| scholarly article | Q13442814 |
| P356 | DOI | 10.1093/BRAIN/AWS303 |
| P698 | PubMed publication ID | 23250881 |
| P50 | author | Nathalie Boddaert | Q28354276 |
| Guy Lenaers | Q56488643 | ||
| Pascal Reynier | Q56541742 | ||
| Jean-Luc Puel | Q63442217 | ||
| Cécile Delettre | Q85171096 | ||
| Emmanuelle Sarzi | Q85727405 | ||
| Valerie Rigau | Q90695581 | ||
| P2093 | author name string | Jing Wang | |
| Christian P Hamel | |||
| Chantal Cazevieille | |||
| Philippe Brabet | |||
| Jean-Pierre Renou | |||
| Anne-Laure Mausset-Bonnefont | |||
| Claire Angebault | |||
| Marie Seveno | |||
| Guy Bielicki | |||
| Naïg Gueguen | |||
| Benjamin Chaix | |||
| P2860 | cites work | Early alterations of brain cellular energy homeostasis in Huntington disease models | Q23912810 |
| OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28 | Q24290356 | ||
| OPA1 mutations induce mitochondrial DNA instability and optic atrophy 'plus' phenotypes | Q24304868 | ||
| Loss of OPA1 perturbates the mitochondrial inner membrane structure and integrity, leading to cytochrome c release and apoptosis | Q24338169 | ||
| Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome | Q24598420 | ||
| Multi-system neurological disease is common in patients with OPA1 mutations | Q24619312 | ||
| Cardiolipin and electron transport chain abnormalities in mouse brain tumor mitochondria: lipidomic evidence supporting the Warburg theory of cancer | Q24658168 | ||
| Mitochondria and the autophagy-inflammation-cell death axis in organismal aging | Q26995343 | ||
| Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy | Q28140286 | ||
| The creatine kinase/creatine connection to Alzheimer's disease: CK-inactivation, APP-CK complexes and focal creatine deposits | Q28200789 | ||
| Mitochondrial dynamics and disease, OPA1 | Q28243097 | ||
| OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion | Q28251846 | ||
| Cisd2 deficiency drives premature aging and causes mitochondria-mediated defects in mice | Q28594937 | ||
| Cardiolipin stabilizes respiratory chain supercomplexes | Q29615470 | ||
| Mitochondrial dynamics--fusion, fission, movement, and mitophagy--in neurodegenerative diseases | Q29615646 | ||
| Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A | Q29616547 | ||
| OPA1 disease alleles causing dominant optic atrophy have defects in cardiolipin-stimulated GTP hydrolysis and membrane tubulation | Q30494366 | ||
| Gait changes precede overt arthritis and strongly correlate with symptoms and histopathological events in pristane-induced arthritis | Q33923285 | ||
| Mitochondrial and Cell Death Mechanisms in Neurodegenerative Diseases | Q34502221 | ||
| Neuroimaging of mitochondrial disease. | Q34593678 | ||
| Subclinical multisystem neurologic disease in "pure" OPA1 autosomal dominant optic atrophy | Q35230955 | ||
| The complexity of age-related hearing impairment: contributing environmental and genetic factors. | Q36896926 | ||
| Ataxia with loss of Purkinje cells in a mouse model for Refsum disease | Q36976826 | ||
| Supercomplexes of the mitochondrial electron transport chain decline in the aging rat heart. | Q37388302 | ||
| Down-regulation of OPA1 alters mouse mitochondrial morphology, PTP function, and cardiac adaptation to pressure overload. | Q37393582 | ||
| Mitochondria: commanders of innate immunity and disease? | Q37964382 | ||
| Respiratory active mitochondrial supercomplexes. | Q39913363 | ||
| A splice site mutation in the murine Opa1 gene features pathology of autosomal dominant optic atrophy. | Q40240586 | ||
| OPA1 links human mitochondrial genome maintenance to mtDNA replication and distribution | Q41554894 | ||
| In vivo and in vitro assessment of brain bioenergetics in aging rats | Q41807410 | ||
| MARF and Opa1 Control Mitochondrial and Cardiac Function in Drosophila | Q42136255 | ||
| OPA1 mutations impair mitochondrial function in both pure and complicated dominant optic atrophy | Q42709140 | ||
| Subtle neurological and metabolic abnormalities in an Opa1 mouse model of autosomal dominant optic atrophy | Q43265018 | ||
| Characterization of OPA1 isoforms isolated from mouse tissues | Q44071219 | ||
| Mutation of OPA1 causes dominant optic atrophy with external ophthalmoplegia, ataxia, deafness and multiple mitochondrial DNA deletions: a novel disorder of mtDNA maintenance. | Q44306523 | ||
| Absence of cardiolipin results in temperature sensitivity, respiratory defects, and mitochondrial DNA instability independent of pet56. | Q44915274 | ||
| Cardiolipin biosynthesis and mitochondrial respiratory chain function are interdependent | Q45005873 | ||
| Deficit of in vivo mitochondrial ATP production in OPA1-related dominant optic atrophy | Q45125308 | ||
| Development and implementation of standardized respiratory chain spectrophotometric assays for clinical diagnosis | Q45859940 | ||
| Mitochondrial fusion protects against neurodegeneration in the cerebellum. | Q45931731 | ||
| OPA1 deficiency associated with increased autophagy in retinal ganglion cells in a murine model of dominant optic atrophy. | Q46113788 | ||
| Molecular symmetry in mitochondrial cardiolipins | Q46754096 | ||
| OPA1 mutations associated with dominant optic atrophy impair oxidative phosphorylation and mitochondrial fusion | Q46789094 | ||
| 1H MRS spectroscopy evidence of cerebellar high lactate in mitochondrial respiratory chain deficiency. | Q46939834 | ||
| Defective brain energy metabolism shown by in vivo 31P MR spectroscopy in 28 patients with mitochondrial cytopathies | Q48291356 | ||
| Ageing alters the supramolecular architecture of OxPhos complexes in rat brain cortex | Q48305782 | ||
| Effects of Alzheimer's disease transgenes on neurochemical expression in the mouse brain determined by 1H MRS in vitro | Q48715143 | ||
| 'CatWalk' automated quantitative gait analysis as a novel method to assess mechanical allodynia in the rat; a comparison with von Frey testing | Q52021251 | ||
| HDAC6 inhibitors reverse axonal loss in a mouse model of mutant HSPB1–induced Charcot-Marie-Tooth disease | Q57244922 | ||
| P433 | issue | Pt 12 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | neurodegeneration | Q1755122 |
| P304 | page(s) | 3599-3613 | |
| P577 | publication date | 2012-12-01 | |
| P1433 | published in | Brain | Q897386 |
| P1476 | title | The human OPA1delTTAG mutation induces premature age-related systemic neurodegeneration in mouse | |
| P478 | volume | 135 |
| Q39063765 | A Review of Mitochondrial Optic Neuropathies: From Inherited to Acquired Forms |
| Q28072026 | A neurodegenerative perspective on mitochondrial optic neuropathies |
| Q27316380 | A splicing mutation in the novel mitochondrial protein DNAJC11 causes motor neuron pathology associated with cristae disorganization, and lymphoid abnormalities in mice |
| Q41825073 | Autophagy controls the pathogenicity of OPA1 mutations in dominant optic atrophy |
| Q38285228 | Disturbed mitochondrial dynamics and neurodegenerative disorders |
| Q38143285 | Dominant optic atrophy, OPA1, and mitochondrial quality control: understanding mitochondrial network dynamics |
| Q37577747 | Dysregulated mitophagy and mitochondrial organization in optic atrophy due to OPA1 mutations. |
| Q37684078 | Exercise pretreatment promotes mitochondrial dynamic protein OPA1 expression after cerebral ischemia in rats |
| Q36577372 | Fatal infantile mitochondrial encephalomyopathy, hypertrophic cardiomyopathy and optic atrophy associated with a homozygous OPA1 mutation |
| Q26752831 | Genetic manipulation for inherited neurodegenerative diseases: myth or reality? |
| Q35708874 | Implications of mitochondrial dynamics on neurodegeneration and on hypothalamic dysfunction |
| Q36159289 | Increase in Cardiac Ischemia-Reperfusion Injuries in Opa1+/- Mouse Model. |
| Q39710935 | Increased steroidogenesis promotes early-onset and severe vision loss in females with OPA1 dominant optic atrophy |
| Q90583371 | Metabolomics hallmarks OPA1 variants correlating with their in-vitro phenotype and predicting clinical severity |
| Q35660977 | Mitochondrial Diseases Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function |
| Q26770420 | Mitochondrial Quality Control and Muscle Mass Maintenance |
| Q89295264 | Mitochondrial dynamics in adaptive and maladaptive cellular stress responses |
| Q42381310 | Mitochondrial dysfunction in an Opa1(Q285STOP) mouse model of dominant optic atrophy results from Opa1 haploinsufficiency. |
| Q34662216 | Mitochondrial dysfunction-associated OPA1 cleavage contributes to muscle degeneration: preventative effect of hydroxytyrosol acetate |
| Q42478276 | Mitochondrial energetic defects in muscle and brain of a Hmbs-/- mouse model of acute intermittent porphyria |
| Q47713821 | Mutations in DNM1L, as in OPA1, result indominant optic atrophy despite opposite effectson mitochondrial fusion and fission |
| Q49758178 | OPA1 gene therapy prevents retinal ganglion cell loss in a Dominant Optic Atrophy mouse model. |
| Q50476012 | OPA1 haploinsufficiency induces a BNIP3-dependent decrease in mitophagy in neurons: relevance to Dominant Optic Atrophy. |
| Q52567077 | OPA1-anchored PKA phosphorylates perilipin 1 on S522 and S497 in adipocytes differentiated from human adipose stem cells. |
| Q49608453 | Optic neuropathies: the tip of the neurodegeneration iceberg |
| Q89174941 | Optical Coherence Tomography: Imaging Mouse Retinal Ganglion Cells In Vivo |
| Q92701983 | Presbycusis: An Update on Cochlear Mechanisms and Therapies |
| Q55034887 | RNA m6A methylation participates in regulation of postnatal development of the mouse cerebellum. |
| Q42875943 | Reply: Early-onset Behr syndrome due to compound heterozygous mutations in OPA1. |
| Q43116350 | Reply: Sensorineural hearing loss in OPA1-linked disorders |
| Q27010216 | Respiratory supercomplexes: plasticity and implications |
| Q28259640 | Syndromic parkinsonism and dementia associated with OPA1 missense mutations |
| Q90699281 | The Metabolomic Bioenergetic Signature of Opa1-Disrupted Mouse Embryonic Fibroblasts Highlights Aspartate Deficiency |
| Q64115753 | The Metabolomic Signature of Opa1 Deficiency in Rat Primary Cortical Neurons Shows Aspartate/Glutamate Depletion and Phospholipids Remodeling |
| Q33595163 | Treatment strategies for inherited optic neuropathies: past, present and future |
| Q57072518 | Validating the RedMIT/GFP-LC3 Mouse Model by Studying Mitophagy in Autosomal Dominant Optic Atrophy Due to the OPA1Q285STOP Mutation |
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