Literature DB >> 162929

Accumulation of oxygenated steryl esters in Wolman's disease.

G Assmann, D S Fredrickson, H R Sloan, H M Fales, R J Highet.   

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.

Entities:  

Mesh：

Substances：

Year:  1975        PMID： 162929

Source DB:  PubMed          Journal:  J Lipid Res        ISSN： 0022-2275            Impact factor:   5.922


  7 in total

1.  Failure to Thrive: An Expanded Differential Diagnosis.

Authors:  Alexandra Lazzara; Carrie Daymont; Roger Ladda; Jordan Lull; Can Ficicioglu; Jennifer L Cohen; Justen Aprile
Journal:  J Pediatr Genet       Date:  2018-08-31

2.  Cholesterolester accumulation.

Authors:  B J Vermeer; F C Reman
Journal:  Arch Dermatol Res       Date:  1980       Impact factor: 3.017

3.  Analysis and physiologic significance of cholesterol epoxide in animal tissues.

Authors:  H S Black
Journal:  Lipids       Date:  1980-09       Impact factor: 1.880

4.  The metabolic fate of cholesterol-5 alpha, 6 alpha-expoxide in vivo.

Authors:  J P Bowden; G M Muschik; J C Kawalek
Journal:  Lipids       Date:  1979-07       Impact factor: 1.880

5.  New pathogenetic hypothesis for Wolman disease: possible role of oxidized low-density lipoproteins in adrenal necrosis and calcification.

Authors:  G Fitoussi; A Nègre-Salvayre; M T Pieraggi; R Salvayre
Journal:  Biochem J       Date:  1994-07-01       Impact factor: 3.857

6.  Cholestane-3β,5α,6β-triol: high levels in Niemann-Pick type C, cerebrotendinous xanthomatosis, and lysosomal acid lipase deficiency.

Authors:  Sonia Pajares; Angela Arias; Judit García-Villoria; Judit Macías-Vidal; Emilio Ros; Javier de las Heras; Marisa Girós; Maria J Coll; Antonia Ribes
Journal:  J Lipid Res       Date:  2015-08-03       Impact factor: 5.922

Review 7.  Targeting Wolman Disease and Cholesteryl Ester Storage Disease: Disease Pathogenesis and Therapeutic Development.

Authors:  Francis Aguisanda; Natasha Thorne; Wei Zheng
Journal:  Curr Chem Genom Transl Med       Date:  2017-01-30
  7 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.