Results 111 to 120 of about 21,239 (181)

MFN2 coordinates mitochondria motility with α-tubulin acetylation and this regulation is disrupted in CMT2A

iScience
Summary: Mitofusin-2 (MFN2), a large GTPase residing in the mitochondrial outer membrane and mutated in Charcot-Marie-Tooth type 2 disease (CMT2A), is a regulator of mitochondrial fusion and tethering with the ER.
Atul Kumar, Delfina Larrea, Maria Elena Pero, Paola Infante, Marilisa Conenna, Grace J. Shin, Vincent Van Elias, Wesley B. Grueber, Lucia Di Marcotullio, Estela Area-Gomez, Francesca Bartolini   +10 more
doaj   +1 more source

Role of the Ca2+-Calcineurin-Nuclear Factor of Activated T cell Pathway in Mitofusin-2-Mediated Immune Function of Jurkat Cells

Chinese Medical Journal, 2018
Background: Mitofusin-2 (MFN2), a well-known mitochondrial fusion protein, has been shown to participate in innate immunity, but its role in mediating adaptive immunity remains poorly characterized.
Xiu-Ping Xu, Yong-Ming Yao, Guang-Ju Zhao, Zong-Sheng Wu, Jun-Cong Li, Yun-Long Jiang, Zhong-Qiu Lu, Guang-Liang Hong   +7 more
doaj   +1 more source

Heterogeneous metabolic response of endothelial cells from different vascular beds to experimental hyperglycaemia and metformin

The Journal of Physiology, EarlyView.
Abstract figure legend Diabetes mellitus is associated with vascular pathology that leads to vascular complications in several tissues, such as retinopathy of the eye. Endothelial dysfunction plays a pivotal role in the progression of each complication.
C. McAleese, G. Joudah, I. P. Salt, J. R. Petrie, J. M. Leiper, Laura B. Dowsett   +5 more
wiley   +1 more source

Fetal glucose availability: a key regulator of the metabolic, hormonal and contractility profiles of the fetal sheep heart

The Journal of Physiology, EarlyView.
Abstract figure legend To investigate the role of glucose availability in fetal left ventricle (LV) development, this study assessed whether maternal late gestation undernutrition (LGUN; 50% of Control diet) induced alterations in the contractility, metabolic, and hormonal profile can be ameliorated in LGUN fetuses receiving glucose infusion (LGUN+G ...
Melanie R. Bertossa, Jack R. T. Darby, Stacey L. Holman, Steven K. S. Cho, Ashley S. Meakin, Mitchell C. Lock, Jordan A. Minns, Michael D. Wiese, Christopher K. Macgowan, Mike Seed, Janna L. Morrison   +10 more
wiley   +1 more source

Energetic microdomains and the vascular control of neuronal and muscle excitability: Toward a unified model

The Journal of Physiology, EarlyView.
Abstract figure legend The capillary–mitochondria–ion channel (CMIC) axis scales structural resources to match functional workload. (Left) In settings of restricted energetic capacity (e.g. cortical neurons), sparse capillary networks and modest mitochondrial pools set a lower energetic ceiling, sufficient to support phasic, low‐workload excitability. (
L. Fernando Santana, Scott Earley
wiley   +1 more source

Demonstration of beat‐to‐beat, on‐demand ATP synthesis in ventricular myocytes reveals sex‐specific mitochondrial and cytosolic dynamics

The Journal of Physiology, EarlyView.
Abstract figure legend Beat‐locked mitochondrial ATP transients reveal modular, sex‐specific bioenergetic control during excitation–contraction coupling. A, each action potential activates L‐type CaV1.2 channels, producing a Ca2+ influx that triggers ryanodine receptors (RyR2) and elicits SR Ca2+ release.
Paula Rhana, Collin Matsumoto, L. Fernando Santana   +2 more
wiley   +1 more source

Mfn2 regulates calcium homeostasis and suppresses PASMCs proliferation via interaction with IP3R3 to mitigate pulmonary arterial hypertension

Journal of Translational Medicine
Background Pulmonary arterial hypertension (PAH) is a chronic disorder characterized by the excessive proliferation of pulmonary arterial smooth muscle cells (PASMCs).
Rui Wang, Jie Wang, Jing Yu, Zhiqiang Li, Minfang Zhang, Yuhu Chen, Fen Liu, Dongmei Jiang, Jingfei Guo, Xiaomei Li, Yun Wu   +10 more
doaj   +1 more source

Biallelic Nonsense Variants in NEFL May Cause a Non‐Length‐Dependent Neuropathy With Temporal Dispersion on Nerve Conduction Studies

Journal of the Peripheral Nervous System, Volume 31, Issue 2, June 2026.
ABSTRACT Background and Aims Pathogenic variants in NEFL, the gene that encodes the light polypeptide subunit of neurofilaments, are an uncommon cause of autosomal recessive Charcot‐Marie‐Tooth (CMT) disease. In this study, we describe the clinical and electrophysiological features of two families with early‐onset CMT carrying nonsense variants in the ...
Marcus Vinícius Vieira da Silva Gomes, Pedro José Tomaselli, Wilson Marques Jr   +2 more
wiley   +1 more source

Mitofusin 2 Promotes Apoptosis of CD4+ T Cells by Inhibiting Autophagy in Sepsis

Mediators of Inflammation, 2017
Apoptosis of CD4+ T cells is a primary pathophysiological mechanism of immune dysfunction in the pathogenesis of sepsis. Mitofusin 2 (Mfn2), an integral mitochondrial outer membrane protein, has been confirmed to be associated with cellular metabolism ...
Lan Ying, Guang-Ju Zhao, You Wu, He-Liang Ke, Guang-Liang Hong, Hui Zhang, Ning Dong, Yao Wu, Yong-Ming Yao, Zhong-Qiu Lu   +9 more
doaj   +1 more source

PIK3CA Mutations Downregulate PPT1 to Promote Adipogenesis by Suppressing P300 Depalmitoylation and Phase Separation

Advanced Science, Volume 13, Issue 19, 2 April 2026.
This study demonstrates that somatic PIK3CA mutations suppress PPT1 expression via activation of the PI3K–AKT–c‐JUN axis. This reduction in PPT1 weakens its interaction with P300, thereby increasing palmitoylation at C1176 of P300 and protecting P300 from lysosomal degradation.
Hongrui Chen, Zening Huang, Rui Chang, Wei Gao, Yajing Qiu, Bin Sun, Chen Hua, Xiaoxi Lin   +7 more
wiley   +1 more source