Role of Elovl4 protein in the biosynthesis of docosahexaenoic acid

Adv Exp Med Biol. 2010:664:233-42. doi: 10.1007/978-1-4419-1399-9_27.

Abstract

The disk membranes of retinal photoreceptor outer segments and other neuronal and reproductive tissues are enriched in docosahexaenoic acid (DHA, 22:6n3), which is essential for their normal function and development. The fatty acid condensing enzyme Elongation of Very Long chain fatty acids-4 (ELOVL4) is highly expressed in retina photoreceptors as well as other tissues with high 22:6n3 content. Mutations in the ELOVL4 gene are associated with autosomal dominant Stargardt-like macular dystrophy (STGD3) and results in synthesis of a truncated protein that cannot be targeted to the endoplasmic reticulum (ER), the site of fatty acid biosynthesis. Considering the abundance and essential roles of 22:6n3 in ELOVL4-expressing tissues (except the skin), it was proposed that the ELOVL4 protein may be involved in 22:6n3 biosynthesis. We tested the hypothesis that the ELOVL4 protein is involved in 22:6n3 biosynthesis by selectively silencing expression of the protein in the cone photoreceptors derived cell line 661 w and showed that the ELOVL4 protein is not involved in DHA biosynthesis from the short chain fatty acid precursors 18:3n3 and 22:5n3.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Line
  • Docosahexaenoic Acids / biosynthesis*
  • Eye Proteins / genetics
  • Eye Proteins / metabolism*
  • Gene Expression Regulation
  • Gene Knockdown Techniques
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism*
  • Mice
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • RNA, Small Interfering / metabolism

Substances

  • Elovl4 protein, mouse
  • Eye Proteins
  • Membrane Proteins
  • RNA, Messenger
  • RNA, Small Interfering
  • Docosahexaenoic Acids