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ISSN 0969-7128, 2017
Leigh syndrome (LS) is the most common infantile mitochondrial encephalopathy. No treatment is currently available for this condition. Mice lacking... 
mouse models | experimental therapy | Leigh syndrome | AAV | gene therapy | Ndufs4 | Mitochondrial disease
Journal Article
ISSN 0300-5127, 2018
Preclinical work aimed at developing new therapies for mitochondrial diseases has recently given new hopes and opened unexpected perspectives for the patients... 
Journal Article
Cell Metabolism, ISSN 1550-4131, 10/2019, Volume 30, Issue 4, pp. 628 - 629
Dysfunctions of the mitochondrial electron transport chain cause severe, currently untreatable, diseases in humans. A new study by reports increased oxygen... 
HYPOXIA | ENDOCRINOLOGY & METABOLISM | CELL BIOLOGY | Anemia
Journal Article
Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 1/2011, Volume 108, Issue 4, pp. 1705 - 1710
Cardiac pacemaking generation and modulation rely on the coordinated activity of several processes. Although a wealth of evidence indicates a relevant role of... 
Heart | Heart rate | Conduction | Electric potential | Bradycardia | Transgenic animals | Heart block | Electrocardiography | Mice | Telemetry | Atrioventricular node | Funny current | Chronotropism | Sinoatrial node | MYOCARDIUM | funny current | heart rate | CONDUCTION SYSTEM | MULTIDISCIPLINARY SCIENCES | sinoatrial node | RABBIT SINOATRIAL NODE | MESSENGER-RNA | EMBRYONIC HEART | atrioventricular node | DIFFERENTIATION | TISSUES | chronotropism | EXPRESSION | INSIGHTS | Bradycardia - genetics | Heart Block - physiopathology | Male | Mice, 129 Strain | Sinoatrial Node - drug effects | Heart Rate - drug effects | Myocardium - metabolism | Female | Receptors, Adrenergic, beta - metabolism | Action Potentials - drug effects | Heart - physiopathology | Sinoatrial Node - physiology | Mice, Inbred C57BL | Cyclic Nucleotide-Gated Cation Channels - genetics | Bradycardia - physiopathology | Cyclic Nucleotide-Gated Cation Channels - physiology | Mice, Transgenic | Blotting, Western | Bone Density Conservation Agents - pharmacology | Heart Block - genetics | Mice, Knockout | Hyperpolarization-Activated Cyclic Nucleotide-Gated Channels | Animals | Myocytes, Cardiac - drug effects | Myocytes, Cardiac - physiology | Fluorescent Antibody Technique | Tamoxifen - pharmacology | Heart - drug effects | Myocytes, Cardiac - metabolism | Sinoatrial Node - metabolism | Physiological aspects | Complications and side effects | Development and progression | Cardiac pacing | Risk factors | Biological Sciences
Journal Article
Biochemical Society Transactions, ISSN 0300-5127, 10/2018, Volume 46, Issue 5, pp. 1247 - 1261
Preclinical work aimed at developing new therapies for mitochondrial diseases has recently given new hopes and opened unexpected perspectives for the patients... 
gene therapy | mitochondrial dysfunction | mitochondrial biogenesis | bypass therapy | rapamycin | LIVER-TRANSPLANTATION | SPINDLE TRANSFER | BIOCHEMISTRY & MOLECULAR BIOLOGY | MOUSE MODEL | REPLACEMENT THERAPY | MYOPATHY PROGRESSION | GENE-THERAPY | HEREDITARY OPTIC NEUROPATHY | KINASE 2 DEFICIENCY | DEPLETION SYNDROME | NUTRITIONAL INTERVENTIONS | Review
Journal Article
Journal Article
BBA - Bioenergetics, ISSN 0005-2728, 06/2015, Volume 1847, Issue 6-7, pp. 544 - 557
Journal Article
Antioxidants & Redox Signaling, ISSN 1523-0864, 11/2019
Journal Article
ISSN 0925-4439, 2018
Leigh syndrome (LS) associated with cytochrome c oxidase (COX) deficiency is an early onset, fatal mitochondrial encephalopathy, leading to multiple... 
Journal Article
Free Radical Biology and Medicine, ISSN 0891-5849, 11/2017, Volume 112, p. 197
To access, purchase, authenticate, or subscribe to the full-text of this article, please visit this link: http://dx.doi.org/10.1016/j.freeradbiomed.2017.10.312 
Muscles
Journal Article
Free Radical Biology and Medicine, ISSN 0891-5849, 11/2017, Volume 112, pp. 197 - 197
Journal Article
Journal Article
Nature Medicine, ISSN 1078-8956, 11/2018, Volume 24, Issue 11, pp. 1691 - 1695
Journal Article