Cargando…

Characterization of small fiber pathology in a mouse model of Fabry disease

Fabry disease (FD) is a life-threatening X-linked lysosomal storage disorder caused by α-galactosidase A (α-GAL) deficiency. Small fiber pathology and pain are major FD symptoms of unknown pathophysiology. α-GAL deficient mice (GLA KO) age-dependently accumulate globotriaosylceramide (Gb3) in dorsal...

Descripción completa

Detalles Bibliográficos
Autores principales: Hofmann, Lukas, Hose, Dorothea, Grießhammer, Anne, Blum, Robert, Döring, Frank, Dib-Hajj, Sulayman, Waxman, Stephen, Sommer, Claudia, Wischmeyer, Erhard, Üçeyler, Nurcan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6255391/
https://www.ncbi.nlm.nih.gov/pubmed/30328411
http://dx.doi.org/10.7554/eLife.39300