Macular pigment and lutein supplementation in choroideremia.

Abstract

Choroideremia is an incurable X-linked retinal degeneration caused by mutations in the gene encoding Rab escort protein-1. A group of clinically defined and genotyped patients were studied to determine: (1) the degree of rod and cone dysfunction and structural abnormality in the central retina and the level of macular pigment; and (2) the response of macular pigment and foveal vision to a 6 month trial of supplementation with oral lutein (at 20 mg per day). Rod and cone-mediated function was measured with dark-adapted static perimetry; in vivo retinal structure was determined with optical coherence tomography; and macular pigment optical density was measured with heterochromatic flicker photometry. In this cohort of patients (ages 15-65 years), both rod- and cone-mediated central function declined with age as did central retinal thickness. Macular pigment levels did not differ between patients and male control subjects. Supplementation of oral lutein in a subset of patients led to an increase in serum lutein and macular pigment levels; absolute foveal sensitivity did not change. It is concluded that macular pigment density can be augmented by oral intake of lutein in patients with choroideremia. There was no short-term change in the central vision of the patients on the supplement, but long-term influences of lutein supplementation on disease natural history warrant further study.

• Authors: Jacque L Duncan, Tomas S Aleman, Leigh M Gardner, Elaine De Castro, Daniel A Marks, Jessica M Emmons, Michelle L Bieber, Janet D Steinberg, Jean Bennett, Edwin M Stone, Ian M MacDonald, Artur V Cideciyan, Maureen G Maguire, Samuel G Jacobson
• Journal: Experimental eye research (Exp Eye Res) Vol. 74 Issue 3 Pg. 371-81 (Mar 2002) ISSN: 0014-4835 [Print] England
• PMID: 12014918 (Publication Type: Clinical Trial, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
• Copyright: Copyright 2002 Elsevier Science Ltd.

Chemical References

• Retinal Pigments
• Lutein

Topics

• Adolescent
• Adult
• Aged
• Aging (physiology)
• Choroideremia (drug therapy, metabolism, physiopathology)
• Dietary Supplements
• Follow-Up Studies
• Humans
• Lutein (therapeutic use)
• Macula Lutea (metabolism)
• Male
• Middle Aged
• Photoreceptor Cells, Vertebrate (physiology)
• Retinal Pigments (metabolism)
• Visual Acuity
• Visual Field Tests