Muscle expression of human retinol-binding protein (RBP). Suppression of the visual defect of RBP knockout mice |
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Authors: | Quadro Loredana Blaner William S Hamberger Leora Van Gelder Russell N Vogel Silke Piantedosi Roseann Gouras Peter Colantuoni Vittorio Gottesman Max E |
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Institution: | Institute of Cancer Research, Columbia University, College of Physicians and Surgeons, New York, New York 10032, USA. |
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Abstract: | Mice lacking retinol-binding protein (RBP) have low circulating retinol levels. They have severe visual defects due to a low content of retinol or retinyl esters in the eye. A transgenic mouse strain that expresses human RBP under the control of the muscle creatine kinase promoter in the null background was generated. The exogenous protein bound retinol and transthyretin in the circulation and effectively delivered retinol to the eye. Thus, RBP expressed from an ectopic source suppresses the visual phenotype, and retinoids accumulate in the eye. No human RBP was found in the retinal pigment epithelium of the transgenic mice, indicating that retinol uptake by the eye does not entail endocytosis of the carrier RBP. |
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