Results 31 to 40 of about 15,870 (189)

CRISPR-Cas9 correction of OPA1 c.1334G>A: p.R445H restores mitochondrial homeostasis in dominant optic atrophy patient-derived iPSCs

Molecular Therapy: Nucleic Acids, 2021
Autosomal dominant optic atrophy (DOA) is the most common inherited optic neuropathy in the United Kingdom. DOA has an insidious onset in early childhood, typically presenting with bilateral, central visual loss caused by the preferential loss of retinal
Paul E. Sladen, Pedro R.L. Perdigão, Grace Salsbury, Tatiana Novoselova, Jacqueline van der Spuy, J. Paul Chapple, Patrick Yu-Wai-Man, Michael E. Cheetham   +7 more
doaj   +1 more source

RIPK3 dampens mitochondrial bioenergetics and lipid droplet dynamics in metabolic liver disease

Hepatology, EarlyView., 2022
RIPK3 dampens mitochondrial bioenergetics and lipid droplet dynamics in metabolic liver disease. Abstract Background and Aims Receptor‐interacting protein kinase 3 (RIPK3) mediates NAFLD progression, but its metabolic function is unclear. Here, we aimed to investigate the role of RIPK3 in modulating mitochondria function, coupled with lipid droplet (LD)
Marta B. Afonso, Tawhidul Islam, Julie Magusto, Ricardo Amorim, Véronique Lenoir, Rui F. Simões, José Teixeira, Liana C. Silva, Dominique Wendum, Isabelle Jéru, Corinne Vigouroux, Rui E. Castro, Paulo J. Oliveira, Carina Prip‐Buus, Vlad Ratziu, Jérémie Gautheron, Cecília M. P. Rodrigues   +16 more
wiley   +1 more source

Dapagliflozin attenuates high glucose-and hypoxia/reoxygenation-induced injury via activating AMPK/mTOR-OPA1-mediated mitochondrial autophagy in H9c2 cardiomyocytes

, 2023
This study investigated the protective effect of dapagliflozin on H9c2 cardiomyocyte function under high glucose and hypoxia/reoxygenation (HG-H/R) conditions and identified the underlying molecular mechanisms.
Xiaoqing Wang (514179), Yan Sun (22436), Weiling Tu (16919387), Liang Li (46069), Junyu Chen (4263742), Ming Yi (15051)   +5 more
core   +1 more source

Solving a 50 year mystery of a missing OPA1 mutation: more insights from the first family diagnosed with autosomal dominant optic atrophy [PDF]

, 2010
Background: Up to the 1950s, there was an ongoing debate about the diversity of hereditary optic neuropathies, in particular as to whether all inherited optic atrophies can be ascribed to Leber's hereditary optic neuropathy (LHON) or represent different ...
Alexander Christiane, Schimpf Simone, Bernd Wissinger, Zrenner, Eberhart, Alavi, M.V., Schimpf, Simone, Auburger Georg, Zrenner, E., Zrenner Eberhart, Schimpf, S., Alavi-Khorassani Moghadam, Marcel Victor, Auburger, Georg, Leo-Kottler, Beate, Marcel V Alavi, Wissinger, Bernd, Christiane Alexander, Nico Fuhrmann, Wissinger, B., Fuhrmann Nico, Beate Leo-Kottler, Alexander, Christiane, Kamenisch, York, Fuhrmann, Nico, Kamenisch, Y., Wissinger Bernd, Auburger, G., Simone Schimpf, Eberhart Zrenner, Georg Auburger, Kamenisch York, York Kamenisch, Alexander, C., Leo-Kottler, B., Leo-Kottler Beate, Alavi Marcel V, Fuhrmann, N.   +35 more
core   +1 more source

Extramitochondrial OPA1 and adrenocortical function [PDF]

, 2013
We have previously described that silencing of the mitochondrial protein OPA1 enhances mitochondrial 27 Ca2+ signaling and aldosterone production in H295R adrenocortical cells.
Fülöp, László, Spät András, Szanda, Gergö, Katona, Dávid, Rajki Anikó, Rajki, Anikó, Fülöp László, Szanda Gergő, Spät, András, Katona Dávid   +9 more
core   +1 more source

Two forms of Opa1 cooperate to complete fusion of the mitochondrial inner-membrane

eLife, 2020
Mitochondrial membrane dynamics is a cellular rheostat that relates metabolic function and organelle morphology. Using an in vitro reconstitution system, we describe a mechanism for how mitochondrial inner-membrane fusion is regulated by the ratio of two
Yifan Ge, Xiaojun Shi, Sivakumar Boopathy, Julie McDonald, Adam W Smith, Luke H Chao   +5 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

Coassembly of Mgm1 isoforms requires cardiolipin and mediates mitochondrial inner membrane fusion [PDF]

, 2009
Two dynamin-related protein (DRP) families are essential for fusion of the outer and inner mitochondrial membranes, Fzo1 (yeast)/Mfn1/Mfn2 (mammals) and Mgm1 (yeast)/Opa1 (mammals), respectively.
Stahlberg, Henning, Laura L. Lackner, Suzanne Hoppins, Nunnari, Jodi, Jodi Nunnari, Henning Stahlberg, Dominguez-Ramirez, Lenin, Lenin Dominguez-Ramirez, Hoppins, Suzanne, Lackner, Laura L., Rachel M. DeVay, DeVay, Rachel M.   +11 more
core   +1 more source

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

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