Muscle expression of human retinol-binding protein (RBP). Suppression of the visual defect of RBP knockout mice.
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.
Quadro
Loredana
L
Institute of Cancer Research, Columbia University, College of Physicians and Surgeons, New York, New York 10032, USA.
Blaner
William S
WS
Hamberger
Leora
L
Van Gelder
Russell N
RN
Vogel
Silke
S
Piantedosi
Roseann
R
Gouras
Peter
P
Colantuoni
Vittorio
V
Gottesman
Max E
ME
D000818:Animals; D002851:Chromatography, High Pressure Liquid; D005260:Female; D006801:Humans; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D011401:Promoter Regions, Genetic; D011994:Recombinant Proteins / Q000235:genetics / Q000378:metabolism; D012177:Retinol-Binding Proteins / Q000235:genetics / Q000502:physiology*; D014801:Vitamin A / Q000097:blood
D011994:Recombinant Proteins; D012177:Retinol-Binding Proteins; D014801:Vitamin A