Abnormal fatty alcohol metabolism in cultured keratinocytes from patients with Sjögren-Larsson syndrome

Abnormal fatty alcohol metabolism in cultured keratinocytes from patients with Sjögren-Larsson syndrome

Sjögren-Larsson syndrome (SLS) is an inherited neurocutaneous disorder characterized by ichthyosis, mental retardation, spasticity, and deficient activity of fatty aldehyde dehydrogenase (FALDH). FALDH is an enzyme component of fatty alcohol:NAD oxidoreductase (FAO), which is necessary for fatty al...

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Bibliographic Details
Main Authors: William B. Rizzo, Debra A. Craft, Tara Somer, Gael Carney, Juliana Trafrova, Marcia Simon
Format: Article
Language:English
Published: Elsevier 2008-02-01
Series:Journal of Lipid Research
Subjects:
aldehyde dehydrogenase
alkylglycerol
epidermis
ether glycerolipid
fatty aldehyde
ichthyosis
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520428627

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