Sphingosine-1-Phosphate Lyase (SGPL1) Deficiency Is Associated with Mitochondrial Dysfunction

The Journal of Steroid Biochemistry and Molecular Biology(2018)

引用 19|浏览12
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摘要
•Sphingosine-1-phosphate (S1P) lyase deficiency leads to accumulation of upstream sphingolipid species.•Acute steroidogenesis is impaired in S1P lyase deficient patient dermal fibroblasts.•S1P lyase deficiency affects mitochondrial morphology and dynamics.•Parameters of oxidative phosphorylation are altered in S1P lyase deficiency.
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关键词
Sphingolipid metabolism,Mitochondrial dynamics,Steroidogenic capacity,Oxidative phosphorylation,Sphingosine-1-phosphate lyase
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