title: Role of long-chain and very-long-chain polyunsaturated fatty acids in macular degenerations and dystrophies.
abstract: Macular degeneration is a progressive, bilateral eye disorder that damages the macula of the human eye. The most common form of macular degeneration is age-related macular degeneration (AMD), which is the leading cause of irreversible blindness in people older than 50 years in developed countries. Autosomal dominant Stargardt disease-3 (STGD3) is an inherited macular dystrophy that has clinical features similar to dry AMD, but occurs at a much earlier age. It is caused by a mutation in the elongation of very-long-chain fatty acids-like 4 (ELOVL4) gene, which is responsible for encoding the elongase enzyme that converts shorter chain fatty acids into C28-C38very long-chain polyunsaturated fatty acids (VLCPUFAs, total number of carbons ≥24). Diets rich in long-chain polyunsaturated fatty acids (LCPUFAs) have inverse associations with the progression of AMD and STGD3, and a deficiency in retinal LCPUFAs and VLCPUFAs has been detected in AMD retinas and STGD3 animal models. This article systematically summarizes the roles of LCPUFAs and VLCPUFAs in AMD and STGD3, and discusses future research directions.
authors.affiliation: Department of Ophthalmology and Visual Sciences, 65 Mario Capecchi Drive, Moran Eye Center, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
authors.forename: Aihua
authors.initials: A
authors.lastname: Liu
authors.affiliation: Department of Ophthalmology and Visual Sciences, 65 Mario Capecchi Drive, Moran Eye Center, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
authors.forename: Yanhua
authors.initials: Y
authors.lastname: Lin
authors.affiliation: Department of Ophthalmology and Visual Sciences, 65 Mario Capecchi Drive, Moran Eye Center, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
authors.forename: Ryan
authors.initials: R
authors.lastname: Terry
authors.affiliation: Department of Ophthalmology and Visual Sciences, 65 Mario Capecchi Drive, Moran Eye Center, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
authors.forename: Kelly
authors.initials: K
authors.lastname: Nelson
authors.affiliation: Department of Ophthalmology and Visual Sciences, 65 Mario Capecchi Drive, Moran Eye Center, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
authors.forename: Paul S
authors.initials: PS
authors.lastname: Bernstein
keywords: age-related macular degeneration (AMD); autosomal dominant Stargardt disease-3 (STGD3); elongation of very-long-chain fatty acids-like 4 (ELOVL4); long-chain polyunsaturated fatty acid (LCPUFA); very-long-chain polyunsaturated fatty acid (VLCPUFA)