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Tissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice
- Publication Year :
- 2013
-
Abstract
- Primary human CoQ10 deficiencies are clinically heterogeneous diseases caused by mutations in PDSS2 and other genes required for CoQ10 biosynthesis. Our in vitro studies of PDSS2 mutant fibroblasts, with
- Subjects :
- Ubiquinone
Alkyl and Aryl Transferase
Mitochondrion
medicine.disease_cause
Kidney
Biochemistry
DNA, Mitochondrial
Research Communications
Electron Transport
Mice
Adenosine Triphosphate
PDSS2
Genetics
medicine
Citrate synthase
Animals
Humans
Tissue Distribution
Molecular Biology
chemistry.chemical_classification
Reactive oxygen species
Alkyl and Aryl Transferases
biology
ATP synthase
Animal
food and beverages
Kidney metabolism
ROS
Oxidative Stre
Fibroblasts
Molecular biology
Mice, Mutant Strains
Mitochondria
ATP
Adenosine Diphosphate
Oxidative Stress
Mitochondrial respiratory chain
chemistry
biology.protein
Mice, Inbred CBA
Fibroblast
Polyprenyl-diphosphate synthase
Oxidative stress
Mice, Mutant Strain
Biotechnology
Human
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....4e25cd88d277d7cbc6bfed94f9041d97