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MicroRNA Expression Profiles of Human iPS Cells, Retinal Pigment Epithelium Derived From iPS, and Fetal Retinal Pigment Epithelium
Published on: June 24, 2014
Altered retinal microRNA expression profile in a mouse model of retinitis pigmentosa
Carol J Loscher1, Karsten Hokamp, Paul F Kenna
1Smurfit Institute of Genetics, Trinity College Dublin, College Green, Dublin 2, Ireland. loschecj@tcd.ie.
Background:
The role played by microRNAs (miRs) as common regulators in physiologic processes such as development and various disease states was recently highlighted. Retinitis pigmentosa (RP) linked to RHO (which encodes rhodopsin) is the most frequent form of inherited retinal degeneration that leads to blindness, for which there are no current therapies. Little is known about the cellular mechanisms that connect mutations within RHO to eventual photoreceptor cell death by apoptosis.
Results:
Global miR expression profiling using miR microarray technology and quantitative real-time RT-PCR (qPCR) was performed in mouse retinas. RNA samples from retina of a mouse model of RP carrying a mutant Pro347Ser RHO transgene and from wild-type retina, brain and a whole-body representation (prepared by pooling total RNA from eight different mouse organs) exhibited notably different miR profiles. Expression of retina-specific and recently described retinal miRs was semi-quantitatively demonstrated in wild-type mouse retina. Alterations greater than twofold were found in the expression of nine miRs in Pro347Ser as compared with wild-type retina (P < 0.05). Expression of miR-1 and miR-133 decreased by more than 2.5-fold (P < 0.001), whereas expression of miR-96 and miR-183 increased by more than 3-fold (P < 0.001) in Pro347Ser retinas, as validated by qPCR. Potential retinal targets for these miRs were predicted in silico.
Conclusion:
This is the first miR microarray study to focus on evaluating altered miR expression in retinal disease. Additionally, novel retinal preference for miR-376a and miR-691 was identified. The results obtained contribute toward elucidating the function of miRs in normal and diseased retina. Modulation of expression of retinal miRs may represent a future therapeutic strategy for retinopathies such as RP.
Insights
MicroRNAs (miRs) show altered expression in retinitis pigmentosa (RP), a leading cause of inherited blindness. This study identifies specific miRs in a mouse model, offering potential therapeutic targets for retinal degeneration.
Area of Science:
- Molecular biology
- Genetics
- Ophthalmology
Background:
- MicroRNAs (miRs) are key regulators in physiological processes and disease.
- Retinitis pigmentosa (RP), an inherited retinal degeneration linked to RHO mutations, leads to blindness with no current therapies.
- Cellular mechanisms connecting RHO mutations to photoreceptor apoptosis remain unclear.
Purpose of the Study:
- To investigate microRNA (miR) expression profiles in a mouse model of RHO-linked retinitis pigmentosa (RP).
- To identify specific miRs with altered expression in diseased retinas.
- To explore the potential of miRs as therapeutic targets for RP.
Main Methods:
- Global microRNA (miR) expression profiling using miR microarray technology.
- Quantitative real-time RT-PCR (qPCR) for validation.
- Analysis of RNA from wild-type and Pro347Ser mutant RHO mouse retinas.
Main Results:
- Significant differences in miR profiles were observed between wild-type and Pro347Ser mutant mouse retinas.
- Nine miRs showed twofold or greater expression changes in the RP model.
- Specific miRs, including miR-1, miR-133, miR-96, and miR-183, exhibited significant alterations validated by qPCR.
Conclusions:
- This is the first miR microarray study on retinal disease, identifying novel retinal miRs (miR-376a, miR-691).
- Altered miR expression patterns provide insights into miR function in normal and diseased retinas.
- Modulating retinal miR expression may offer a future therapeutic strategy for retinopathies like RP.
