| Autor: | M.J. Richards, Richard J. Cenedella, Neal S. Peachey, Steven J. Fliesler, Chi Yen Miller |
|---|---|
| Rok vydání: | 2000 |
| Předmět: |
medicine.medical_specialty
Retina medicine.diagnostic_test Cholesterol Retinal Context (language use) General Medicine Biology medicine.disease Biochemistry Sterol Desmosterolosis Cellular and Molecular Neuroscience chemistry.chemical_compound Endocrinology medicine.anatomical_structure chemistry Internal medicine Desmosterol medicine Electroretinography |
| Zdroj: | Neurochemical Research. 25:685-694 |
| ISSN: | 0364-3190 |
| DOI: | 10.1023/a:1007519321917 |
| Popis: | Desmosterolosis is a rare, autosomal recessive, human disease characterized by multiple congenital anomalies in conjunction with grossly elevated levels of desmosterol and markedly reduced levels of cholesterol in all bodily tissues. Herein, we evaluated retinal sterol composition, histology, and electrophysiological function in an animal model that exhibited the biochemical features of desmosterolosis, produced by treating pregnant rats and their progeny with U18666A, an inhibitor of desmosterol reductase. Treated rats had cataracts, were substantially smaller, and had markedly high levels of desmosterol and profoundly low levels of cholesterol in their retinas and other tissues compared to age-matched controls. However, their retinas were histologically normal and electrophysiologically functional. These results suggest that desmosterol may be able to replace cholesterol in the retina, both structurally and functionally. These findings are discussed in the context of "sterol synergism". |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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