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Very Long Chain Polyunsaturated Fatty Acids and Rod Cell Structure and Function

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Retinal Degenerative Diseases

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 801))

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.

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Acknowledgments

This work was supported by NIH Grants EY00871, EY04149, EY12190, EY21725, EY22071 and RR17703; Foundation Fighting Blindness, Inc., and Research to Prevent Blindness, Inc.

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Authors and Affiliations

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

    L. D. Marchette, D. M. Sherry & Robert E. Anderson

  2. Department of Ophthalmology, University of Oklahoma Health Sciences Center, 73104, Oklahoma City, OK, USA

    R. S. Brush, M. Chan, Robert E. Anderson & N. A. Mandal PhD

  3. Oklahoma Center for Neuroscience, University of Oklahoma Health Sciences Center, 73104, Oklahoma City, OK, USA

    D. M. Sherry

  4. Department of Pharmaceutical Sciences, University of Oklahoma Health Sciences Center, 73104, Oklahoma City, OK, USA

    D. M. Sherry

  5. Dean McGee Eye Institute, University of Oklahoma Health Sciences Center, 608 Stanton L. Young Blvd., 73104, Oklahoma City, OK, USA

    L. D. Marchette, R. S. Brush, M. Chan, Robert E. Anderson & N. A. Mandal PhD

  6. Amherst College, Amherst, MA, USA

    Y. Wen

  7. University of Florida, Gainesville, FL, USA

    J. Wang & John D. Ash

Authors
  1. L. D. Marchette
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  2. D. M. Sherry
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  3. R. S. Brush
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  4. M. Chan
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  5. Y. Wen
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  6. J. Wang
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  7. John D. Ash
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  8. Robert E. Anderson
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  9. N. A. Mandal PhD
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Corresponding author

Correspondence to N. A. Mandal PhD .

Editor information

Editors and Affiliations

  1. Department of Ophthalmology, University of Florida, Gainesville, Florida, USA

    John D. Ash

  2. University Hospital Zurich, Zurich, Switzerland

    Christian Grimm

  3. Cole Eye Institute at the Cleveland Clin Division of Ophthalmology, Cleveland, Ohio, USA

    Joe G. Hollyfield

  4. Dean A. McGee Eye Inst., University of Oklahoma Health Science Center, Oklahoma City, Oklahoma, USA

    Robert E. Anderson

  5. Beckman Vision Center, University of California, San Francisco School of Medicine, San Francisco, California, USA

    Matthew M. LaVail

  6. Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina, USA

    Catherine Bowes Rickman

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Marchette, L. et al. (2014). Very Long Chain Polyunsaturated Fatty Acids and Rod Cell Structure and Function. In: Ash, J., Grimm, C., Hollyfield, J., Anderson, R., LaVail, M., Bowes Rickman, C. (eds) Retinal Degenerative Diseases. Advances in Experimental Medicine and Biology, vol 801. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-3209-8_80

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  • DOI: https://doi.org/10.1007/978-1-4614-3209-8_80

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-3208-1

  • Online ISBN: 978-1-4614-3209-8

  • eBook Packages: MedicineMedicine (R0)

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