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Ultrastructure of skin from Refsum disease with emphasis on epidermal lamellar bodies and stratum corneum barrier lipid organization

Classic Refsum disease (RD) is a rare, autosomal recessively-inherited disorder of peroxisome metabolism due to a defect in the initial step in the alpha oxidation of phytanic acid (PA), a C 16 saturated fatty acid with four methyl side groups, which accumulates in plasma and lipid enriched tissues...

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Detalhes bibliográficos
Main Authors: Menon, G.K., Orsó, E., Aslanidis, Charalampos, Crumrine, D., Schmitz, G., Elias, P.M.
Formato: Artigo
Idioma:Inglês
Publicado em: 2014
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4169723/
https://ncbi.nlm.nih.gov/pubmed/24920240
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00403-014-1478-2
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