescence microscopy studies of mixtures of phospholipids and cholesterol at the air-water interface often exhibit coexisting liquid phases. The properties of these liquids point to the formation of "condensed complexes" between cholesterol and certain phospholipids, such as sphingomyelin. It is found that monolayers that form complexes can incorporate a low concentration of a ganglioside G(M1). This glycolipid is visualized by using a fluorescently labeled B subunit of cholera toxin. Three coexisting liquid phases are found by using this probe together with a fluorescent phospholipid probe. The three liquid phases are identified as a phospholipid-rich phase, a cholesterol-rich phase, and a condensed complex-rich phase. The cholera toxin B labeled ganglioside G(M1) is found exclusively in the condensed complex-rich phase. Condensed complexes are likely present in animal cell membranes, where they should facilitate the formation of specialized domains such as rafts. Condensed complexes also have a major effect in determining the chemical activity of cholesterol. It is suggested that this chemical activity plays an essential role in the regulation of cholesterol biosynthesis. Gradients in the chemical activity of cholesterol should likewise govern the rates and direction of intracellular intermembrane cholesterol transport.  (+info)

TY - JOUR. T1 - Condensed complexes in vesicles containing cholesterol and phospholipids. AU - Radhakrishnan, Arun. AU - McConnell, Harden. PY - 2005/9/6. Y1 - 2005/9/6. N2 - Animal cell membranes pose conceptual problems related to the physical chemistry of liquids. An avenue to the solution of some of these problems has been opened by the discovery of liquid-liquid immiscibility in synthetic membranes composed of cholesterol and phospholipids. This discovery has led to the development of a thermodynamic model involving condensed complexes. In this model, the phospholipids with longer fatty-acid chains react reversibly with cholesterol to form complexes. The complexes themselves can have a repulsive interaction with other phospholipids, leading to immiscibility. A striking example of this effect is revealed in the phase diagrams of ternary mixtures of cholesterol, a saturated phosphatidylcholine (or sphingomyelin), and an unsaturated phosphatidylcholine. As found by a number of investigators, ...
25-Hydroxyvitamin D2 and D3 (25-OH-VitD) are steroid hormones that require 1-alpha-hydroxylation before expressing biological activity. Vitamin D compounds are derived from dietary ergocalciferol (from plants, VitD2) or cholecalciferol (from animals, VitD3), or by conversion of 7-dihydrocholesterol to VitD3 in the skin upon ultraviolet exposure. VitD2 and VitD3 are subsequently 25-hydroxylated in the liver to 25-OH-VitD. 25-OH-VitD represents the main body reservoir and transport form of vitamin D, being stored in adipose tissue and tightly bound by a transport protein while in circulation. A fraction of circulating 25-OH-VitD is converted to its active metabolites 1,25-dihydroxy vitamin D2 and D3 (1,25-OH-VitD), mainly by the kidneys. This process is regulated by parathyroid hormone (PTH), which increases 1,25-OH-VitD synthesis at the expense of the alternative, biologically inactive hydroxylation product 24,25-OH-VitD. Like other steroid hormones, 1,25-OH-VitD binds to a nuclear receptor, ...
In this study, the capacity of oysters to bioaccumulate fecal stanols and to record a source-specific fingerprint was investigated by the short-term contamination of seawater microcosms containing oysters with a human effluent. Contaminated oysters bioaccumulated the typical fecal stanols coprostanol and 24-ethylcoprostanol and their bioaccumulation kinetics were similar to that of the Fecal Indicator Bacteria Escherichia coli used in European legislation. Although stanol fingerprints of contaminated water allowed the identification of the human specific fingerprint, this was not the case for oysters. This discrepancy is attributed to (i) high concentrations of endogenous cholestanol and sitostanol, responsible for unbalanced stanol fingerprints, (ii) different accumulation/depuration kinetics of fecal coprostanol and 24-ethylcoprostanol and (iii) the limits of the analytical pathway used. These results show that fecal stanols bioaccumulated by oysters are useful to record fecal
Results: Sugar-cholestanols mainly consisting of GlcNAc or GlcNAc-Gal attached to cholestanol showed strong activities of cell proliferation inhibition against various cancer cells but cholestanol without sugar-moieties did not show any activity. When sugar-cholestanols were added into the culture medium of cancer cells, they were incorporated into the cells through the lipid rafts/microdomains on their cell surface in a short period of time and then into the mitochondria followed by release of cytochrome c from the mitochondria, which resulted in the induction of apoptosis by the activation of mitocondria-dependent intrinsic pathways and the caspase-cascade. Further, in vivo experiments in the mouse model for the peritoneal dissemination using colon26 cancer cells showed that tumor growth was significantly reduced and the survival rates of mice were markedly enhanced by administration of sugar-cholestanols ...
The flights were fine. (If you dont count the flight from Boston to Las Vegas, which included an extra hour on the trip due to a strong headwind, expired food, not enough meals for passengers, no movie, and no working radios. US Airways, you sucked it up on that one.) Lovenox was a success. And now I feel like Ive managed to fit in a workout, even at 35,000 feet. But Im curious to know if anyone else has ever taken Lovenox, or another kind of anti-coagulant. And if anyone has ANY tips at all on dealing with this frigging itchy rash. Its starting to go away, and I know its just part of the side-effect fun, but its making me scratchy-crazy. Its all new to me, and Id appreciate any feedback from someone whos been there. (And if you have airplane exercise tips, share those, too. Im heading to Florida in two weeks and am wondering how Im going to embarrass myself on that plane, too!). ...
0088] Colon26 cells (derived from mouse colon cancer) were inoculated to a 96-well plate (1×104 cells/50 μL, 10% FCS-RPMI medium/well), and incubated at 37° C. for 16 hours. To each well, well-known anti-cancers agent (Oxaliplatin (abbreviated as L-OHP), Fluorouracil (5-FU), Paclitaxel (TXL; abbreviated as PTX), Docetaxel (TXT; abbreviated as DTX), Irinotecan (CPT-11; abbreviated as CPT), or Cyclophosphamide (abbreviated as CPA) and/or a cyclodextrin inclusion compound (abbreviated as GC-CD) of a cholestanol derivative in which G in formula (1) is GlcNAcβ- (abbreviated as GC) was added (multi-fold dilution by FCS(-)-medium: final concentration: ≦500 μM, 50 μL), followed by incubation at 37° C. for two days. GC-CD was prepared in accordance with a method disclosed in Example 1(2) in WO 2007/026869. Specifically, a 40% aqueous solution of hydroxypropyl-β-cyclodextrin was prepared, and GC was added to the solution, followed by mixing with stirring (80° C. for 30 minutes), ...
Medical definition of coprostanol: a crystalline sterol C27H47OH formed by bacterial reduction of cholesterol in the intestines and present in feces.
Encyclopedia article on this inherited disorder associated with the deposition of a form of cholesterol (cholestanol) in the brain and other tissues. ...
Seattle-based digital artist Dain Fagerholm has created a series of amazing 3D stereographic drawings. Made from a ballpoint pen and color dye...
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).. ...
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. ...
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.
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
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 ...
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. ...
Lipid tracers including fatty acids, hydroxyacids, n-alkanols, sterols and triterpenoids were used to determine the origin and fate of suspended particulate organic matter (POM) collected in the Rhône River (France). This seasonal survey (April 2011 to May 2013) revealed a year-round strong terrestrial higher-plant contribution to the particulate organic matter (POM), with significant algal inputs observed in March and attributed to phytoplanktonic blooms likely dominated by diatoms. Major terrigenous contributors to our samples are gymnosperms, and more precisely their roots and stems, as evidenced by the presence of high proportions of ω-hydroxydocosanoic acid (a suberin biomarker). The high amounts of coprostanol detected clearly show that the Rhône River is significantly affected by sewage waters. Specific sterol degradation products were quantified and used to assess the part of biotic and abiotic degradation of POM within the river. Higher-plant-derived organic matter appears to be mainly
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The critical points are x=-2 and x=1. You evaluate the first derivative and find its zeroes: y=2*3x^2+3*2x-12*1+0=6x^2+6x-12 y=6x^2+6x-12 And now we can use the quadratic formula Delta=6^2-4*6*(-12)=324 sqrt(Delta)=18 x_1=(-6-18)/(2*6)=(-24)/12=-2 x_2=(-6+18)/(2*6)=12/12=1
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Medical definition of zymosterol: a crystalline unsaturated sterol C27H43OH occurring with ergosterol in yeast fat, resembling ergosterol chemically, and yielding cholestanol on hydrogenation.
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.
d) Plot a bifurcation diagram - this is a plot of the location of the critical points as a function of alpha (plot a graph alpha as x axis and y axis showing the location of the critical point ...
A three-parameter generalized equation is proposed for surface tension from the triple point to the critical point. This equation not only fits the data well but also is good for interpolation...
Would beta sitosterols ability to inhibit DHT from binding to cytosolic androgen receptors be usefull as soon as post cycle therapy begins?
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Cholesterol metabolism may be involved in pediatric gallstone disease. We aimed to reveal cholesterol metabolites and phytosterols and their relation to stone composition of sterols in children having black pigment and cholesterol stones. We performed retrospective controlled clinical study, in which we examined parameters of cholesterol metabolism and liver function values in serum (n = 28) and gallstones (n = 46) of consecutively cholecystectomized children. Serum values of age-, body mass index- and sex-matched children (n = 82) and adult gallstones (n = 187) served as controls. Surrogate markers of cholesterol synthesis in serum (squalene/cholesterol, cholestenol/cholesterol and lathosterol/cholesterol) were 26-52 % higher in both stone subclasses compared to controls (p | 0.05 for all). Respectively, cholestanol/cholesterol and plant sterols campesterol/cholesterol and sitosterol/cholesterol (cholesterol absorption markers) had decreasing order in serum: black pigment stone group | controls |
Define Critical point drying. Critical point drying synonyms, Critical point drying pronunciation, Critical point drying translation, English dictionary definition of Critical point drying. adj. 1. Judging severely and finding fault: a writer who is very critical of the governments foreign policy. 2. a. Relating to or characterized by...
Sitosterolemia, also known as phytosterolemia, is a rare inherited plant sterol storage disease. Bhattacharyya and Connor first described this disease in 1974.{ref1}{ref2} The original report detailed... more
Source Naturals Beta Sitosterol 375mg, Mega Strength 240 tablet hytosterols are natural components of plants, including vegetables and grains. Numerous clinical stu...
INGREDIENTS Each softgel contains: Free plant sterols (non‑GMO, from soy) 350 mg β‑Sitosterols 151.2 mg Stigmasterols 84.4 mg Campes
Cholesterol is an essential component of both the peripheral and central nervous systems of mammals. Over the last decade, evidence has accumulated that disturbances in cholesterol metabolism are associated with the development of various neurological conditions. In addition to genetically defined defects in cholesterol synthesis, which will be covered in another review in this Thematic Series, defects in cholesterol metabolism (cerebrotendinous xanthomatosis) and intracellular transport (Niemann Pick Syndrome) lead to neurological disease. A subform of hereditary spastic paresis (type SPG5) and Huntingtons disease are neurological diseases with mutations in genes that are of importance for cholesterol metabolism. Neurodegeneration is generally associated with disturbances in cholesterol metabolism, and presence of the E4 isoform of the cholesterol transporter apolipoprotein E as well as hypercholesterolemia are important risk factors for development of Alzheimers disease. In the present review, we
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experts believe that the increased frequency of prostate cancer is definitely related to greater incidence of BPH.. By the age 50, nearly half of all men already have BPH. And by the age of 80, almost all men have it. As more ...
This chapter describes the use of callbacks in XML Export. Callbacks allow the developer to intervene at critical points in the export process. Each heading in this chapter is a possible value for the dwCommandOrInfoId parameter passed to the developers callback.
In the field of neurodegeneration, research on oligomeric Abeta and glutamate toxicity, as well as Pin1 isomerase activity are coupled with experimental therapeutic attempts in fibroblasts from Alzheimers disease patients and neuroblastoma cells. Voltage-gated currents and postsynaptic potentials, functional alterations of the blood-brain barrier (BBB) are also investigated. The role of Parkinsons disease-related toxins, genetic alterations and neuroprotective agents on lysosomal catabolic pathways for alpha-synuclein are also investigated, as well as cholinergic and hypocretinergic regulation of synaptic circuits in the prefrontal cortex. Cerebrotendinous xanthomatosis and Spastic paraplegia type 5 are used as models of neurodegeneration due to defective cholesterol metabolism. Metalloprotease ADAM10 is investigated as a new potential target in the Huntingtons disease brain. Prion Protein (PrPc) folding in neuronal membranes and lipid rafts constituents are also established research topics, ...
Dynamical fluctuations in globally conserved quantities such as baryon number, strangeness, charge, and isospin are suggested to carry information about the de-confinement and chiral phase transitions. An observation of enhanced dynamical fluctuations or non-monotonic behavior of transverse momentum correlations as a function of colliding energy might indicate the system has probed the predicted QCD critical point. The STAR experiment has performed a comprehensive study of the energy and charge dependence of dynamical particle ratio ($K/\pi$, $p/\pi$, and $K/p$) fluctuations, net-charge fluctuations, and transverse momentum correlations in the STAR TPC at mid-rapidity, as well as neutral-charge pion fluctuations at forward rapidity. The charge dependence of particle ratio fluctuations exhibit differences between same and opposite sign dynamical particle ratio fluctuations compared to inclusive charged dynamical fluctuations. Neutral-charge pion fluctuations at forward rapidity are measured by ...
Thank you for your interest in spreading the word about Biochemical Journal.. NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.. ...
Dr. Samantha Morris three December 2018 Publications Evaluation of Wu et al.: Comprehending Global and Local Structure of Single-Cell Datasets. Kong W, Morris SA. Cell… ...
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In these final few weeks prior to ICD-10 implementation, your coding countdown checklist should include several key and critical points to consider. T...
Fecal sample extraction (using 100% ethanol) and radioimmunoassay procedures were validated to permit accurate noninvasive assessments of fecal steroid metabolites in captive golden eagles (Aquila chrysaetos) and peregrine falcons (Falcoperegrinus). Furthermore, laboratory validations illustrated that serial dilutions of fecal extracts yielded displacement curves parallel to those of standard preparations for all assays. Recovery of known concentrations of unlabeled steroids in males and females for both species ranged from 97.3 9.8% (y = 1.03x - 0.63, R2= 0.98) to 119.4 5.3% (y = 1.04x + 4.11, R2 = 1.00). Additionally, in the golden eagles, HPLC analyses of fecal B in males and females illustrated that 2.7% and 2.5%, respectively, coeluted with [3HJB; however, a total of 7.5% and 11 .9% of total immunoreactivity coeluted with the reference tracer in the peregrine falcon males and females, respectively. Chromatographic profiles of E metabolites in golden eagle and peregrine falcon females ...
Lane and coworkers go on to show that the electron density in the three diols is really topologically identical, just differing in a matter of degree. They conclude that the existence of a bond critical point should not be the sole arbiter of bonding, but one of the criteria that can be utilized to assess bonding.. While I am not at all in conflict with this conclusion, the paper contains some issues that need be addressed. First off, bonding is not a concept of either-or, rather there is a continuum of bonding. Hydrogen bonding should not at all be confused with covalent or ionic bonding - it is dramatically weaker and so one might consider whether the bcp criteria is applicable at all. The authors really fall into this trap stating … the absence of a BCP should not necessarily be considered evidence as to the absence of a chemical bond (emphasis mine). Do we want to consider a hydrogen bond as a chemical bond?. I think the key element overlooked in this study is the strength of the ...
Fingerprint Dive into the research topics of Fecal Steroid Profile of Female Javan Gibbons (Hylobates moloch) Maintained in Pairing-Typed Cage. Together they form a unique fingerprint. ...
Plant sterols appear to be beneficial for lowering cholesterol and reducing the risk of heart disease. This eMedTV segment discusses other potential benefits of plant sterols and explains how the natural compounds work for reducing cholesterol levels.
Campesterol is a phytochemincal in the class of Phytosterols, belonging to the group of Lipids, found abundantly in banana, pomegranate, pepper, coffee, grapefruit, cucumber, onion, oat, potato, etc. Health benefits 1. Cardiovascular diseases In the review of the studies published between January 1950 and April 2010 that reported either risk ratios (RR) of CVD in …. ...
Vitamart.ca is Canadas Health Store. We carry vitamins, supplements, whey protein, vegan protein with FREE Shipping & 100% Satisfaction Guarantee!
Scanning electron microscope for the visualisation of biological and material samples. Sample preparation is also available (critical point dryer and gold-coater ...
1] N.A Campo, Sur la monodromie des singularités isolées dhypersurfaces complexes (Invent. Math., vol. 20, 1973, p. 147-169). , MR 49 #3201 , Zbl 0264.14002 [2] V. I. ArnolD, Normal forms for functions near degenerate critical points (Funct. Anal. and appl., 6-4, 1972, p. 254-272). , Zbl 0278.57011 [3] V. I. ArnolD, Remarques sur la méthode de la phase stationnaire [Uspekhi Mat. Nauk, 28-5, 1973, p. 17-45, (en russe)]. , MR 53 #1635 , Zbl 0285.40002 [4] M. F. Atiyah, Resolution of singularities and division of distributions (Comm. pure and appl. Math., vol. 23, 1970, p. 145-150). , MR 41 #815 , Zbl 0188.19405 [5] I. N. Bernstein et S. I. Gelfand, Meromorphic property of the function Pλ, (Funct. Anal. and appl., 3-1, 1969, p. 84-85). , MR 40 #723 , Zbl 0208.15201 [6] E. Brieskorn, Die Monodromie der isolierten Singularitäten von Hyperflächen (Manuscripta Math., vol. 2, 1970, p. 103-161). , MR 42 #2509 , Zbl 0186.26101 [7] P. Deligne, Equations différentielles à points singuliers ...
Das ABCG5-Gen befindet sich in einer Kopf-an-Kopf-Position zum ABCG8-Gen. Beide nutzen offensichtlich den selben Promotor und kodieren Transportproteine mit ähnlicher Funktion. Sie sind für die Exkretion von pflanzlichen Sterolen sowohl in die Gallenflüssigkeit wie auch in den Darm verantwortlich. Mutationen können zu den autosomal rezessiven Erkrankungen Sitosterolämia und Mediterrane Makrothrombozytopenie führen.. ...
Salam semua.....menurut pakar kesihatan dari Filipina Herminia de Guzman, kangkung dianggap sebagai penghilang racun dari tubuh. Kangkung juga kaya dengan vitamin A, B1, C, protein, kalsium, fosfor, zat besi, karoten, sitosterol dan hentriakontan. Lepas ni kena la makan kangkung banyak-banyak. Lagipun harga seikat kangkung tak la mahal dan senang untuk didapati. Kalau rajin dan ada masa, ada ruang boleh la tanam sendiri kangkung ni. Lagi pun nak tanam kangkung bukannya susahkan. Lepas ni melor nak tanam kangkung banyak-banyak kat belakang rumah. Boleh la dapat bekalan segar setiap hari ...
Hydrolysis of this ester gave cholestanol 39. The route from cholestanol to cholesterol was already known (see: Robinson ... The conversion of cholestanol to cholesterol was already demonstrated by oxidation of the ketone, bromination to the ... 7 and then to allopregnanolone 8 allowed the addition of the tail group as the acetate in 9 and then conversion to cholestanol ...
An inherited disorder associated with the deposition of a steroid known as cholestanol in the brain and other tissues and with ... Elevated levels of serum cholestanol are diagnostic of CTX. Alternatively analysis of 27-hydroxycholesterol and 7 alpha ...
"Mechanism of accumulation of cholesterol and cholestanol in tendons and the role of sterol 27-hydroxylase (CYP27A1)". ...
5α-cholestanol is formed naturally in the environment by bacteria and generally does not have a faecal origin. Samples with ... As well as the faecally derived stanol, two other isomers can be identified in the environment; 5α-cholestanol 5β-coprostanol ... This reaction occurs principally in anaerobic reducing sediments and the 5α-cholestanol / cholesterol ratio may be used as a ... In the environment, bacteria preferentially produce 5α-cholestan-3β-ol (5α-cholestanol) from cholesterol rather than the 5β ...
Cholestanol, cholesterol, androsterone, Δ4-3α-hydroxy-17-ketoandrostene, 5β,3α-hydroxy-17-ketoandrostane, 5α,3β,17α- ...
... some of which are unique tracers of dung combustion such as cholestanol and coprostanol. Dung cakes are generally a higher ...
... cholestanol MeSH D10.570.938.208.222 - cholesterol, dietary MeSH D10.570.938.208.250 - cholesterol esters MeSH D10.570.938.208. ...
... cholestanol MeSH D04.808.247.808.197.200 - cholesterol esters MeSH D04.808.247.808.197.250 - dehydrocholesterols MeSH D04.808. ... cholestanol MeSH D04.808.247.125 - cholestanones MeSH D04.808.247.222 - cholestenes MeSH D04.808.247.222.159 - cholecalciferol ...
A molecule called cholestanol, which is similar to cholesterol, is produced as well as substances called bile alcohols. ... Cholestanol and bile alcohols are increased in the blood, while blood cholesterol levels are typically normal. In various ... Cholestanol metabolism in patients with cerebrotendinous xanthomatosis: absorption, turnover, and tissue deposition. J Lipid ... The accumulation of cholesterol and cholestanol throughout the bodys tissues causes the signs and symptoms of cerebrotendinous ...
Cholestanol plasma concentration assessment. Elevated cholestanol results (or changes in clinical status) may warrant further ... A diet low in cholestanol containing foods (eg, egg yolk, butter, cheddar cheese) can significantly reduce plasma cholestanol ... The storage of cholestanol within the nervous system. Arch Neurol. 1968 Jul. 19(1):47-53. [QxMD MEDLINE Link]. ... Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism. Ann Intern Med. 1971 Dec. 75(6):843-51 ...
Cholestanol plasma concentration assessment. Elevated cholestanol results (or changes in clinical status) may warrant further ... A diet low in cholestanol containing foods (eg, egg yolk, butter, cheddar cheese) can significantly reduce plasma cholestanol ... The storage of cholestanol within the nervous system. Arch Neurol. 1968 Jul. 19(1):47-53. [QxMD MEDLINE Link]. ... Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism. Ann Intern Med. 1971 Dec. 75(6):843-51 ...
Hydrolysis of this ester gave cholestanol 39. The route from cholestanol to cholesterol was already known (see: Robinson ... The conversion of cholestanol to cholesterol was already demonstrated by oxidation of the ketone, bromination to the ... 7 and then to allopregnanolone 8 allowed the addition of the tail group as the acetate in 9 and then conversion to cholestanol ...
cholestanol 7alpha,8alpha-epoxide. 0. cholesterol beta-epoxide. 0. deacetyltanghinin. 0. eplerenone. 30. ...
Cholestanol 0.64 ± 0.11 0.64 ± 0.12 0.62 ± 0.15 0.56 ± 0.16 0.60 ± 0.08 0.59 ± 0.07 0.61 ± 0.10 0.61 ± 0.14 0.60 ± 0.12 ...
In contrast, snails contain two additional sterols, desmosterol and cholestanol. Of the polyunsaturated fatty acids in slugs, ...
Monitoring patients through testing plasma cholestanol levels and neurologic examination was recommended, although further ... Consensus agreement that genetic analyses and/or determination of serum cholestanol levels should be used to diagnose CTX, and ... However, while serum cholestanol may be useful for evaluating treatment compliance [3], research on the impact of cholestanol ... All panellists responded that patients always have elevated serum cholestanol at diagnosis and that measuring serum cholestanol ...
It is not as effective as CDCA in suppressing BAS and the production of cholestanol, but lacks the hepatotoxicity of CDCA. ... Defective enzymatic function disrupts bile acid synthesis leading to cholesterol and cholestanol deposits, which result in a ... First-line treatment is based on chenodeoxycholic acid (CDCA) replacement therapy, which normalizes BAS and cholestanol ... Early diagnosis and treatment are crucial to prevent progressive accumulation of cholestanol and cholesterol: disease ...
World Association of Social Psychiatry (WASP) 2014 Book of Abstracts ...
... such as POE cholestanol ether and the like; POE hydrogenated castor oils, such as POE hydrogenated castor oil, POE hydrogenated ...
The total neutral sterol amount was defined as the sum of cholesterol, coprostanol, coprostanone, cholestanol and cholestanone ...
Cholestanol Current Synonym true false 166122013 3-beta-Hydroxycholestane Current Synonym true false ...
Choi, J. W., van Rosendael, A. R., Bax, A. M., van den Hoogen, I. J., Gianni, U., Baskaran, L., Andreini, D., De Cecco, C. N., Earls, J., Ferencik, M., Hecht, H., Leipsic, J. A., Maurovich-Horvat, P., Nicol, E., Pontone, G., Raman, S., Schoenhagen, P., Arbab-Zadeh, A., Choi, A. D., Feuchtner, G., & 6 othersWeir-McCall, J., Chinnaiyan, K., Whelton, S., Min, J. K., Villines, T. C. & AlAref, S. J., Mar 1 2020, In: Journal of Cardiovascular Computed Tomography. 14, 2, p. 107-117 11 p.. Research output: Contribution to journal › Review article › peer-review ...
Cholestanol plasma concentration assessment. Elevated cholestanol results (or changes in clinical status) may warrant further ... A diet low in cholestanol containing foods (eg, egg yolk, butter, cheddar cheese) can significantly reduce plasma cholestanol ... The storage of cholestanol within the nervous system. Arch Neurol. 1968 Jul. 19(1):47-53. [QxMD MEDLINE Link]. ... Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism. Ann Intern Med. 1971 Dec. 75(6):843-51 ...
Cholestanol plasma concentration assessment. Elevated cholestanol results (or changes in clinical status) may warrant further ... A diet low in cholestanol containing foods (eg, egg yolk, butter, cheddar cheese) can significantly reduce plasma cholestanol ... The storage of cholestanol within the nervous system. Arch Neurol. 1968 Jul. 19(1):47-53. [QxMD MEDLINE Link]. ... Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism. Ann Intern Med. 1971 Dec. 75(6):843-51 ...
Competitive inhibition of bile acid synthesis by endogenous cholestanol and sitosterol in sitosterolemia with xanthomatosis. ...
Cholestanol is formed in a pathway from the bile acid precursor 7-alpha-hydroxy-4-cholesten-3-one. [12] Deposition of ... The storage of cholestanol within the nervous system. Arch Neurol. 1968 Jul. 19(1):47-53. [QxMD MEDLINE Link]. ... Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism. Ann Intern Med. 1971 Dec. 75(6):843-51 ... Skrede S, Björkhem I, Buchmann MS, Hopen G, Fausa O. A novel pathway for biosynthesis of cholestanol with 7 alpha-hydroxylated ...
Additionally, highly significant correlations have been found for coprostanol and cholesterol (r=0.986*) and for cholestanol ... Additionally, highly significant correlations have been found for coprostanol and cholesterol (r=0.986*) and for cholestanol ... Additionally, highly significant correlations have been found for coprostanol and cholesterol (r=0.986*) and for cholestanol ... Additionally, highly significant correlations have been found for coprostanol and cholesterol (r=0.986*) and for cholestanol ...
Baseline serum cholestanol as predictor of recurrent coronary events in subgroup of Scandinavian simvastatin survival study. ...
Key words: FTIR spectral study, 2-pyridine aldoxime methyl chloride, cholinesterase reactivator, cholestanol, hydrogen bonded ... cholestanol, cholinesterase reactivator, CMC, colloidal silver, conformational analysis, cyclic imides, D2 receptor, database, ...
... cholestanol, cholic acid, 12-deoxycholic acid, 7-deoxycholic acid, or a derivative thereof, such as cholesterol sulfate), and ... cholestanol, cholic acid, 12-deoxycholic acid, 7-deoxycholic acid, or a derivative thereof, and mixtures thereof and conjugated ... cholestanol, cholestenone, desmosterol, 7-dehydrodesmosterol, dehydroepiandrosterone (DHEA), desmosterol, diosgenin, FF-MAS (14 ...
Serum cholesterol and cholestanol levels confirmed CTX in our patient.. CTX is an autosomal recessive disorder of bile acid ... Laboratory testing revealed a serum cholestanol level of 10.11 μg/mL, which confirmed a diagnosis of cerebrotendinous ... Deficiency of mitochondrial enzyme sterol 27-hydroxylase leads to excessive cholestanol and cholesterol production and ...
Cholestanol * C26:0-Lyso-PC (fibroblasten) * C26:0-Lyso-PC (bloed) * Complementatie analyse ...
Long-chain fatty acids, cholesterol, cholestanol. *. Serum ceruloplasmin. *. B1, B12 and folic acid ...
Potent anti-respiratory syncytial virus activity of a cholestanol-sulfated tetrasaccharide conjugate. Antiviral Research, 93 (1 ...
... cholestanol (5α-cholestan-3β-ol) and cholesterol (cholest-5-en-3β-ol). Extractable Σcoprostanol and cholestanol concentrations ... Extractable cholestanol exhibits the same trend, but extractable cholesterol shows the opposite trend. This indicates ... In addition, both bound Σcoprostanol/TOC and cholestanol/TOC display a decreasing trend with core depth and no pronounced ... The Zcoprostanol/cholesterol and cholestanol/cholesterol ratios are comparatively higher in sediment than in the sewage ...
HMDB0000908, Cholestanol, Dihydrocholesterol, 5α-Cholestan-3β-ol, (CAS 80-97-7). 326. HMDB0000054, Bilirubin, (CAS 635-65-4). ...
... glucose AUC for all synthesis markers and for the absorption markers for cholestanol . In addition, there was an interaction of ... the percentage change in the serum insulin AUC was negatively correlated with cholestanol , campesterol , and sitosterol . When ... AUC was positively correlated with the baseline cholesterol synthesis markers but negatively correlated with the cholestanol ...
  • A molecule called cholestanol, which is similar to cholesterol, is produced as well as substances called bile alcohols. (medlineplus.gov)
  • Cholestanol and bile alcohols are increased in the blood, while blood cholesterol levels are typically normal. (medlineplus.gov)
  • In various tissues in the body, including the brain and heart, cholesterol and cholestanol levels are increased. (medlineplus.gov)
  • The accumulation of cholesterol and cholestanol throughout the body's tissues causes the signs and symptoms of cerebrotendinous xanthomatosis. (medlineplus.gov)
  • Salen G, Meriwether TW, Nicolau G. Chenodeoxycholic acid inhibits increased cholesterol and cholestanol synthesis in patients with cerebrotendinous xanthomatosis. (medscape.com)
  • The conversion of cholestanol to cholesterol was already demonstrated by oxidation of the ketone, bromination to the bromoketone and elimination to the enone. (wikipedia.org)
  • Defective enzymatic function disrupts bile acid synthesis leading to cholesterol and cholestanol deposits, which result in a degenerative process. (orpha.net)
  • A short sediment core with a length of approximately 40 cm taken from the anoxic Tan-Shui estuary, Taiwan, was analysed for extractable and bound coprostanol (5β-cholestan-3β-ol), cholestanol (5α-cholestan-3β-ol) and cholesterol (cholest-5-en-3β-ol). (9lib.co)
  • The Zcoprostanol/cholesterol and cholestanol/cholesterol ratios are comparatively higher in sediment than in the sewage effluent, implying some degree of diagenesis in the extractable phase. (9lib.co)
  • Extractable cholestanol exhibits the same trend, but extractable cholesterol shows the opposite trend. (9lib.co)
  • Consensus agreement that genetic analyses and/or determination of serum cholestanol levels should be used to diagnose CTX, and dried bloodspot testing should facilitate detection in newborns, was reached. (biomedcentral.com)
  • Diagnosis in adults is based on two of the following criteria: intractable diarrhea, presenile cataracts, tendinous xanthomata and neurologic abnormalities, and abnormal amounts of cholestanol in serum and tendons. (orpha.net)
  • Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. (medscape.com)
  • [ 12 ] Deposition of cholestanol and other intermediate metabolites in the CNS (the brain and spinal cord), muscle (including the heart), blood vessels, eyes, and tendons results in progressive dysfunction unless treatment is initiated to prevent further accumulation of toxic metabolites. (medscape.com)
  • In contrast, snails contain two additional sterols, desmosterol and cholestanol. (raysahelian.com)
  • Monitoring patients through testing plasma cholestanol levels and neurologic examination was recommended, although further research regarding monitoring treatment and progression of the disease is required. (biomedcentral.com)
  • First-line treatment is based on chenodeoxycholic acid (CDCA) replacement therapy, which normalizes BAS and cholestanol concentrations, and improves neurological symptoms. (orpha.net)
  • Extractable Σcoprostanol and cholestanol concentrations exhibit an abrupt change at a depth of about 20 cm, which supposedly marks the time when a sewage treatment plant became operational in the estuary in 1980. (9lib.co)
  • Alongside the hallmark clinical features, biochemical and molecular genetic tests are typically used to diagnose CTX through detection of increased levels of plasma cholestanol and/or identification of CYP27A1 pathogenic variants [ 12 ]. (biomedcentral.com)
  • Cholestanol is formed in a pathway from the bile acid precursor 7-alpha-hydroxy-4-cholesten-3-one. (medscape.com)
  • In addition, both bound Σcoprostanol/TOC and cholestanol/TOC display a decreasing trend with core depth and no pronounced concentration change at 20 cm depth. (9lib.co)
  • Because of the slow buildup of cholestanol in the brain in people with CTX, it can take years for neurological problems to show up. (aboutctx.com)
  • The storage of cholestanol within the nervous system. (medscape.com)
  • 5alpha-Cholest-7-en-3beta-ol = 13 reactions were found. (tu-bs.de)
  • Conversion of 6 to pregnenolone 7 and then to allopregnanolone 8 allowed the addition of the tail group as the acetate in 9 and then conversion to cholestanol 10. (wikipedia.org)
  • Elevated cholestanol results (or changes in clinical status) may warrant further investigation and potential treatment modification. (medscape.com)
  • Cholestanol is formed in a pathway from the bile acid precursor 7-alpha-hydroxy-4-cholesten-3-one. (medscape.com)
  • Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. (medscape.com)
  • What is the difference between Cholestanol 5alpha and 5beta? (stackexchange.com)
  • What is the isomeric relation between 5Alpha Cholestanol and 5Beta Cholestanol. (stackexchange.com)
  • Of the 18 tissues analyzed at biopsy and autopsy, the cholestanol content varied from 0.09 mg/g in psoas muscle to 76 mg/g in a cerebellar xanthoma. (nih.gov)
  • The result is reduced bile acid synthesis and increased levels of cholestanol in plasma, tissues, and CSF. (arizona.edu)
  • These conditions lead to a significant increase in the levels of bile alcohols and levels of cholestanol, a byproduct of abnormal bile acid synthesis. (eyewiki.org)
  • Figure 1: Mutations in the CYP27A1 gene prevent critical steps in the bile acid synthesis that leads to bile acid deficiency, which results in a buildup of cholestanol throughout the body that causes distinctive clinical manifestations. (eyewiki.org)
  • In the absence of the key enzyme, sterol 27-hydroxylase, other metabolites are increased such as cholestanol. (faoj.org)
  • The importance of cholesterol's planar ring structure was tested by comparing planar cholestanol (5α-cholestan-3β-ol) with nonplanar coprostanol (5β-cholestan-3β-ol). (helmholtz-muenchen.de)
  • Liver enzymes and a lipid panel found no abnormalities except for an elevated cholestanol level of 25.8 ug/mL (normal value is 4.2 +/- 1.2 ug/mL). (faoj.org)
  • Elevated cholestanol results (or changes in clinical status) may warrant further investigation and potential treatment modification. (medscape.com)
  • 16. A novel etiologic factor of highly elevated cholestanol levels: progressive familial intrahepatic cholestasis. (nih.gov)
  • cholestanol was especially high in nerve tissue. (nih.gov)
  • Our data indicate that CTX patients absorb cholestanol from the diet. (nih.gov)
  • The rapid exchangeable pool of cholestanol was 233 mg, and the total exchangeable pool was 752 mg. (nih.gov)
  • They have a higher than normal cholestanol production rate. (nih.gov)