Retinal very long-chain PUFAs

New insights from studies on ELOVL4 protein

Martin Paul Agbaga, Nawajes Mandal, Robert E. Anderson

Research output: Contribution to journalReview article

76 Citations (Scopus)

Abstract

Compared with other mammalian tissues, retina is highly enriched in PUFA. Long-chain PUFA (LC-PUFA; C18-C24) are essential FAs that are enriched in the retina and are necessary for maintenance of normal retinal development and function. The retina, brain, and sperm also contain very LC-PUFA (VLC-PUFA; >C24). Although VLC-PUFA were discovered more than two decades ago, very little is known about their biosynthesis and functional roles in the retina. This is due mainly to intrinsic difficulties associated with working on these unusually long polyunsaturated hydrocarbon chains and their existence in small amounts. Recent studies on the FA elongase elongation of very long chain fatty acids-4 (ELOVL4) protein, however, suggest that VLC-PUFA probably play some uniquely important roles in the retina as well as the other tissues. Mutations in the ELOVL4 gene are found in patients with autosomal dominant Stargardt disease.jlr Here, we review the recent literature on VLC-PUFA with special emphasis on the elongases responsible for their synthesis. We focus on a novel elongase, ELOVL4, involved in the synthesis of VLC-PUFA, and the importance of these FAs in maintaining the structural and functional integrity of retinal photoreceptors.

Original languageEnglish (US)
Pages (from-to)1624-1642
Number of pages19
JournalJournal of Lipid Research
Volume51
Issue number7
DOIs
StatePublished - Jul 1 2010
Externally publishedYes

Fingerprint

Retina
Tissue
Biosynthesis
Hydrocarbons
Elongation
Brain
Proteins
Fatty Acids
Genes
Vertebrate Photoreceptor Cells
Spermatozoa
Maintenance
Mutation

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Endocrinology
  • Cell Biology

Cite this

Retinal very long-chain PUFAs : New insights from studies on ELOVL4 protein. / Agbaga, Martin Paul; Mandal, Nawajes; Anderson, Robert E.

In: Journal of Lipid Research, Vol. 51, No. 7, 01.07.2010, p. 1624-1642.

Research output: Contribution to journalReview article

Agbaga, Martin Paul ; Mandal, Nawajes ; Anderson, Robert E. / Retinal very long-chain PUFAs : New insights from studies on ELOVL4 protein. In: Journal of Lipid Research. 2010 ; Vol. 51, No. 7. pp. 1624-1642.
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