TY  - JOUR
AU  - Romero-Moya, Damià
AU  - Santos-Ocaña, Carlos
AU  - Castaño, Julio
AU  - Garrabou, Gloria
AU  - Rodríguez-Gómez, José A
AU  - Ruiz-Bonilla, Vanesa
AU  - Bueno, Clara
AU  - González-Rodríguez, Patricia
AU  - Giorgetti, Alessandra
AU  - Perdiguero, Eusebio
AU  - Prieto, Cristina
AU  - Moren-Nuñez, Constanza
AU  - Fernández-Ayala, Daniel J
AU  - Victoria Cascajo, Maria
AU  - Velasco, Iván
AU  - Canals, Josep Maria
AU  - Montero, Raquel
AU  - Yubero, Delia
AU  - Jou, Cristina
AU  - López-Barneo, José
AU  - Cardellach, Francesc
AU  - Muñoz-Cánoves, Pura
AU  - Artuch, Rafael
AU  - Navas, Plácido
AU  - Menendez, Pablo
PY  - 2017
DO  - 10.1002/stem.2634
UR  - http://hdl.handle.net/10668/11172
T2  - Stem cells (Dayton, Ohio)
AB  - Coenzyme Q10 (CoQ10 ) plays a crucial role in mitochondria as an electron carrier within the mitochondrial respiratory chain (MRC) and is an essential antioxidant. Mutations in genes responsible for CoQ10 biosynthesis (COQ genes) cause primary CoQ10...
LA  - en
KW  - COQ4
KW  - CRISPR-Cas9
KW  - Coenzyme Q10
KW  - Dopaminergic and motor neurons
KW  - Induced pluripotent stem cell
KW  - Skeletal muscle
KW  - Ataxia
KW  - CRISPR-Cas Systems
KW  - Cell Differentiation
KW  - Child, Preschool
KW  - Dopaminergic Neurons
KW  - Electron Transport Chain Complex Proteins
KW  - Fatal Outcome
KW  - Female
KW  - Fibroblasts
KW  - Gene Editing
KW  - Gene Expression
KW  - Genes, Lethal
KW  - Humans
KW  - Induced Pluripotent Stem Cells
KW  - Intellectual Disability
KW  - Mitochondria
KW  - Mitochondrial Diseases
KW  - Mitochondrial Proteins
KW  - Motor Neurons
KW  - Muscle Weakness
KW  - Primary Cell Culture
KW  - Rhabdomyolysis
KW  - Ubiquinone
TI  - Genetic Rescue of Mitochondrial and Skeletal Muscle Impairment in an Induced Pluripotent Stem Cells Model of Coenzyme Q10 Deficiency.
TY  - research article
VL  - 35
ER  -