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  - http://hdl.handle.net/10668/4589
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
PB  - MDPI
KW  - Coenzyme Q10
KW  - Coenzyme Q deficiency
KW  - Mitochondrial diseases
KW  - Cell models
KW  - Yeast
KW  - iPSC
KW  - Human fibroblasts
KW  - Ubiquinona
KW  - Enfermedades mitocondriales
KW  - Modelos anatómicos
KW  - Levaduras
KW  - Células madre pluripotentes inducidas
KW  - Fibroblastos
KW  - Medical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Signs and Symptoms::Neurologic Manifestations::Dyskinesias::Ataxia
KW  - Medical Subject Headings::Phenomena and Processes::Genetic Phenomena::Genetic Structures::Genome::Exome
KW  - Medical Subject Headings::Phenomena and Processes::Genetic Phenomena::Genetic Structures::Genome
KW  - Medical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Genetic Techniques::Sequence Analysis::High-Throughput Nucleotide Sequencing
KW  - Medical Subject Headings::Anatomy::Cells::Cellular Structures::Intracellular Space::Cytoplasm::Cytoplasmic Structures::Organelles::Mitochondria
KW  - Medical Subject Headings::Diseases::Nutritional and Metabolic Diseases::Metabolic Diseases::Mitochondrial Diseases
KW  - Medical Subject Headings::Diseases::Musculoskeletal Diseases::Muscular Diseases::Muscle Weakness
KW  - Medical Subject Headings::Organisms::Eukaryota::Fungi::Ascomycota::Saccharomycetales::Saccharomyces::Saccharomyces cerevisiae
KW  - Medical Subject Headings::Chemicals and Drugs::Organic Chemicals::Quinones::Benzoquinones::Ubiquinone
KW  - Medical Subject Headings::Anatomy::Cells::Stem Cells::Pluripotent Stem Cells::Induced Pluripotent Stem Cells
KW  - Medical Subject Headings::Organisms::Eukaryota::Fungi::Yeasts
TI  - Cellular Models for Primary CoQ Deficiency Pathogenesis Study
TY  - review article
ER  -