2015
DOI: 10.1007/978-3-319-17121-0_19
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Mouse Models of Stargardt 3 Dominant Macular Degeneration

Abstract: Stargardt type 3 macular degeneration is dependent on a dominant defect in a single gene, ELOVL4 (elongase of very long chain fatty acids 4). The encoded enzyme, ELOVL4, is required for the synthesis of very long chain polyunsaturated fatty acids (VLC-PUFAs), a rare class of > C24 lipids. In vitro expression studies suggest that mutated ELOVL4STGD3 proteins fold improperly, resulting in ER stress and formation of cytosolic aggresomes of wild type and mutant ELOVL4. Although a number of mouse models have been d… Show more

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Cited by 3 publications
(2 citation statements)
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“…FE was not significantly different between groups at lower spatial frequency (0.033 cycle/degree). In our hands DBA/2J mice did not show a consistent OKHR at baseline which is in accordance with the literature 37. These results were excluded from further analysis.…”
Section: Resultssupporting
confidence: 92%
See 1 more Smart Citation
“…FE was not significantly different between groups at lower spatial frequency (0.033 cycle/degree). In our hands DBA/2J mice did not show a consistent OKHR at baseline which is in accordance with the literature 37. These results were excluded from further analysis.…”
Section: Resultssupporting
confidence: 92%
“…In our hands DBA/2J mice did not show a consistent OKHR at baseline which is in accordance with the literature. 37 These results were excluded from further analysis.…”
Section: Resultsmentioning
confidence: 99%