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Autophagy, ISSN 1554-8627, 01/2009, Volume 5, Issue 1, pp. 19 - 32
Coenzyme Q 10 (CoQ) is a small lipophilic molecule critical for the transport of electrons from complexes I and II to complex III in the mitochondrial... 
Binding | Proteins | Landes | Calcium | Bioscience | Biology | Cell | Cycle | Cancer | Organogenesis | Mitochondria | Mitochondrial permeability transition | Free radicals | Coenzyme Q | Mitophagy | Autophagy | Mitochondrial disease | PERMEABILITY TRANSITION PORE | free radicals | OXIDATIVE STRESS | autophagy | mitochondria | mitochondrial permeability transition | CULTURED SKIN FIBROBLASTS | COQ DEFICIENCY | RESPIRATORY-CHAIN SUPERCOMPLEXES | Q DEFICIENCY | POLYPRENYL TRANSFERASE | mitochondrial disease | CELL-DEATH | CELL BIOLOGY | mitophagy | CEREBELLAR-ATAXIA | HEART-MITOCHONDRIA | coenzyme Q | Mitochondria - enzymology | Electron Transport - drug effects | Reactive Oxygen Species - metabolism | Apoptosis - drug effects | Microtubule-Associated Proteins - metabolism | Humans | Ubiquinone - deficiency | Membrane Potential, Mitochondrial - drug effects | RNA, Messenger - metabolism | Fibroblasts - ultrastructure | Mitochondria - ultrastructure | Autophagy - drug effects | Ubiquinone - pharmacology | Mitochondrial Proteins - metabolism | Biomarkers - metabolism | Cell Survival - drug effects | Mitochondrial Membrane Transport Proteins - metabolism | RNA, Messenger - genetics | Mitochondria - metabolism | Mitochondria - drug effects | Fibroblasts - pathology | Blotting, Western | Syndrome | Gene Expression Regulation - drug effects | Animals | Autophagy-Related Protein 5 | Fibroblasts - drug effects | Cell Proliferation - drug effects | Mice
Journal Article
Journal Article
Journal of Psychiatric Research, ISSN 0022-3956, 2011, Volume 46, Issue 3, pp. 341 - 345
Journal Article
Molecular Genetics and Metabolism Reports, ISSN 2214-4269, 06/2018, Volume 15, pp. 96 - 97
Journal Article
JOURNAL OF NEUROLOGY, ISSN 0340-5354, 11/2014, Volume 261, Issue 11, pp. 2192 - 2198
Inherited ataxias are heterogeneous disorders affecting both children and adults, with over 40 different causative genes, making molecular genetic diagnosis... 
Autosomal recessive ataxia | Mitochondrial | ANO10 | PATIENT | Coenzyme Q (CoQ) deficiency | CLINICAL NEUROLOGY | REVEALS
Journal Article
Molecular Genetics and Metabolism Reports, ISSN 2214-4269, 12/2018, Volume 17, pp. 56 - 56
Journal Article
EXPERT REVIEW OF MOLECULAR DIAGNOSTICS, ISSN 1473-7159, 08/2015, Volume 15, Issue 8, pp. 1049 - 1059
Coenzyme Q(10) (CoQ) deficiency syndromes comprise a growing number of neurological and extraneurological disorders. Primary-genetic but also secondary CoQ... 
next-generation sequencing | RESISTANT NEPHROTIC SYNDROME | MUSCLE | COQ DEFICIENCY | PATHOLOGY | muscle biopsy | SACCHAROMYCES-CEREVISIAE | coenzyme Q10 deficiency syndromes | mitochondrial diseases | MUTATIONS CAUSE | Q BIOSYNTHESIS | GENE | CEREBELLAR-ATAXIA | functional studies in yeasts | MITOCHONDRIAL DISORDERS | UBIQUINONE DEFICIENCY
Journal Article
EXPERT REVIEW OF MOLECULAR DIAGNOSTICS, ISSN 1473-7159, 2018, Volume 18, Issue 6, pp. 491 - 498
Introduction: Coenzyme Q(10) (CoQ) deficiency syndromes comprise a growing number of genetic disorders. While primary CoQ deficiency syndromes are rare... 
Coenzyme Q deficiency syndromes | OXIDATIVE STRESS | WHOLE-GENOME | SULFIDE OXIDATION | next generation sequencing | RENAL-DISEASE | PATHOLOGY | muscle biopsy | COQ BIOSYNTHESIS | oxidative phosphorylation | INBORN-ERRORS | mitochondrial diseases | CEREBELLAR-ATAXIA | MITOCHONDRIAL DYSFUNCTION | NEPHROTIC SYNDROME | UBIQUINONE DEFICIENCY
Journal Article
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, ISSN 0925-4439, 12/2018, Volume 1864, Issue 12, pp. 3697 - 3713
Journal Article
MITOCHONDRION, ISSN 1567-7249, 07/2013, Volume 13, Issue 4, pp. 337 - 341
We evaluated coenzyme Q(10) (CoQ) levels in patients studied under suspicion of mitochondrial DNA depletion syndromes (MDS) (n = 39). CoQ levels were... 
DIAGNOSIS | CELLS | FORM | HUMANS | CHILDREN | CELL BIOLOGY | Mitochondrial disorders | SKELETAL-MUSCLE | Mitochondrial DNA depletion syndrome | Coenzyme Q deficiency | GENES | GENETICS & HEREDITY | MUTATIONS | RESPIRATORY-CHAIN DISORDERS
Journal Article
EMBO Molecular Medicine, ISSN 1757-4676, 05/2015, Volume 7, Issue 5, pp. 670 - 687
Primary coenzyme Q10 (CoQ10) deficiency is due to mutations in genes involved in CoQ biosynthesis. The disease has been associated with five major phenotypes,... 
CoQ multiprotein complex | Mitochondrial myopathy | Mouse model | Coq9 | Nonsense-mediated mRNA decay | Hair | Enzymes | Phenotypes | Animal models | Peptides | Amino acids | Coenzyme Q | Biosynthesis | Grants | Kinases | Experiments | Skeletal muscle | Atrophy | Proteins | Genotype & phenotype | Mitochondria | Brain research | Coenzyme Q10 | Fibroblasts | Mutation | Genotypes | Myopathy
Journal Article
AMERICAN JOURNAL OF HUMAN GENETICS, ISSN 0002-9297, 12/2006, Volume 79, Issue 6, pp. 1125 - 1129
Coenzyme Q(10) (CoQ(10)) is a vital lipophilic molecule that transfers electrons from mitochondrial respiratory chain complexes I and II to complex III.... 
PROTEIN | MITOCHONDRIAL ENCEPHALOMYOPATHY | CEREBELLAR-ATAXIA | GENETICS & HEREDITY | MYOPATHY | COENZYME Q DEFICIENCY
Journal Article
JOURNAL OF CLINICAL MEDICINE, ISSN 2077-0383, 03/2017, Volume 6, Issue 3
Coenzyme Q(10) (CoQ(10)) deficiency syndrome includes clinically heterogeneous mitochondrial diseases that show a variety of severe and debilitating symptoms.... 
RESPIRATORY-CHAIN | CoQ deficiency syndrome | DIPHOSPHATE SYNTHASE SUBUNIT-2 | mitochondria diseases | PHENOTYPIC VARIABILITY | COQ DEFICIENCY | MEDICINE, GENERAL & INTERNAL | MUTATIONS CAUSE | Q BIOSYNTHESIS | MITOCHONDRIAL ENCEPHALOMYOPATHY | CEREBELLAR-ATAXIA | ADCK3 | CoQ biosynthesis | UBIQUINONE DEFICIENCY | coenzyme Q
Journal Article
MOLECULAR GENETICS AND METABOLISM, ISSN 1096-7192, 09/2018, Volume 125, Issue 1-2, pp. 38 - 43
Background: Coenzyme Q(10) (CoQ(10)) is an important component of the mitochondrial respiratory chain (RC) and is critical for energy production. Although the... 
Electron transport chain | MEDICINE, RESEARCH & EXPERIMENTAL | MITOCHONDRIAL | Complex II plus III | ENDOCRINOLOGY & METABOLISM | GENETICS & HEREDITY | OXPHOS | PREVALENCE | Coenzyme Q10 deficiency | Reference range | RESPIRATORY-CHAIN DISORDERS | CHILDREN
Journal Article
BBA - Bioenergetics, ISSN 0005-2728, 08/2016, Volume 1857, Issue 8, pp. 1079 - 1085
Journal Article