Accumulation of oxygenated steryl esters in Wolman's disease.
J Lipid Res
; 16(1): 28-38, 1975 Jan.
Article
em En
| MEDLINE
| ID: mdl-162929
ABSTRACT
7alpha- and 7beta-hydroxycholesteryl esters, 7-ketocholesteryl esters, and 5,6alpha- and 5,6beta-epoxycholesteryl esters have been identified in tissues of patients affected by Wolman's disease. Their structural identities were determined by mass spectroscopy and nuclear magnetic resonance spectroscopy and confirmed by chemical synthesis. It is postulated that cholesteryl ester hydrolase deficiency in Wolman's disease might lead to accumulation of oxygenated steryl esters in vivo and impairment of bile acid formation.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Ácidos Esteáricos
/
Xantomatose
/
Colesterol
/
Lipidoses
Limite:
Adult
/
Female
/
Humans
/
Infant
Idioma:
En
Revista:
J Lipid Res
Ano de publicação:
1975
Tipo de documento:
Article