Febs Journal, vol.283, pp.64, 2017 (SCI-Expanded)
Sickle cell disease (SCD) occurs due to a point mutation in the hemoglobin gene. It
is characterized by anemia, vasooclusion and chronic inflammation.
Hypocholesterolemia is a common finding in SCD patients; however, there are only a
few studies to explain its etiology. We had previously shown that SCD patients had a
negative correlation between their plasma cholesterol and erythrocyte
malonyldialdehyde levels. We hypothesized that increased cholesterol oxidation may be
a factor for hypocholesterolemia in SCD.
MATERIAL AND METHODS
The study consisted of pediatric SCD patients (N=22) and healthy controls (N=8). The
patients hadn’t had any crisis for the last three months except two. Blood samples
were drawn into EDTA tubes to separate plasma. Levels of holesterol oxidation
products, 7-ketocholesterol and Colestane-3β,5α,6β-triol were measured by LCMS/MS with the method by Griffiths et al. Plasma total cholesterol levels were
measured by commercial kits. Statistical analysis was performed with Graphpad Prism
This study was approved by the Institutional Review Board of Mersin University.
Mean 7-ketocholesterol levels were 10.47±1.83 ng/ml in patients and 8.97±1.05 ng/ml
in controls (P=0.0298). Mean Colestane-3β,5α,6β-triol levels were 6.49±2.31 in
patients and 5.69±2.69 in controls (P=0.4283). Mean Cholesterol levels were
106±19.1 mg/dL in patients and 149.6±28.9 in controls (P<0.0001).
We found significantly lower plasma cholesterol and higher 7-ketocholesterol levels
in SCD patients than controls. We suggest that increased oxidative stress should be
considered as a factor contributing to hypocholesterolemia in SCD besides hemolysis
and inflammation suggested by two previous studies. This is the first report
investigating plasma oxysterol levels in SCD patients in literature. Further studies
are needed to understand the effects of hypocholesterolemia and increased cholesterol
oxidation products in SCD.