Age-related reference intervals for serum phytosterols in children by gas chromatography-mass spectrometry and its application in diagnosing sitosterolemia

Wu, Mengyuan; Zhou, Pei; Sun, Wei; Wu, Hongping; Sun, Yan; Wu, Biao; Zhou, Wenhao; Luo, Feihong; Lü, Wei

doi:10.1016/j.cca.2023.117234

Cited by 2 publications

(3 citation statements)

References 27 publications

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“…The levels of phytosterols (canolesterol and stigmasterol), [ 70 ] and cholesterol were reduced in the SD group. Cholesterol has important structural roles in cell membranes and lipid metabolism and is a biosynthetic precursor of bile acids, vitamin D, and steroid hormones (glucocorticoids, estrogens, progesterone, androgens, and aldosterone).…”

Section: Discussionmentioning

confidence: 99%

“…[68] Arachidonic acid is a polyunsaturated essential fatty acid substrate for synthesizing prostaglandins, thromboxanes, and leukotrienes, mediating inflammatory processes and immune function, and acting as an antidepressant role. [69] The levels of phytosterols (canolesterol and stigmasterol), [70] and cholesterol were reduced in the SD group. Cholesterol has important structural roles in cell membranes and lipid metabolism and is a biosynthetic precursor of bile acids, vitamin D, and steroid hormones (glucocorticoids, estrogens, progesterone, androgens, and aldosterone).…”

Section: Fecal Granulesmentioning

confidence: 95%

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Systematic Metabolic Profiling of Mice with Sleep‐Deprivation

Si,

Sun,

Liang

et al. 2023

Advanced Biology

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Adequate sleep is essential for the biological maintenance of physical energy. Lack of sleep can affect thinking, lead to emotional anxiety, reduce immunity, and interfere with endocrine and metabolic processes, leading to disease. Previous studies have focused on long‐term sleep deprivation and the risk of cancer, heart disease, diabetes, and obesity. However, systematic metabolomics analyses of blood, heart, liver, spleen, kidney, brown adipose tissue, and fecal granules have not been performed. This study aims to systematically assess the metabolic changes in the target organs caused by sleep deprivation in vivo, to search for differential metabolites and the involved metabolic pathways, to further understand the impact of sleep deprivation on health, and to provide strong evidence for the need for early intervention.

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Section: Discussionmentioning

confidence: 99%

Section: Fecal Granulesmentioning

confidence: 95%

Systematic Metabolic Profiling of Mice with Sleep‐Deprivation

Si,

Sun,

Liang

et al. 2023

Advanced Biology

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“…Unfortunately, access to quantitative measurements of non-cholesterol sterols in clinical samples is currently limited to highly specialized labs due to the requirement of both elaborate sample preparation and advanced instruments including gas-(GC) or liquid chromatography (LC) coupled to mass spectrometry (MS). Especially plant sterol analyses are scarce: most methods attempt to measure total concentrations (combined free and esterified sterols) and this requires a complex and labor intensive sample preparation with multiple manual steps: saponification (base hydrolysis) at elevated temperatures, liquid-liquid extraction using hazardous, chlorinated solvents and/or derivatization are commonly employed (5)(6)(7)(8)(9)(10).…”

Section: Introductionmentioning

confidence: 99%

A Sterol Panel for Multiple Rare Lipid Disorders: Validation and Application for Sitosterolemia, Cerebrotendinous Xanthomatosis and Smith-Lemli-Opitz Syndome

Westbye,

Dizdarevic,

Dahl

et al. 2024

Preprint

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BACKGROUND. Disease-specific sterols accumulate in the blood of patients with several rare lipid disorders. Biochemical measurement of these sterols is important for correct diagnosis and sometimes monitoration of treatment. Existing methods to measure sterols in blood, particularly plant sterols, are often laborious and time consuming. Partly as a result, clinical access to sterol measurements are limited in many parts of the world. METHODS. A simple and rapid method to extract free sterols from human serum and quantitate their concentration using isotope-dilution liquid chromatography tandem mass spectrometry (LC-MS/MS) without derivatization was developed. The method was designed to be “compatible” with routine workflows (eg. 96-well format) in a clinical lab and was extensively validated. Serum from 73 controls were analyzed and used to estimate the upper reference limits for sitosterol, campesterol, stigmasterol, desmosterol, 7-dehydrocholesterol (7DHC), lathosterol and cholestanol. Serum from patients with the rare lipid disorders sitosterolemia (n=7), Smith-Lemli-Optiz syndrome (SLOS; n=1) and cerebrotendinous xanthomatosis (CTX; n=1) were analyzed. RESULTS. All seven sitosterolemia patients were measured to have greatly elevated levels of free plant sterols (sitosterol, campesterol and stigmasterol) compared to the controls. The SLOS serum contained massively increased concentrations of 7DHC and an unidentified compound (likely 8-dehydrocholesterol). CTX serum contained greatly increased concentrations of cholestanol, as well as 7DHC and lathosterol. Spiking experiments indicated that the method is likely also useful in the diagnosis of desmosterolosis and lathosterolosis. CONCLUSION. The reported method is a relatively simple and fast method capable of quantitating diagnostically important sterols and differentiating patients with several rare lipid disorders from controls.

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Age-related reference intervals for serum phytosterols in children by gas chromatography-mass spectrometry and its application in diagnosing sitosterolemia

Cited by 2 publications

References 27 publications

Systematic Metabolic Profiling of Mice with Sleep‐Deprivation

Systematic Metabolic Profiling of Mice with Sleep‐Deprivation

A Sterol Panel for Multiple Rare Lipid Disorders: Validation and Application for Sitosterolemia, Cerebrotendinous Xanthomatosis and Smith-Lemli-Opitz Syndome

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