Abstract | BACKGROUND: Patients with abetalipoproteinemia develop progressive ataxic neuropathy and retinopathy that are thought to be due, in part, to oxidative damage resulting from deficiencies of vitamins E and A. OBJECTIVE: DESIGN: Ten patients aged 3-25 y were studied. Assessed were plasma carbonyl concentrations as a marker of oxidative damage to proteins; total plasma oxidizability, which was used to evaluate the susceptibility of plasma lipoproteins to oxidation; and cyclic voltammetry, which represents the overall reducing and antioxidant capacity stemming from low-molecular-weight antioxidants in plasma. RESULTS: Concentrations of plasma carbonyls did not differ significantly between patients and control subjects ( +/- SE: 0.5670 +/- 0.031 and 0.5039 +/- 0.0134 nmol/mg protein, respectively). The lag phase of plasma oxidizability was 28.03 +/- 3.16 min in the patients and 24.0 +/- 2.79 min in healthy subjects in whom oxidizability of isolated HDL was measured (NS). Cyclic voltammetry showed a peak potential of 330 +/- 8.3 mV in all samples studied, denoting that the same antioxidants were present in the plasma of the patients and the control subjects. The anodic current of the samples, a measure of the concentration of hydrophilic low-molecular-weight antioxidants, was 5.227 +/- 0.25 and 5.38 +/- 0.20 micro A in the patients and the control subjects, respectively (NS). CONCLUSION: Enhanced oxidative stress is not apparent in the plasma of abetalipoproteinemia patients receiving long-term supplementation with vitamins E and A.
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Authors | Esther Granot, Ron Kohen |
Journal | The American journal of clinical nutrition
(Am J Clin Nutr)
Vol. 79
Issue 2
Pg. 226-30
(Feb 2004)
ISSN: 0002-9165 [Print] United States |
PMID | 14749227
(Publication Type: Journal Article)
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Chemical References |
- beta Carotene
- Vitamin A
- Vitamin E
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Topics |
- Abetalipoproteinemia
(blood, drug therapy)
- Adolescent
- Adult
- Case-Control Studies
- Child
- Child, Preschool
- Humans
- Oxidative Stress
- Reference Values
- Vitamin A
(therapeutic use)
- Vitamin E
(blood, therapeutic use)
- beta Carotene
(blood)
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