Abstract
Retinitis pigmentosa (RP) is the predominant form of inherited retinal degenerations (IRDs) caused by abnormalities and loss of photoreceptor cells ensuing diminishment of vision. RP is a heterogenous genetic disorder associated with mutations in over 80 genes, showing various inheritance patterns. Laboratory mouse models are important for our understanding of disease mechanisms, modifier effects, and development of therapeutic modalities. In this review, we have summarized a comprehensive comparison of our previously reported Fam161a knockout (KO) mouse model with other well-studied RP mouse models, Fam161aGT/GT, Pde6brd1, Nr2e3rd7, Rpgrrd9, and Pde6brd10 using structural and functional analysis of the retina. Fam161atm1b/tm1b mouse models are important for developing novel therapies and mainly AAV-based gene therapy and translational read-through-inducing drugs.
Original language | English |
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Title of host publication | Advances in Experimental Medicine and Biology |
Publisher | Springer |
Pages | 365-370 |
Number of pages | 6 |
DOIs | |
State | Published - 2023 |
Publication series
Name | Advances in Experimental Medicine and Biology |
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Volume | 1415 |
ISSN (Print) | 0065-2598 |
ISSN (Electronic) | 2214-8019 |
Bibliographical note
Publisher Copyright:© 2023, The Author(s), under exclusive license to Springer Nature Switzerland AG.
Keywords
- Fam161a
- Knockout
- Mouse model
- Retina
- Retinitis pigmentosa