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Adv Exp Med Biol. 2014;801:637-45. doi: 10.1007/978-1-4614-3209-8_80.

Very long chain polyunsaturated fatty acids and rod cell structure and function.

Author information

1
Department of Cell Biology, University of Oklahoma Health Sciences Center, 73104, Oklahoma City, OK, USA, leamarchette@ouhsc.edu.

Abstract

The gene encoding Elongation of Very Long Chain Fatty Acids-4 (ELOVL4) is mutated in patients with autosomal dominant Stargardt's Macular Dystrophy Type 3 (STDG3). ELOVL4 catalyzes the initial condensation step in the elongation of polyunsaturated fatty acids (PUFA) containing more than 26 carbons (26C) to very long chain PUFA (VLC-PUFA; C28 and greater). To investigate the role of VLC-PUFA in rod photoreceptors, we generated mice with rod-specific deletion of Elovl4 (RcKO). The mosaic deletion of rod-expressed ELOVL4 protein resulted in a 36 % lower amount of VLC-PUFA in the retinal phosphatidylcholine (PC) fraction compared to retinas from wild-type mice. However, this reduction was not sufficient to cause rod dysfunction at 7 months or photoreceptor degeneration at 9 or 15 months.

PMID:
24664753
PMCID:
PMC4456017
DOI:
10.1007/978-1-4614-3209-8_80
[Indexed for MEDLINE]
Free PMC Article

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