TY - JOUR AU - Suárez-Rivero, Juan M AU - de la Mata, Mario AU - Pavón, Ana Delgado AU - Villanueva-Paz, Marina AU - Povea-Cabello, Suleva AU - Cotán, David AU - Álvarez-Córdoba, Mónica AU - Villalón-García, Irene AU - Ybot-González, Patricia AU - Salas, Joaquín J AU - Muñiz, Ovidio AU - Cordero, Mario D AU - Sánchez-Alcázar, José A PY - 2018 DO - 10.1016/j.bbadis.2018.10.009 UR - http://hdl.handle.net/10668/13036 T2 - Biochimica et biophysica acta. Molecular basis of disease AB - Familial Hypercholesterolemia (FH) is an autosomal co-dominant genetic disorder characterized by elevated low-density lipoprotein (LDL) cholesterol levels and increased risk for premature cardiovascular disease. Here, we examined FH pathophysiology in... LA - en KW - Cholesterol KW - Coenzyme Q(10) deficiency KW - Familial Hypercholesterolemia KW - Inflammasome KW - LDL-R KW - Mitochondria dysfunction KW - Mitophagy KW - Ataxia KW - Cells, Cultured KW - Cholesterol KW - Fibroblasts KW - Humans KW - Hyperlipoproteinemia Type II KW - Lipoproteins, LDL KW - Membrane Potential, Mitochondrial KW - Mitochondria KW - Mitochondrial Diseases KW - Mitophagy KW - Muscle Weakness KW - Reactive Oxygen Species KW - Receptors, LDL KW - Ubiquinone TI - Intracellular cholesterol accumulation and coenzyme Q10 deficiency in Familial Hypercholesterolemia. TY - research article VL - 1864 ER -