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Ichthyosis in Sjögren–Larsson syndrome reflects defective barrier function due to abnormal lamellar body structure and secretion

Sjögren–Larsson syndrome is a genetic disease characterized by ichthyosis, mental retardation, spasticity and mutations in the ALDH3A2 gene coding for fatty aldehyde dehydrogenase, an enzyme necessary for oxidation of fatty aldehydes and fatty alcohols. We investigated the cutaneous abnormalities in...

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Gorde:
Xehetasun bibliografikoak
Egile Nagusiak: Rizzo, William B., S’Aulis, Dana, Jennings, M. Anitia, Crumrine, Debra A., Williams, Mary L., Elias, Peter M.
Formatua: Artigo
Hizkuntza:Inglês
Argitaratua: Springer-Verlag 2010
Gaiak:
Sarrera elektronikoa:https://ncbi.nlm.nih.gov/pmc/articles/PMC2892059/
https://ncbi.nlm.nih.gov/pubmed/20049467
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00403-009-1022-y
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