The human OPA1delTTAG mutation induces premature age-related systemic neurodegeneration in mouse.
about
Genetic manipulation for inherited neurodegenerative diseases: myth or reality?Mitochondrial Quality Control and Muscle Mass MaintenanceRespiratory supercomplexes: plasticity and implicationsA splicing mutation in the novel mitochondrial protein DNAJC11 causes motor neuron pathology associated with cristae disorganization, and lymphoid abnormalities in miceA neurodegenerative perspective on mitochondrial optic neuropathiesSyndromic parkinsonism and dementia associated with OPA1 missense mutationsTreatment strategies for inherited optic neuropathies: past, present and futureMitochondrial dysfunction-associated OPA1 cleavage contributes to muscle degeneration: preventative effect of hydroxytyrosol acetate.Mitochondrial Diseases Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function.Implications of mitochondrial dynamics on neurodegeneration and on hypothalamic dysfunction.Increase in Cardiac Ischemia-Reperfusion Injuries in Opa1+/- Mouse Model.Fatal infantile mitochondrial encephalomyopathy, hypertrophic cardiomyopathy and optic atrophy associated with a homozygous OPA1 mutation.Dysregulated mitophagy and mitochondrial organization in optic atrophy due to OPA1 mutations.Exercise pretreatment promotes mitochondrial dynamic protein OPA1 expression after cerebral ischemia in rats.Dominant optic atrophy, OPA1, and mitochondrial quality control: understanding mitochondrial network dynamics.Disturbed mitochondrial dynamics and neurodegenerative disorders.A Review of Mitochondrial Optic Neuropathies: From Inherited to Acquired FormsIncreased steroidogenesis promotes early-onset and severe vision loss in females with OPA1 dominant optic atrophy.Autophagy controls the pathogenicity of OPA1 mutations in dominant optic atrophy.Mitochondrial dysfunction in an Opa1(Q285STOP) mouse model of dominant optic atrophy results from Opa1 haploinsufficiency.Mitochondrial energetic defects in muscle and brain of a Hmbs-/- mouse model of acute intermittent porphyria.Reply: Early-onset Behr syndrome due to compound heterozygous mutations in OPA1.Reply: Sensorineural hearing loss in OPA1-linked disorders.Mutations in DNM1L, as in OPA1, result indominant optic atrophy despite opposite effectson mitochondrial fusion and fission.Optic neuropathies: the tip of the neurodegeneration iceberg.OPA1 gene therapy prevents retinal ganglion cell loss in a Dominant Optic Atrophy mouse model.OPA1 haploinsufficiency induces a BNIP3-dependent decrease in mitophagy in neurons: relevance to Dominant Optic Atrophy.OPA1-anchored PKA phosphorylates perilipin 1 on S522 and S497 in adipocytes differentiated from human adipose stem cells.RNA m6A methylation participates in regulation of postnatal development of the mouse cerebellum.Validating the RedMIT/GFP-LC3 Mouse Model by Studying Mitophagy in Autosomal Dominant Optic Atrophy Due to the OPA1Q285STOP Mutation
P2860
Q26752831-91459C52-7F0F-472D-8C2D-6856A406053EQ26770420-CFC926B5-0D7E-4C0D-8A3D-06FBD93938D6Q27010216-6418F67A-4391-4B8F-91F5-29E96C60EF5DQ27316380-0E171256-CF87-45F0-9E49-B64B10A98EC8Q28072026-996ADC60-683B-4FF2-A094-4170974A4593Q28259640-7F4EADBE-2AB3-420A-B3F3-AAC6F06AF3B0Q33595163-2D0FCC97-1F6A-4695-82E4-D23DA4B802E4Q34662216-AA932FCB-B226-419A-BCDC-158FCD999542Q35660977-A9C45C11-AD79-4AF8-B457-B4186D9C1754Q35708874-A3B1EB28-474E-4817-9868-8B8A162628C7Q36159289-1EB4A7DA-49C6-42A4-AAB3-9A52F5FAA525Q36577372-7A270D74-6F5C-40D2-A6F9-8DA163F2A6B0Q37577747-360260CC-CF15-4C77-9F6A-E106C561C77CQ37684078-F08D675C-D0AB-4ED9-88E5-BCF0F968CF5FQ38143285-B0B72C29-879F-4205-8064-F3D8F6ADF5A3Q38285228-E7897B5D-8A73-4B74-9851-163A6658FC5FQ39063765-A376F0B4-B22B-4084-A1F9-196EB39F0936Q39710935-DE5BA2F7-8625-42A3-B0B4-A8F4B8D9D35CQ41825073-423293E5-A30E-4C1C-B878-23EEFA1F1500Q42381310-D225D094-4642-4B04-A1B3-128DA406C714Q42478276-9184C785-A88A-41F0-B29D-F7AA4E500F98Q42875943-928404B7-8E7B-4A13-A43A-E16FDCD0297AQ43116350-95197D72-7383-4168-BB61-3D4F58F4838EQ47713821-0B7FA3B2-15DF-47AE-A82E-74D6E7D3C94AQ49608453-FE666818-5E24-4B3F-AD5D-A1A192F0EAACQ49758178-5F4D15A7-7288-4ADF-BB95-57E88DF7E1F9Q50476012-2EC2423A-855D-4D93-9A09-45516DD97714Q52567077-51C33673-6ADA-4918-AFE2-117D0B62C58AQ55034887-4E862387-2924-43AA-A20B-666D3A0F8C76Q57072518-00861EDD-DC34-4C0C-AED5-125BF8F83FF9
P2860
The human OPA1delTTAG mutation induces premature age-related systemic neurodegeneration in mouse.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年学术文章
@wuu
2012年学术文章
@zh
2012年学术文章
@zh-cn
2012年学术文章
@zh-hans
2012年学术文章
@zh-my
2012年学术文章
@zh-sg
2012年學術文章
@yue
2012年學術文章
@zh-hant
name
The human OPA1delTTAG mutation ...... ic neurodegeneration in mouse.
@en
The human OPA1delTTAG mutation ...... ic neurodegeneration in mouse.
@nl
type
label
The human OPA1delTTAG mutation ...... ic neurodegeneration in mouse.
@en
The human OPA1delTTAG mutation ...... ic neurodegeneration in mouse.
@nl
prefLabel
The human OPA1delTTAG mutation ...... ic neurodegeneration in mouse.
@en
The human OPA1delTTAG mutation ...... ic neurodegeneration in mouse.
@nl
P2093
P2860
P50
P356
P1433
P1476
The human OPA1delTTAG mutation ...... mic neurodegeneration in mouse
@en
P2093
Anne-Laure Mausset-Bonnefont
Benjamin Chaix
Chantal Cazevieille
Christian P Hamel
Claire Angebault
Guy Bielicki
Jean-Pierre Renou
Marie Seveno
Naïg Gueguen
P2860
P304
P356
10.1093/BRAIN/AWS303
P407
P577
2012-12-01T00:00:00Z