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Published on: December 22, 2014
Postnatal expression and distribution of Refsum disease gene associated protein in the rat retina and visual cortex:
Kyu Youn Ahn1, Kwang Il Nam, Baik Yoon Kim
1Research Institute of Medical Sciences, Chonnam National University Medical School, Hak-Dong 5, Dong-Ku, Kwangju 501-190, South Korea.
Insights
Visual stimulation is crucial for maintaining phytanoyl-CoA alpha-hydroxylase-associated protein 1 (PAHX-AP1) expression in the retina and visual cortex. Light exposure influences PAHX-AP1 levels, suggesting its role in photoreceptor development and function.
Area of Science:
- Neuroscience
- Developmental Biology
- Molecular Biology
Background:
- Phytanoyl-CoA alpha-hydroxylase-associated protein 1 (PAHX-AP1) is a neuron-specific protein interacting with the Refsum disease gene PAHX.
- PAHX-AP1 expression increases in the brain post-eyelid opening and is maintained into adulthood.
Purpose of the Study:
- To investigate the hypothesis that light triggers increased PAHX-AP1 expression.
- To examine the developmental distribution of PAHX-AP1 in rat retina and visual cortex.
- To assess changes in PAHX-AP1 expression following binocular deprivation.
Main Methods:
- Northern blot analysis to quantify PAHX-AP1 expression levels.
- In situ hybridization to determine the cellular localization of PAHX-AP1.
- Binocular deprivation to assess the impact of visual stimulation.
Main Results:
- PAHX-AP1 expression peaked in the visual cortex and eyeball at 4 weeks post-birth and remained elevated in adulthood.
- Visual deprivation led to decreased PAHX-AP1 expression in the eyeball and visual cortex.
- PAHX-AP1 localization shifted in the retina from ganglionic cells to inner and outer nuclear layers, with expression decreasing in all layers, including photoreceptors, after deprivation.
Conclusions:
- Visual stimulation is essential for maintaining PAHX-AP1 expression in retinal photoreceptor cells and the visual cortex.
- PAHX-AP1 may play a role in the developmental regulation of photoreceptor function.
Abstract:
Previously, phytanoyl-CoA alpha-hydroxylase-associated protein 1 (PAHX-AP1) was isolated as a novel neuron-specific protein to interact with Refsum disease (RfD) gene PAHX. Its expression in the brain increased after eyelid opening, and the elevated level was maintained through adulthood. In this report, to verify the hypothesis that light could trigger this increase, we have examined the developmental distribution pattern of PAHX-AP1 in rat retina and visual cortex, and changes of its expression by binocular deprivation. Northern blot analyses demonstrated PAHX-AP1 expression reached its highest level in the visual cortex and eyeball at 4 weeks after birth, and these levels were maintained through adult life. Two weeks after visual deprivation, its expression in the eyeball and visual cortex decreased compared with the control. In situ hybridization analyses of the retina showed that PAHX-AP1 expression was limited to the ganglionic cell layer at 10 days after birth, but expressed in the inner nuclear cell layer and extended to the outer nuclear cell layer at 2 and 3 weeks after birth, respectively. Two weeks after visual deprivation, however, it decreased in the ganglionic and inner nuclear cell layer, and disappeared in the rod and cone cell layers. In the visual cortex, strong signals of PAHX-AP1 were detected in layers IV and VI, and II-VI at 10 days and 2 weeks after birth, respectively. Its expression decreased after 2 weeks of visual deprivation. These results indicate that visual stimulation is essential for the maintenance of PAHX-AP1 expressions in the retina, especially in the rod and cone cell layers, and visual cortex, and suggest that PAHX-AP1 may be involved in the developmental regulation of the photoreceptor's function.

