This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The Retinol Dehydrogenases RDH11 and RDH12 are closely related microsomal oxidoreductases located in photoreceptor inner segments. Mutations in the human RDH12 gene induce the most severe and early onset type of inherited retinal degeneration, called Leber Congenital Amaurosis Type 3 (LCA3). We are investigating the function of this enzyme and the molecular mechanisms leading to vision loss and photoreceptor cell death in RDH12-induced LCA. We are specifically interested by the detoxification role of RDH12, and of other retinol dehydrogenases such as RDH11, RDH13, and DHRS3, all located in photoreceptor cells, in different subcellular compartments. We have recently discovered that RDH12 can detoxify damaging aldehydes produced by lipid peroxidation in photoreceptor cells. Such endogenous protection might be necessary to maintain the functional integrity of photoreceptor cells. This hypothesis predicts that photoreceptors die in LCA3 patients due to an absence of this detoxification activity.

National Institute of Health (NIH)
National Center for Research Resources (NCRR)
Exploratory Grants (P20)
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Special Emphasis Panel (ZRR1-RI-5 (01))
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University of Oklahoma Health Sciences Center
Schools of Medicine
Oklahoma City
United States
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Bhatti, Faizah; Kung, Johannes W; Vieira, Frederico (2018) Retinal degeneration mutation in Sftpa1tm1Kor/J and Sftpd -/- targeted mice. PLoS One 13:e0199824
Vieira, Frederico; Kung, Johannes W; Bhatti, Faizah (2017) Structure, genetics and function of the pulmonary associated surfactant proteins A and D: The extra-pulmonary role of these C type lectins. Ann Anat 211:184-201
Simón, María Victoria; Agnolazza, Daniela L; German, Olga Lorena et al. (2016) Synthesis of docosahexaenoic acid from eicosapentaenoic acid in retina neurons protects photoreceptors from oxidative stress. J Neurochem 136:931-46
Stiles, Megan; Qi, Hui; Sun, Eleanor et al. (2016) Sphingolipid profile alters in retinal dystrophic P23H-1 rats and systemic FTY720 can delay retinal degeneration. J Lipid Res 57:818-31
Bennett, Lea D; Anderson, Robert E (2016) Current Progress in Deciphering Importance of VLC-PUFA in the Retina. Adv Exp Med Biol 854:145-51
Ding, Xi-Qin; Thapa, Arjun; Ma, Hongwei et al. (2016) The B3 Subunit of the Cone Cyclic Nucleotide-gated Channel Regulates the Light Responses of Cones and Contributes to the Channel Structural Flexibility. J Biol Chem 291:8721-34
Ma, Hongwei; Ding, Xi-Qin (2016) Thyroid Hormone Signaling and Cone Photoreceptor Viability. Adv Exp Med Biol 854:613-8
Cai, Xue; Chen, Lijuan; McGinnis, James F (2015) Correlation of ER stress and retinal degeneration in tubby mice. Exp Eye Res 140:130-138
Bhatti, Faizah; Ball, Genevieve; Hobbs, Ronald et al. (2015) Pulmonary surfactant protein a is expressed in mouse retina by Müller cells and impacts neovascularization in oxygen-induced retinopathy. Invest Ophthalmol Vis Sci 56:232-42
Parkunan, Salai Madhumathi; Astley, Roger; Callegan, Michelle C (2014) Role of TLR5 and flagella in bacillus intraocular infection. PLoS One 9:e100543

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