The Role of Sulfide Oxidation Impairment in the Pathogenesis of Primary CoQ Deficiency

Por Quinzii, Catarina M., Luna-Sanchez, Marta, Ziosi, Marcello, Hidalgo-Gutierrez, Agustin, Kleiner, Giulio, Lopez, Luis C.
Publicado no Front Physiol (2017)

Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9(R239X) Mice

Por Barriocanal-Casado, Eliana, Cueto-Ureña, Cristina, Benabdellah, Karim, Gutiérrez-Guerrero, Alejandra, Cobo, Marién, Hidalgo-Gutiérrez, Agustín, Rodríguez-Sevilla, Juan José, Martín, Francisco, López, Luis C.
Publicado no PLoS One (2016)

CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome

Por Luna‐Sánchez, Marta, Hidalgo‐Gutiérrez, Agustín, Hildebrandt, Tatjana M, Chaves‐Serrano, Julio, Barriocanal‐Casado, Eliana, Santos‐Fandila, Ángela, Romero, Miguel, Sayed, Ramy KA, Duarte, Juan, Prokisch, Holger, Schuelke, Markus, Distelmaier, Felix, Escames, Germaine, Acuña‐Castroviejo, Darío, López, Luis C
Publicado no EMBO Mol Med (2016)