Results 31 to 40 of about 22,170 (231)

OPA1 Dominant Optic Atrophy: Pathogenesis and Therapeutic Targets

Journal of Neuro-Ophthalmology, 2023
No description ...
David C. S. Wong, Joshua P. Harvey, Neringa Jurkute, Sara M. Thomasy, Mariya Moosajee, Patrick Yu-Wai-Man, Michael J. Gilhooley   +6 more
openaire   +3 more sources

Cryo-EM structures of S-OPA1 reveal its interactions with membrane and changes upon nucleotide binding

eLife, 2020
Mammalian mitochondrial inner membrane fusion is mediated by optic atrophy 1 (OPA1). Under physiological conditions, OPA1 undergoes proteolytic processing to form a membrane-anchored long isoform (L-OPA1) and a soluble short isoform (S-OPA1).
Danyang Zhang, Yan Zhang, Jun Ma, Chunmei Zhu, Tongxin Niu, Wenbo Chen, Xiaoyun Pang, Yujia Zhai, Fei Sun   +8 more
doaj   +1 more source

The Role of Swelling in the Regulation of OPA1-Mediated Mitochondrial Function in the Heart In Vitro

Cells, 2023
Optic atrophy-1 (OPA1) plays a crucial role in the regulation of mitochondria fusion and participates in maintaining the structural integrity of mitochondrial cristae.
Xavier R. Chapa-Dubocq, Keishla M. Rodríguez-Graciani, Jorge García-Báez, Alyssa Vadovsky, Jason N. Bazil, Sabzali Javadov   +5 more
doaj   +1 more source

OPA1 helical structures give perspective to mitochondrial dysfunction

Nature, 2023
Dominant optic atrophy is one of the leading causes of childhood blindness. Around 60-80% of cases1 are caused by mutations of the gene that encodes optic atrophy protein 1 (OPA1), a protein that has a key role in inner mitochondrial membrane fusion and remodelling of cristae and is crucial for the dynamic organization and regulation of mitochondria2 ...
Sarah B. Nyenhuis, Xufeng Wu, Marie-Paule Strub, Yang-In Yim, Abigail E. Stanton, Valentina Baena, Zulfeqhar A. Syed, Bertram Canagarajah, John A. Hammer, Jenny E. Hinshaw   +9 more
openaire   +2 more sources

Mitochondrial and Lipid Droplet Dynamics Regulate Intra- and Intercellular Fatty Acid Trafficking

Molecular & Cellular Oncology, 2018
Imaging of fatty acid (FA) trafficking revealed that FAs stored in lipid droplets were delivered to mitochondria when the cells were starved. This delivery required cytoplasmic lipases and mitochondrial fusion activity, whereas lipid droplets were ...
Sarah Cohen, Angelika S. Rambold, Jennifer Lippincott-Schwartz   +2 more
doaj   +1 more source

OPA1 deletion in brown adipose tissue improves thermoregulation and systemic metabolism via FGF21

eLife, 2021
Adrenergic stimulation of brown adipocytes alters mitochondrial dynamics, including the mitochondrial fusion protein optic atrophy 1 (OPA1). However, direct mechanisms linking OPA1 to brown adipose tissue (BAT) physiology are incompletely understood.
Renata O Pereira, Alex Marti, Angela Crystal Olvera, Satya Murthy Tadinada, Sarah Hartwick Bjorkman, Eric Thomas Weatherford, Donald A Morgan, Michael Westphal, Pooja H Patel, Ana Karina Kirby, Rana Hewezi, William Bùi Trân, Luis Miguel García-Peña, Rhonda A Souvenir, Monika Mittal, Christopher M Adams, Kamal Rahmouni, Matthew J Potthoff, E Dale Abel   +18 more
doaj   +1 more source

Medical management of hereditary optic neuropathies. [PDF]

, 2014
Hereditary optic neuropathies are diseases affecting the optic nerve. The most common are mitochondrial hereditary optic neuropathies, i.e., the maternally inherited Leber's hereditary optic neuropathy (LHON) and dominant optic atrophy (DOA).
Barboni, Piero, Carbonelli, Michele, Carelli, Valerio, La Morgia, Chiara, Sadun, Alfredo Arrigo   +4 more
core   +1 more source

Restoration of L-OPA1 alleviates acute ischemic stroke injury in rats via inhibiting neuronal apoptosis and preserving mitochondrial function

Redox Biology, 2020
Background: Ischemic stroke can induce changes in mitochondrial morphology and function. As a regulatory gene in mitochondria, optic atrophy 1 (OPA1) plays a pivotal role in the regulation of mitochondrial dynamics and other related functions.
Yongxing Lai, Peiqiang Lin, Manli Chen, Yixian Zhang, Jianhao Chen, Mouwei Zheng, Ji Liu, Houwei Du, Ronghua Chen, Xiaodong Pan, Nan Liu, Hongbin Chen   +11 more
doaj   +1 more source

Role of inner mitochondrial protein OPA1 in mitochondrial dysfunction by tobacco smoking and in the pathogenesis of COPD

Redox Biology, 2021
Background: Chronic lung diseases, such as chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) are linked to several mitochondrial alterations. Cigarette smoke (CS) alters the structure and function of mitochondria.
Krishna Prahlad Maremanda, Isaac Kirubakaran Sundar, Irfan Rahman   +2 more
doaj   +1 more source

OPA1 supports mitochondrial dynamics and immune evasion to CD8+ T cell in lung adenocarcinoma [PDF]

PeerJ, 2022
Background Mitochondrial fusion and fission were identified to play key roles during multiple biology process. Thus, we aim to investigate the roles of OPA1 in mitochondria fusion and immune evasion of non-small cell lung cancer cells.
Ying Wang, Yadong Li, Xuanwei Jiang, Yayun Gu, Hui Zheng, Xiaoxuan Wang, Haotian Zhang, Jixiang Wu, Yang Cheng   +8 more
doaj   +2 more sources