A gatekeeper helix determines the substrate specificity of Sjögren–Larsson Syndrome enzyme fatty aldehyde dehydrogenase

Mutations in the gene coding for membrane-bound fatty aldehyde dehydrogenase (FALDH) lead to toxic accumulation of lipid species and development of the Sjögren–Larsson Syndrome (SLS), a rare disorder characterized by skin defects and mental retardation. Here, we present the crystallographic structur...

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Autori principali: Keller, Markus A., Zander, Ulrich, Fuchs, Julian E., Kreutz, Christoph, Watschinger, Katrin, Mueller, Thomas, Golderer, Georg, Liedl, Klaus R., Ralser, Markus, Kräutler, Bernhard, Werner, Ernst R., Marquez, Jose A.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Nature Pub. Group 2014
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Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4109017/
https://ncbi.nlm.nih.gov/pubmed/25047030
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/ncomms5439
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