Abstract | BACKGROUND: METHODS: 7DHC levels and PON1 arylesterase activities were measured spectrophotometrically in 11 SLOS patients and 10 healthy children. Lipoprotein subfractions were detected by polyacrylamide gel electrophoresis. RESULTS: Compared to controls, there was a shift towards the small-dense LDL subfraction and the large HDL subfraction in SLOS. PON1 arylesterase activity was significantly decreased in SLOS patients and correlated negatively with the proportion of small-dense LDL subfraction and the proportion of large HDL subfraction. Significant positive correlations were detected between PON1 arylesterase activity and the ratios of intermediate and small HDL subfractions. CONCLUSIONS: Decreased PON1 activity and the deleterious shift in the distribution of lipoprotein subfractions may contribute to the impaired antioxidant status observed in SLOS. Monitoring of serum PON1 arylesterase activity may be a complementary biomarker in SLOS.
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Authors | Hajnalka Lőrincz, Mariann Harangi, Anna V Oláh, Gabriella P Szabó, Péter Fülöp, Sándor Somodi, György Paragh, Ildikó Seres |
Journal | Pediatric research
(Pediatr Res)
Vol. 77
Issue 5
Pg. 703-9
(May 2015)
ISSN: 1530-0447 [Electronic] United States |
PMID | 25668223
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Antioxidants
- Biomarkers
- Cholesterol, HDL
- Lipids
- Lipoproteins, HDL
- Oxidoreductases Acting on CH-CH Group Donors
- 7-dehydrocholesterol reductase
- Aryldialkylphosphatase
- PON1 protein, human
- Oxygen
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Topics |
- Antioxidants
(metabolism)
- Aryldialkylphosphatase
(blood)
- Biomarkers
(blood)
- Child
- Child, Preschool
- Cholesterol, HDL
(blood)
- Female
- Humans
- Lipids
(blood)
- Lipoproteins, HDL
(blood)
- Male
- Oxidative Stress
- Oxidoreductases Acting on CH-CH Group Donors
(blood)
- Oxygen
(chemistry)
- Smith-Lemli-Opitz Syndrome
(blood)
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