Defective cholesterol metabolism in amyotrophic lateral sclerosis

As neurons die, cholesterol is released in the central nervous system (CNS); hence, this sterol and its metabolites may represent a biomarker of neurodegeneration, including in amyotrophic lateral sclerosis (ALS), in which altered cholesterol levels have been linked to prognosis. More than 40 differ...

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Published inJournal of lipid research Vol. 58; no. 1; pp. 267 - 278
Main Authors Abdel-Khalik, Jonas, Yutuc, Eylan, Crick, Peter J., Gustafsson, Jan-Åke, Warner, Margaret, Roman, Gustavo, Talbot, Kevin, Gray, Elizabeth, Griffiths, William J., Turner, Martin R., Wang, Yuqin
Format Journal Article
LanguageEnglish
Published United States Elsevier Inc 01.01.2017
Journal of Lipid Research
The American Society for Biochemistry and Molecular Biology
Elsevier
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Abstract As neurons die, cholesterol is released in the central nervous system (CNS); hence, this sterol and its metabolites may represent a biomarker of neurodegeneration, including in amyotrophic lateral sclerosis (ALS), in which altered cholesterol levels have been linked to prognosis. More than 40 different sterols were quantified in serum and cerebrospinal fluid (CSF) from ALS patients and healthy controls. In CSF, the concentration of cholesterol was found to be elevated in ALS samples. When CSF metabolite levels were normalized to cholesterol, the cholesterol metabolite 3β,7α-dihydroxycholest-5-en-26-oic acid, along with its precursor 3β-hydroxycholest-5-en-26-oic acid and product 7α-hydroxy-3-oxocholest-4-en-26-oic acid, were reduced in concentration, whereas metabolites known to be imported from the circulation into the CNS were not found to differ in concentration between groups. Analysis of serum revealed that (25R)26-hydroxycholesterol, the immediate precursor of 3β-hydroxycholest-5-en-26-oic acid, was reduced in concentration in ALS patients compared with controls. We conclude that the acidic branch of bile acid biosynthesis, known to be operative in-part in the brain, is defective in ALS, leading to a failure of the CNS to remove excess cholesterol, which may be toxic to neuronal cells, compounded by a reduction in neuroprotective 3β,7α-dihydroxycholest-5-en-26-oic acid.
AbstractList As neurons die, cholesterol is released in the central nervous system (CNS); hence, this sterol and its metabolites may represent a biomarker of neurodegeneration, including in amyotrophic lateral sclerosis (ALS), in which altered cholesterol levels have been linked to prognosis. More than 40 different sterols were quantified in serum and cerebrospinal fluid (CSF) from ALS patients and healthy controls. In CSF, the concentration of cholesterol was found to be elevated in ALS samples. When CSF metabolite levels were normalized to cholesterol, the cholesterol metabolite 3β,7α-dihydroxycholest-5-en-26-oic acid, along with its precursor 3β-hydroxycholest-5-en-26-oic acid and product 7α-hydroxy-3-oxocholest-4-en-26-oic acid, were reduced in concentration, whereas metabolites known to be imported from the circulation into the CNS were not found to differ in concentration between groups. Analysis of serum revealed that (25R)26-hydroxycholesterol, the immediate precursor of 3β-hydroxycholest-5-en-26-oic acid, was reduced in concentration in ALS patients compared with controls. We conclude that the acidic branch of bile acid biosynthesis, known to be operative in-part in the brain, is defective in ALS, leading to a failure of the CNS to remove excess cholesterol, which may be toxic to neuronal cells, compounded by a reduction in neuroprotective 3β,7α-dihydroxycholest-5-en-26-oic acid.
As neurons die, cholesterol is released in the central nervous system (CNS); hence, this sterol and its metabolites may represent a biomarker of neurodegeneration, including in amyotrophic lateral sclerosis (ALS), in which altered cholesterol levels have been linked to prognosis. More than 40 different sterols were quantified in serum and cerebrospinal fluid (CSF) from ALS patients and healthy controls. In CSF, the concentration of cholesterol was found to be elevated in ALS samples. When CSF metabolite levels were normalized to cholesterol, the cholesterol metabolite 3 beta ,7 alpha -dihydroxycholest-5-en-26-oic acid, along with its precursor 3 beta -hydroxycholest-5-en-26-oic acid and product 7 alpha -hydroxy-3-oxocholest-4-en-26-oic acid, were reduced in concentration, whereas metabolites known to be imported from the circulation into the CNS were not found to differ in concentration between groups. Analysis of serum revealed that (25R)26-hydroxycholesterol, the immediate precursor of 3 beta -hydroxycholest-5-en-26-oic acid, was reduced in concentration in ALS patients compared with controls. We conclude that the acidic branch of bile acid biosynthesis, known to be operative in-part in the brain, is defective in ALS, leading to a failure of the CNS to remove excess cholesterol, which may be toxic to neuronal cells, compounded by a reduction in neuroprotective 3 beta ,7 alpha -dihydroxycholest-5-en-26-oic acid.
Author Crick, Peter J.
Gray, Elizabeth
Roman, Gustavo
Wang, Yuqin
Griffiths, William J.
Yutuc, Eylan
Warner, Margaret
Gustafsson, Jan-Åke
Talbot, Kevin
Abdel-Khalik, Jonas
Turner, Martin R.
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  organization: Department of Biology and Biochemistry, Center for Nuclear Receptors and Cell Signaling, University of Houston, Houston, TX
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  surname: Turner
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  organization: Swansea University Medical School, Swansea, United Kingdom
BackLink https://www.ncbi.nlm.nih.gov/pubmed/27811233$$D View this record in MEDLINE/PubMed
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Issue 1
Keywords cholestenoic acids
brain lipids
neurodeneneration
bile acids and salts/biosynthesis
oxysterols
nuclear receptors/LXR
mass spectrometry
cytochrome P450
Language English
License This is an open access article under the CC BY license.
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Snippet As neurons die, cholesterol is released in the central nervous system (CNS); hence, this sterol and its metabolites may represent a biomarker of...
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SubjectTerms Acids
Aged
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - blood
Amyotrophic Lateral Sclerosis - cerebrospinal fluid
Amyotrophic Lateral Sclerosis - pathology
Bile
Bile Acids and Salts - blood
Bile Acids and Salts - cerebrospinal fluid
Bile Acids and Salts - isolation & purification
bile acids and salts/biosynthesis
brain lipids
Central nervous system
Central Nervous System - metabolism
Central Nervous System - pathology
Cerebrospinal fluid
cholestenoic acids
Cholesterol
Cholesterol - blood
Cholesterol - cerebrospinal fluid
Cholesterol - isolation & purification
cytochrome P450
Female
Humans
Lipid metabolism
Lipids - blood
Lipids - cerebrospinal fluid
Lipids - isolation & purification
Male
mass spectrometry
Metabolites
Middle Aged
Nerve Degeneration - blood
Nerve Degeneration - cerebrospinal fluid
Nerve Degeneration - pathology
Neurodegeneration
neurodeneneration
Neurons - metabolism
Neurons - pathology
Neuroprotection
nuclear receptors/LXR
oxysterols
Patient-Oriented and Epidemiological Research
Sterols
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Title Defective cholesterol metabolism in amyotrophic lateral sclerosis
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