Muscle expression of human retinol-binding protein (RBP). Suppression of the visual defect of RBP knockout mice.

Loredana Quadro, William S. Blaner, Leora Hamberger, Russell N. Van Gelder, Silke Vogel, Roseann Piantedosi, Peter Gouras, Vittorio Colantuoni, Max E. Gottesman

Research output: Contribution to journalArticlepeer-review

42 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)30191-30197
Number of pages7
JournalThe Journal of biological chemistry
Volume277
Issue number33
DOIs
StatePublished - Aug 16 2002
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Molecular Biology
  • Cell Biology

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