An ideal animal model of retinoblastoma should exhibit all the features of the human form. As a comprehensive supplier of preclinical animal models in ophthalmology, Ace Therapeutics provides a series of genetic mouse retinoblastoma models to customers around the world. These models pave the way for you to develop powerful diagnostic tools, to test the efficacy and to predictive prognostic markers of various treatment modalities.
Retinoblastoma (RB), the most common pediatric ocular malignancy, results from the defective formation of the pRB protein due to the inactivation of two alleles of the retinoblastoma (Rb) gene. pRB is a major tumor suppressor gene involved in cell cycle progression, terminal differentiation, and DNA replication. Loss of pRB activity in retinal progenitor cells leads to impaired cell cycle and uncontrolled cell proliferation. Retinoblastoma presents in unilateral and bilateral forms, depending on whether it is sporadic or familial. The bilateral form is inherited, while the unilateral form is usually non-inherited. In recent years, researchers' understanding of retinoblastoma genetic mechanisms has accelerated early diagnosis and genetic prediction of the disease and developed preclinical animal models that more closely resemble human disease. Studies have shown that retinoblastoma gene knockout with additional loss of p107, p130, p53 and use of Nestin, Chx10, and Pax6 promoter genes shows histological and phenotypic changes similar to human retinoblastoma forms.
Fig. 1. Genetic Models of Retinoblastoma with their key features. (Nair RM, et al., 2013)
Animal models provide a powerful experimental tool to study retinoblast generation and tumorigenesis. Over the past few years, Ace Therapeutics has developed several genetic mouse models of retinoblastoma by gaining insights into the tumor suppressor role of the Rb gene. These models bear a moderate to high resemblance to the human form of the tumor. This helps our clients understand tumor initiation and progression and evaluate treatment effects.
Ace Therapeutics researchers use gene knockout technology to create a genetic mouse model with molecular characteristics similar to human retinoblastoma. Importantly, our genetic mouse model spontaneously develops retinoblastoma, which allows our customers to study early-stage,late-stage tumors, or both. In addition, it is also a powerful tool for validating drug candidates' therapeutic effects and testing drug delivery methods. It has been widely used by our customers with gratifying results.
Ace Therapeutics provides the following genetic mouse models of retinoblastoma to customers around the world, including but not limited to:
Tab.1. Ace Therapeutics' genetic murine models of retinoblastoma.
Genotype | Research objectives |
---|---|
Rb1/p107 DKO | Additional genes required in cooperation with pRb loss for tumorigenesis and early RB. |
Rb1/p130 DKO | Early and advanced RB. |
Rb1/p107/p130 TKO | Very aggressive RB. |
Rb1/p107/p53 TKO | Advanced and aggressive RB. |
Rb1/p107 DKO/ MDMXTg | Advanced and aggressive RB. |
Rb1/p107/Pten TKO | Tumor progression related to the PI3K/AKT pathway. |
Rb1 KO/MYCN | Oncogenic effects of MYCN on RB. |
It is worth mentioning that we also provide rabbit models of retinoblastoma to help you discover and study candidate pathways and therapies for molecularly targeted therapy of human retinoblastoma. Please click the link to access.
With rich experience in the field of retinoblastoma, Ace Therapeutics is confident to help you overcome any upcoming difficulties and challenges. If you are interested in our services or need more detailed information, please feel free to contact us. Our experienced scientists are ready to help you!
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