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Long-suspected cause of blindness from eye disease disproved
Date:3/11/2013

um into the cell cytosol, which blocks the synthesizing of very long chain fatty acids in photoreceptor cells. But proving that the lack of these fatty acids actually causes blindness has been difficult to show in experiments, because mice in which the ELOVL4 was knocked out did not survive.

Krizaj and his colleagues overcame that problem by engineering mouse models that lacked ELOVL4 only in their photoreceptor cells, allowing the mice to survive but with the fatty acids in those cells reduced up to 90 percent. This allowed them to test directly whether loss of very long chain fatty acids replicates vision loss observed in children with Stargardt's disease. As they report in the journal, electrophysiological and behavioral testing of daytime and night vision in genetically engineered mice showed that sight was not affected despite the dramatic reduction in very long chain fatty acids in photoreceptor cells.

Researchers now must look for a different cause of Stargardt type 3. "If it's not the loss of fatty acids causing the disease, then we'll have to find other strategies to help these kids," Krizaj says.

One possibility, according to Krizaj, is that mutated proteins, escaping from the endoplasmic reticulum are aggregating in the cytoplasm causing large deposits consisting of mutated and normal proteins, which is "almost like causing photoreceptor cell death by blocking intracellular traffic and clogging the cells' drains."


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Contact: Phil Sahm
phil.sahm@hsc.utah.edu
801-581-2517
University of Utah Health Sciences
Source:Eurekalert

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