Characterization of native retinal fluorophores involved in biosynthesis of A2E and lipofuscin-associated retinopathies

The Journal of Biological Chemistry
Tam V BuiN L Mata

Abstract

Mutations in the photoreceptor-specific ABCA4 gene are associated with several inherited retinal and macular degenerations. A prominent phenotype of these diseases is the accumulation of cytotoxic lipofuscin fluorophores such as A2E within the retinal pigment epithelium. Another compound, dihydro-N-retinylidene-N-retinylphosphatidyl-ethanolamine (A2PE-H(2)), also accumulates in retinas of mice and humans harboring ABCA4 mutations and was proposed to be a precursor of A2E. The role of A2PE-H(2) in the biogenesis of A2E and its relationship to other retinal fluorophores has not been previously investigated. We report spectral properties and structural relationships of the principal retinal fluorophores that accumulate in retina and retinal pigment epithelium of abca4(-/-) mice. A long wavelength fluorescence emission intrinsic to abca4(-/-) retinal explants is shown to emanate from A2PE-H(2). All-trans retinal dimer conjugates, which were also identified in the retinal explants, possessed distinct fluorescence and structural properties and, unlike A2PE-H(2), did not accumulate in an age-dependent manner. Derivative absorbance and fluorescence spectroscopy revealed that A2PE-H(2), A2E, and N-retinylidene-N-retinyl-phosphatidyletha...Continue Reading

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