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Targeting Wolman Disease and Cholesteryl Ester Storage Disease: Disease Pathogenesis and Therapeutic Development

Wolman disease (WD) and cholesteryl ester storage disease (CESD) are lysosomal storage diseases (LSDs) caused by a deficiency in lysosomal acid lipase (LAL) due to mutations in the LIPA gene. This enzyme is critical to the proper degradation of cholesterol in the lysosome. LAL function is completely...

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Détails bibliographiques
Publié dans:Curr Chem Genom Transl Med
Auteurs principaux: Aguisanda, Francis, Thorne, Natasha, Zheng, Wei
Format: Artigo
Langue:Inglês
Publié: Bentham Open 2017
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC5362971/
https://ncbi.nlm.nih.gov/pubmed/28401034
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.2174/2213988501711010001
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