Results 211 to 220 of about 22,170 (231)

FGF21 and GDF15 Act Synergistically to Regulate Systemic Metabolic Homeostasis in Mice Lacking OPA1 in Thermogenic Adipocytes. [PDF]

Obesity (Silver Spring)
Peterson J, Jena J, Sood A, Roitershtein S, Smith D, Pereira RO.   +5 more
europepmc   +1 more source

Mitochondrial Dysfunction Rewires Macrophage Metabolism, Driving Pro-inflammatory Priming and Immune System Remodeling

Markov N, Hosseini A, Fettrelet T, Oberson K, Brichkina A, Yousefi S, Bourquin C, Simon H.   +7 more
europepmc   +1 more source

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OPA1 trans‐splicing: A gene therapy approach to treat OPA1‐related optic neuropathies

Acta Ophthalmologica, 2022
AbstractDominant Optic Atrophy is a blinding disease related in 70% of cases to OPA1 variants. OPA1 variants are leading to haplo‐insufficiency or dominant negative effects, leading in 20% of cases to a syndromic presentation, with neurosensorial hearing loss as the most prevalent secondary symptom. Bi‐allelic OPA1 variants are also leading to the Behr
Yannick le Dantec, Olivier Baris, Guy Lenaers   +2 more
openaire   +1 more source

Metabolic reprogramming in the OPA1-deficient cells

Cellular and Molecular Life Sciences, 2022
OPA1, a dynamin-related GTPase mutated in autosomal dominant optic atrophy, is essential for the fusion of the inner mitochondrial membrane. Although OPA1 deficiency leads to impaired mitochondrial morphology, the role of OPA1 in central carbon metabolism remains unclear.
Wenting Dai, Zhichao Wang, Qiong A. Wang, David Chan, Lei Jiang   +4 more
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OPA1 (dys)functions

Seminars in Cell & Developmental Biology, 2010
Mitochondrial morphology varies according to cell type and cellular context from an interconnected filamentous network to isolated dots. This morphological plasticity depends on mitochondrial dynamics, a balance between antagonistic forces of fission and fusion. DRP1 and FIS1 control mitochondrial outer membrane fission and Mitofusins its fusion.
Thomas, Landes, Ingrid, Leroy, Ambre, Bertholet, Alan, Diot, Farnoosh, Khosrobakhsh, Marlène, Daloyau, Noélie, Davezac, Marie-Christine, Miquel, Delphine, Courilleau, Emmanuelle, Guillou, Aurélien, Olichon, Guy, Lenaers, Laetitia, Arnauné-Pelloquin, Laurent J, Emorine, Pascale, Belenguer   +14 more
openaire   +2 more sources

OPA1-associated disorders: Phenotypes and pathophysiology

The International Journal of Biochemistry & Cell Biology, 2009
The OPA1 gene, encoding a dynamin-like mitochondrial GTPase, is involved in autosomal dominant optic atrophy (ADOA, OMIM #165500). ADOA, also known as Kjer's optic atrophy, affects retinal ganglion cells and the axons forming the optic nerve, leading to progressive visual loss.
Amati-Bonneau, Patrizia, Milea, Dan, Bonneau, Dominique, Chevrollier, Arnaud, Ferré, Marc, Guillet, Virginie, Gueguen, Naïg, Loiseau, Dominique, de Crescenzo, Marie-Anne Pou, Verny, Christophe, Procaccio, Vincent, Lenaers, Guy, Reynier, Pascal   +12 more
openaire   +3 more sources