Cholestanol
Xanthomatosis, Cerebrotendinous
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.
Xanthomatosis
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.
Cholestanols
Sitosterols
Cholestanes
Sterols
Jejunoileal Bypass
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.
Cholestanetriol 26-Monooxygenase
Cholestenones
Cholesterol
Cholestyramine Resin
Bile Acids and Salts
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.
Biliary Fistula
Cholesterol 7-alpha-Hydroxylase
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.
Steroid Hydroxylases
Cholestenes
Brain Diseases, Metabolic
Brain Diseases
Tendons
Carbon Radioisotopes
Chromatography, Gas
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.
Cholestenone 5 alpha-Reductase
Cholic Acid
Gas Chromatography-Mass Spectrometry
Lipid Metabolism, Inborn Errors
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)Condensed complexes in vesicles containing cholesterol and phospholipids<...
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Cholesterol total synthesis
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 ...
Cerebrotendineous xanthomatosis
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 ...
CYP27A1
"Mechanism of accumulation of cholesterol and cholestanol in tendons and the role of sterol 27-hydroxylase (CYP27A1)". ...
Coprostanol
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 ...
Siberian musk deer
Cholestanol, cholesterol, androsterone, Δ4-3α-hydroxy-17-ketoandrostene, 5β,3α-hydroxy-17-ketoandrostane, 5α,3β,17α- ...
Dry dung fuel
... some of which are unique tracers of dung combustion such as cholestanol and coprostanol. Dung cakes are generally a higher ...
List of MeSH codes (D10)
... cholestanol MeSH D10.570.938.208.222 - cholesterol, dietary MeSH D10.570.938.208.250 - cholesterol esters MeSH D10.570.938.208. ...
List of MeSH codes (D04)
... 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 ...
Cerebrotendinous xanthomatosis: MedlinePlus Genetics
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 ...
Cerebrotendinous Xanthomatosis (CTX) Treatment & Management: Medical Care, Surgical Care, Consultations
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 ...
Cerebrotendinous Xanthomatosis (CTX) Treatment & Management: Medical Care, Surgical Care, Consultations
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 ...
Cholesterol total synthesis - Wikipedia
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 ...
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Expert opinion on diagnosing, treating and managing patients with cerebrotendinous xanthomatosis (CTX): a modified Delphi study...
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 ...
Orphanet: Cerebrotendinous xanthomatosis
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 ...
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Cerebrotendinous Xanthomatosis (CTX) Treatment & Management: Medical Care, Surgical Care, Consultations
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 ...
Cerebrotendinous Xanthomatosis (CTX) Treatment & Management: Medical Care, Surgical Care, Consultations
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 ...
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Cerebrotendinous Xanthomatosis (CTX): Background, Pathophysiology, Epidemiology
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 ...
Sedimentary coprostanol in Kaohsiung Harbour and the Tan-Shui Estuary, Taiwan<...
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 ...
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... 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 ...
Multiple Huge Tendinous Xanthomas with Normal Lipid Profiles in All Extremities
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 ...
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Coprostanol in a sediment core from the anoxic Tan-Shui estuary, Taiwan.
... 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 ...
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How To Lower Glucose And Cholesterol - HealthyCholesterolClub.com
... 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 ...
Cholesterol10
- 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)
Serum2
- 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)
Deposition2
- 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)
Sterols1
- In contrast, snails contain two additional sterols, desmosterol and cholestanol. (raysahelian.com)
Neurologic1
- 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)
Concentrations2
- 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)
Genetic1
- 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)
Bile acid1
- Cholestanol is formed in a pathway from the bile acid precursor 7-alpha-hydroxy-4-cholesten-3-one. (medscape.com)
Plasma1
- Cholestanol plasma concentration assessment. (medscape.com)
Concentration1
- 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)
Brain1
- 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)
Storage1
- The storage of cholestanol within the nervous system. (medscape.com)
Found1
- 5alpha-Cholest-7-en-3beta-ol = 13 reactions were found. (tu-bs.de)
Conversion1
- 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)
Results1
- Elevated cholestanol results (or changes in clinical status) may warrant further investigation and potential treatment modification. (medscape.com)
Bile1
- Cholestanol is formed in a pathway from the bile acid precursor 7-alpha-hydroxy-4-cholesten-3-one. (medscape.com)
Cerebrotendinous1
- Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. (medscape.com)
5beta2
- What is the difference between Cholestanol 5alpha and 5beta? (stackexchange.com)
- What is the isomeric relation between 5Alpha Cholestanol and 5Beta Cholestanol. (stackexchange.com)
Tissues2
- 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)
Bile acid synthesis2
- 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)
Metabolites1
- In the absence of the key enzyme, sterol 27-hydroxylase, other metabolites are increased such as cholestanol. (faoj.org)
Coprostanol1
- 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)
Liver1
- 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)
Clinical1
- Elevated cholestanol results (or changes in clinical status) may warrant further investigation and potential treatment modification. (medscape.com)
Levels1
- 16. A novel etiologic factor of highly elevated cholestanol levels: progressive familial intrahepatic cholestasis. (nih.gov)
High1
- cholestanol was especially high in nerve tissue. (nih.gov)
Patients1
- Our data indicate that CTX patients absorb cholestanol from the diet. (nih.gov)
Total1
- The rapid exchangeable pool of cholestanol was 233 mg, and the total exchangeable pool was 752 mg. (nih.gov)
Normal1
- They have a higher than normal cholestanol production rate. (nih.gov)