TY - JOUR AU - Santos-Ocaña, Carlos AU - Cascajo, María V AU - Alcázar-Fabra, María AU - Staiano, Carmine AU - López-Lluch, Guillermo AU - Brea-Calvo, Gloria AU - Navas, Plácido PY - 2021 DO - 10.3390/ijms221910211 UR - https://hdl.handle.net/10668/28406 T2 - International journal of molecular sciences AB - Primary coenzyme Q10 (CoQ) deficiency includes a heterogeneous group of mitochondrial diseases characterized by low mitochondrial levels of CoQ due to decreased endogenous biosynthesis rate. These diseases respond to CoQ treatment mainly at the early... LA - en KW - cell models KW - coenzyme Q KW - coenzyme Q deficiency KW - human fibroblasts KW - iPSC KW - mitochondrial diseases KW - yeast KW - Ataxia KW - Exome KW - Genome KW - High-Throughput Nucleotide Sequencing KW - Humans KW - Mitochondria KW - Mitochondrial Diseases KW - Muscle Weakness KW - Saccharomyces cerevisiae KW - Ubiquinone KW - Exome Sequencing KW - Whole Genome Sequencing TI - Cellular Models for Primary CoQ Deficiency Pathogenesis Study. TY - research article VL - 22 ER -