Results 31 to 40 of about 3,700 (219)

Age-related changes of cholestanol and lathosterol plasma concentrations: an explorative study

open access: yesLipids in Health and Disease, 2019
Background Cerebrotendinous xanthomatosis (CTX) and Lathosterolosis represent two treatable inherited disorders of cholesterol metabolism that are characterized by the accumulation of cholestanol and lathosterol, respectively.
Monica Gelzo   +7 more
doaj   +1 more source

Usefulness of cholestanol levels in the diagnosis and follow-up of patients with cerebrotendinous xanthomatosis

open access: yesNeurología (English Edition), 2011
Introduction: Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive disease caused by a deficiency of mitochondrial enzyme sterol 27-hydrolylase.
B. Pilo de la Fuente   +8 more
doaj   +1 more source

Successful Treatment of a Rare Cholesterol Homeostasis Disorder Due to CYP27A1 Gene Mutation with Chenodeoxycholic Acid Therapy

open access: yesBiomedicines, 2023
Cerebrotendinous xanthomatosis (CTX) is a genetic disorder of the cholesterol metabolic pathway, most often associated with variants in the CYP27A1 gene.
Petar Brlek   +8 more
doaj   +1 more source

The microstratigraphy of middens: capturing daily routine in rubbish at Neolithic Çatalhöyük, Turkey [PDF]

open access: yes, 2011
Microstratigraphy — the sequencing of detailed biological signals on site — is an important new approach being developed in the Çatalhöyük project. Here the authors show how microscopic recording of the strata and content of widespread middens on the ...
Almond, Matthew J.   +3 more
core   +1 more source

Biosynthesis of cholesterol, lanosterol, and delta 7-cholestenol, but not cholestanol, in cultured fibroblasts from normal individuals and patients with cerebrotendinous xanthomatosis.

open access: yesJournal of Lipid Research, 1982
The cholesterol and cholestanol biosynthetic pathways and the control of cholesterolgenesis were investigated in skin fibroblasts, from patients with cerebrotendinous xanthomatosis (CTX) and from normal subjects, growth in a lipoprotein deficient (d less
G S Tint, G Salen
doaj   +1 more source

Low density lipoprotein cholesterol is inversely correlated with abdominal visceral fat area: a magnetic resonance imaging study [PDF]

open access: yes, 2011
Background Visceral Fat Area (VFA) is an independent predictor of coronary disease. While low density lipoprotein cholesterol (LDL-C) is used to determine risk and guide therapy, its accuracy fails in obese patients who may have low LDL-C despite high ...
Michel R Hoenig   +4 more
core   +1 more source

Metabolism of coprostanol-C14 and cholestanol-4-C14 in man

open access: yesJournal of Lipid Research, 1963
Coprostanol-C14, biosynthetically prepared, was administered orally to two patients, and cholestanol-4-C14 was administered to one of them 10 months later.
R.S. Rosenfeld   +2 more
doaj   +1 more source

Sterol concentration and distribution in sunflower seeds (Helianthus annuus L.) during seed development [PDF]

open access: yes, 2010
Sunflower seeds are currently used for edible oil production. Among oil minor compounds, phytosterols are of special interest due to their cholesterol reducing properties.
Alignan, Marion   +5 more
core   +2 more sources

Highly sensitive analysis of sterol profiles in human serum by LC-ESI-MS/MSs⃞

open access: yesJournal of Lipid Research, 2008
We have developed a highly sensitive and specific method for the analysis of serum sterol profiles. Sterols in 1 μl of dried serum were derivatized into picolinyl esters (3β-picolinate) and analyzed by liquid chromatography-tandem mass spectrometry (LC ...
Akira Honda   +8 more
doaj   +1 more source

Gene supplementation of CYP27A1 in the liver restores bile acid metabolism in a mouse model of cerebrotendinous xanthomatosis

open access: yesMolecular Therapy: Methods & Clinical Development, 2021
Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive disease caused by mutations in the CYP27A1 gene, encoding the sterol 27-hydroxylase. Disruption of the bile acid biosynthesis pathway and accumulation of toxic precursors such as cholestanol ...
Sara Lumbreras   +12 more
doaj   +1 more source

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