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Squalene and Noncholesterol Sterols in Serum and Lipoproteins of Children with and without Familial Hypercholesterolemia

Squalene and noncholesterol sterols, e.g. lathosterol and plant sterols, the respective markers of cholesterol synthesis and absorption, are transported with cholesterol in serum lipoproteins. Their concentrations and ratios to cholesterol in serum and lipoproteins have not been carefully compared,... Full description

Journal Title: Pediatric research 2003, Vol.53 (4), p.648-653
Main Author: KETOMÄKI, Anna
Other Authors: GYLLING, Helena , SIIMES, Martti A , VUORIO, Alpo , MIETTINEN, Tatu A
Format: Electronic Article Electronic Article
Language: English
Subjects:
Publisher: Hagerstown, MD: Lippincott Williams & Wilkins
ID: ISSN: 0031-3998
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recordid: cdi_proquest_miscellaneous_73145240
title: Squalene and Noncholesterol Sterols in Serum and Lipoproteins of Children with and without Familial Hypercholesterolemia
format: Article
creator:
  • KETOMÄKI, Anna
  • GYLLING, Helena
  • SIIMES, Martti A
  • VUORIO, Alpo
  • MIETTINEN, Tatu A
subjects:
  • Adolescent
  • Apolipoproteins E - genetics
  • Biological and medical sciences
  • Child
  • Child, Preschool
  • Cholesterol - blood
  • Cholesterol, HDL - blood
  • Cholesterol, LDL - blood
  • Cholesterol, VLDL - blood
  • Disorders of blood lipids. Hyperlipoproteinemia
  • Errors of metabolism
  • Female
  • Heterozygote
  • Humans
  • Hyperlipoproteinemia Type II - blood
  • Hyperlipoproteinemia Type II - genetics
  • Lipids (lysosomal enzyme disorders, storage diseases)
  • Lipoproteins - blood
  • Male
  • Medical sciences
  • Metabolic diseases
  • Phenotype
  • Phytosterols - blood
  • Squalene - blood
ispartof: Pediatric research, 2003, Vol.53 (4), p.648-653
description: Squalene and noncholesterol sterols, e.g. lathosterol and plant sterols, the respective markers of cholesterol synthesis and absorption, are transported with cholesterol in serum lipoproteins. Their concentrations and ratios to cholesterol in serum and lipoproteins have not been carefully compared, especially in children and in marked hypercholesterolemia. Thus, we measured these variables with gas-liquid chromatography in 18 children with and 29 without familial hypercholesterolemia, all aged 5-17 y. Concentrations of most noncholesterol sterols were higher in serum, LDL, and intermediate density lipoprotein in the children with than those without familial hypercholesterolemia. Despite accumulation of noncholesterol sterols mainly in LDL (75% in familial hypercholesterolemia and 55% in non-familial hypercholesterolemia, p < 0.001), their ratios were mostly similar in serum and lipoproteins of the two groups. The ratios of squalene and lathosterol were higher in VLDL and intermediate density lipoprotein, whereas in LDL that of lathosterol was lower than the respective serum values in both groups. Absorption marker sterol ratios were highest in HDL in both groups. Thus, even though the ratios of noncholesterol sterols to cholesterol in serum reflect, in general, synthesis and absorption of cholesterol, their ratios in different lipoproteins could give additional information of cholesterol metabolism.
language: eng
source:
identifier: ISSN: 0031-3998
fulltext: no_fulltext
issn:
  • 0031-3998
  • 1530-0447
url: Link


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titleSqualene and Noncholesterol Sterols in Serum and Lipoproteins of Children with and without Familial Hypercholesterolemia
creatorKETOMÄKI, Anna ; GYLLING, Helena ; SIIMES, Martti A ; VUORIO, Alpo ; MIETTINEN, Tatu A
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descriptionSqualene and noncholesterol sterols, e.g. lathosterol and plant sterols, the respective markers of cholesterol synthesis and absorption, are transported with cholesterol in serum lipoproteins. Their concentrations and ratios to cholesterol in serum and lipoproteins have not been carefully compared, especially in children and in marked hypercholesterolemia. Thus, we measured these variables with gas-liquid chromatography in 18 children with and 29 without familial hypercholesterolemia, all aged 5-17 y. Concentrations of most noncholesterol sterols were higher in serum, LDL, and intermediate density lipoprotein in the children with than those without familial hypercholesterolemia. Despite accumulation of noncholesterol sterols mainly in LDL (75% in familial hypercholesterolemia and 55% in non-familial hypercholesterolemia, p < 0.001), their ratios were mostly similar in serum and lipoproteins of the two groups. The ratios of squalene and lathosterol were higher in VLDL and intermediate density lipoprotein, whereas in LDL that of lathosterol was lower than the respective serum values in both groups. Absorption marker sterol ratios were highest in HDL in both groups. Thus, even though the ratios of noncholesterol sterols to cholesterol in serum reflect, in general, synthesis and absorption of cholesterol, their ratios in different lipoproteins could give additional information of cholesterol metabolism.
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languageeng
publisherHagerstown, MD: Lippincott Williams & Wilkins
subjectAdolescent ; Apolipoproteins E - genetics ; Biological and medical sciences ; Child ; Child, Preschool ; Cholesterol - blood ; Cholesterol, HDL - blood ; Cholesterol, LDL - blood ; Cholesterol, VLDL - blood ; Disorders of blood lipids. Hyperlipoproteinemia ; Errors of metabolism ; Female ; Heterozygote ; Humans ; Hyperlipoproteinemia Type II - blood ; Hyperlipoproteinemia Type II - genetics ; Lipids (lysosomal enzyme disorders, storage diseases) ; Lipoproteins - blood ; Male ; Medical sciences ; Metabolic diseases ; Phenotype ; Phytosterols - blood ; Squalene - blood
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descriptionSqualene and noncholesterol sterols, e.g. lathosterol and plant sterols, the respective markers of cholesterol synthesis and absorption, are transported with cholesterol in serum lipoproteins. Their concentrations and ratios to cholesterol in serum and lipoproteins have not been carefully compared, especially in children and in marked hypercholesterolemia. Thus, we measured these variables with gas-liquid chromatography in 18 children with and 29 without familial hypercholesterolemia, all aged 5-17 y. Concentrations of most noncholesterol sterols were higher in serum, LDL, and intermediate density lipoprotein in the children with than those without familial hypercholesterolemia. Despite accumulation of noncholesterol sterols mainly in LDL (75% in familial hypercholesterolemia and 55% in non-familial hypercholesterolemia, p < 0.001), their ratios were mostly similar in serum and lipoproteins of the two groups. The ratios of squalene and lathosterol were higher in VLDL and intermediate density lipoprotein, whereas in LDL that of lathosterol was lower than the respective serum values in both groups. Absorption marker sterol ratios were highest in HDL in both groups. Thus, even though the ratios of noncholesterol sterols to cholesterol in serum reflect, in general, synthesis and absorption of cholesterol, their ratios in different lipoproteins could give additional information of cholesterol metabolism.
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1Apolipoproteins E - genetics
2Biological and medical sciences
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4Child, Preschool
5Cholesterol - blood
6Cholesterol, HDL - blood
7Cholesterol, LDL - blood
8Cholesterol, VLDL - blood
9Disorders of blood lipids. Hyperlipoproteinemia
10Errors of metabolism
11Female
12Heterozygote
13Humans
14Hyperlipoproteinemia Type II - blood
15Hyperlipoproteinemia Type II - genetics
16Lipids (lysosomal enzyme disorders, storage diseases)
17Lipoproteins - blood
18Male
19Medical sciences
20Metabolic diseases
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titleSqualene and Noncholesterol Sterols in Serum and Lipoproteins of Children with and without Familial Hypercholesterolemia
authorKETOMÄKI, Anna ; GYLLING, Helena ; SIIMES, Martti A ; VUORIO, Alpo ; MIETTINEN, Tatu A
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1Apolipoproteins E - genetics
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5Cholesterol - blood
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8Cholesterol, VLDL - blood
9Disorders of blood lipids. Hyperlipoproteinemia
10Errors of metabolism
11Female
12Heterozygote
13Humans
14Hyperlipoproteinemia Type II - blood
15Hyperlipoproteinemia Type II - genetics
16Lipids (lysosomal enzyme disorders, storage diseases)
17Lipoproteins - blood
18Male
19Medical sciences
20Metabolic diseases
21Phenotype
22Phytosterols - blood
23Squalene - blood
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abstractSqualene and noncholesterol sterols, e.g. lathosterol and plant sterols, the respective markers of cholesterol synthesis and absorption, are transported with cholesterol in serum lipoproteins. Their concentrations and ratios to cholesterol in serum and lipoproteins have not been carefully compared, especially in children and in marked hypercholesterolemia. Thus, we measured these variables with gas-liquid chromatography in 18 children with and 29 without familial hypercholesterolemia, all aged 5-17 y. Concentrations of most noncholesterol sterols were higher in serum, LDL, and intermediate density lipoprotein in the children with than those without familial hypercholesterolemia. Despite accumulation of noncholesterol sterols mainly in LDL (75% in familial hypercholesterolemia and 55% in non-familial hypercholesterolemia, p < 0.001), their ratios were mostly similar in serum and lipoproteins of the two groups. The ratios of squalene and lathosterol were higher in VLDL and intermediate density lipoprotein, whereas in LDL that of lathosterol was lower than the respective serum values in both groups. Absorption marker sterol ratios were highest in HDL in both groups. Thus, even though the ratios of noncholesterol sterols to cholesterol in serum reflect, in general, synthesis and absorption of cholesterol, their ratios in different lipoproteins could give additional information of cholesterol metabolism.
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doi10.1203/01.PDR.0000055771.28409.40
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