Abstract:
:Recent studies suggest that 7-dehydrocholesterol (7-DHC)-derived oxysterols play important roles in the pathophysiology of Smith-Lemli-Opitz syndrome (SLOS), a metabolic disorder that is caused by defective 3β-hydroxysterol-Δ(7)-reductase (DHCR7). Although 14 oxysterols have been identified as the primary products of 7-DHC autoxidation in organic solution, the metabolic fate of these oxysterols in a biological environment has not yet been elucidated. Therefore, we incubated these primary 7-DHC oxysterols in control Neuro2a and control human fibroblast cells and identified metabolites of these oxysterols by HPLC-MS. We also incubated Dhcr7-deficient Neuro2a cells and fibroblasts from SLOS patients with isotopically labeled 7-DHC (d(7)-7-DHC). The observation of matching d(0)- and d(7) peaks in HPLC-MS confirmed the presence of true metabolites of 7-DHC after excluding the possibility of ex vivo oxidation. The metabolites of primary 7-DHC oxysterols were found to contribute to the majority of the metabolic profile of 7-DHC in cells. Furthermore, based on this new data, we identified three new 7-DHC-derived metabolites in the brain of Dhcr7-KO mice. Our studies suggest that 7-DHC peroxidation is a major source of oxysterols observed in cells and in vivo and that the stable metabolites of primary 7-DHC oxysterols can be used as markers of 7-DHC peroxidation in these biological systems.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Xu L,Korade Z,Rosado DA Jr,Mirnics K,Porter NAdoi
10.1194/jlr.M035733subject
Has Abstractpub_date
2013-04-01 00:00:00pages
1135-43issue
4eissn
0022-2275issn
1539-7262pii
jlr.M035733journal_volume
54pub_type
杂志文章abstract::Using [13C]-tracers and direct fetal doses, we show for the first time that the fetal primate converts alpha-linolenic acid (18:3) to docosahexaenoic acid (22:6) in vivo, and we estimate the relative bioefficacy of the two substrates for brain 22:6 accretion. Pregnant female baboons consumed a diet free of long chain ...
journal_title:Journal of lipid research
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abstract::In mammals, the apolipoprotein (apo) A-I gene is expressed predominantly in liver and intestine, while in avian species it is expressed in all tissues. Although liver and intestine are the major sites of chicken apoA-I mRNA synthesis, there are appreciable amounts of apoA-I mRNA in kidney, ovary/testes, brain, lung, s...
journal_title:Journal of lipid research
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journal_title:Journal of lipid research
pub_type: 杂志文章,评审
doi:
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abstract::Rat spermatogenic cells contain sphingomyelins (SMs) and ceramides (Cers) with very long-chain PUFAs (VLCPUFAs) in nonhydroxylated (n-V) and 2-hydroxylated (h-V) forms. How these atypical species distribute among membrane fractions during differentiation was investigated here using a detergent-free procedure to isolat...
journal_title:Journal of lipid research
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doi:10.1194/jlr.M072595
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journal_title:Journal of lipid research
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更新日期:1982-05-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1992-03-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1986-06-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:2001-01-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
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更新日期:1998-12-01 00:00:00
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journal_title:Journal of lipid research
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更新日期:2001-11-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1966-05-01 00:00:00
abstract::We have developed a simplified and improved high performance liquid chromatography (HPLC) method for the detection and quantitation of tissue lipid using a new laser light-scattering detector (Varex model ELSD II). This detector has a limit of sensitivity of 50 ng for neutral lipid and 200 ng for most phospholipids wi...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1990-11-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1991-05-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1976-05-01 00:00:00
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journal_title:Journal of lipid research
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doi:10.1194/jlr.M800058-JLR200
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
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journal_title:Journal of lipid research
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doi:
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journal_title:Journal of lipid research
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journal_title:Journal of lipid research
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doi:
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journal_title:Journal of lipid research
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更新日期:1996-12-01 00:00:00
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journal_title:Journal of lipid research
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更新日期:1993-07-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
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更新日期:2011-05-01 00:00:00