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Age-Dependent Increase in Schmidt-Lanterman Incisures and a Cadm4-Associated Membrane Skeletal Complex in Fatty Acid 2-hydroxylase Deficient Mice: a Mouse Model of Spastic Paraplegia SPG35

PNS and CNS myelin contain large amounts of galactocerebroside and sulfatide with 2-hydroxylated fatty acids. The underlying hydroxylation reaction is catalyzed by fatty acid 2-hydroxylase (FA2H). Deficiency in this enzyme causes a complicated hereditary spastic paraplegia, SPG35, which is associate...

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Detalles Bibliográficos
Autores principales: Jordans, Silvia, Hardt, Robert, Becker, Ivonne, Winter, Dominic, Wang-Eckhardt, Lihua, Eckhardt, Matthias
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9167166/
https://www.ncbi.nlm.nih.gov/pubmed/35445918
http://dx.doi.org/10.1007/s12035-022-02832-4