Mitofusins, from mitochondria to metabolism

E Schrepfer, L Scorrano - Molecular cell, 2016 - cell.com
Mitochondrial architecture is involved in several functions crucial for cell viability,
proliferation, senescence, and signaling. In particular, mitochondrial dynamics, through the …

Mitochondrial dynamics: coupling mitochondrial fitness with healthy aging

D Sebastián, M Palacín, A Zorzano - Trends in molecular medicine, 2017 - cell.com
Aging is associated with a decline in mitochondrial function and the accumulation of
abnormal mitochondria. However, the precise mechanisms by which aging promotes these …

Deficient endoplasmic reticulum-mitochondrial phosphatidylserine transfer causes liver disease

MI Hernández-Alvarez, D Sebastián, S Vives… - Cell, 2019 - cell.com
Non-alcoholic fatty liver is the most common liver disease worldwide. Here, we show that the
mitochondrial protein mitofusin 2 (Mfn2) protects against liver disease. Reduced Mfn2 …

Mfn2 is critical for brown adipose tissue thermogenic function

M Boutant, SS Kulkarni, M Joffraud, J Ratajczak… - The EMBO …, 2017 - embopress.org
Mitochondrial fusion and fission events, collectively known as mitochondrial dynamics, act
as quality control mechanisms to ensure mitochondrial function and fine‐tune cellular …

Mfn2 deficiency links age‐related sarcopenia and impaired autophagy to activation of an adaptive mitophagy pathway

D Sebastián, E Sorianello, J Segalés, A Irazoki… - The EMBO …, 2016 - embopress.org
Mitochondrial dysfunction and accumulation of damaged mitochondria are considered major
contributors to aging. However, the molecular mechanisms responsible for these …

MFN2 mutations in Charcot–Marie–Tooth disease alter mitochondria-associated ER membrane function but do not impair bioenergetics

D Larrea, M Pera, A Gonnelli… - Human molecular …, 2019 - academic.oup.com
Abstract Charcot–Marie–Tooth disease (CMT) type 2A is a form of peripheral neuropathy,
due almost exclusively to dominant mutations in the nuclear gene encoding the …

Mfn2 localization in the ER is necessary for its bioenergetic function and neuritic development

S Casellas‐Díaz, R Larramona‐Arcas… - EMBO …, 2021 - embopress.org
Mfn2 is a mitochondrial fusion protein with bioenergetic functions implicated in the
pathophysiology of neuronal and metabolic disorders. Understanding the bioenergetic …

Oroxylin a promotes PGC-1α/Mfn2 signaling to attenuate hepatocyte pyroptosis via blocking mitochondrial ROS in alcoholic liver disease

J Kai, X Yang, Z Wang, F Wang, Y Jia, S Wang… - Free Radical Biology …, 2020 - Elsevier
Background It is well acknowledged that alcoholic liver disease (ALD) is widely prevalent all
over the world, characterized by aberrant lipid deposition and excessive oxidative stress in …

Three‐dimensional reconstruction of the human skeletal muscle mitochondrial network as a tool to assess mitochondrial content and structural organization

R Dahl, S Larsen, TL Dohlmann, K Qvortrup… - Acta …, 2015 - Wiley Online Library
Aim Mitochondria undergo continuous changes in shape as result of complex fusion and
fission processes. The physiological relevance of mitochondrial dynamics is still unclear. In …

Mitofusins: disease gatekeepers and hubs in mitochondrial quality control by E3 ligases

M Escobar-Henriques, M Joaquim - Frontiers in physiology, 2019 - frontiersin.org
Mitochondria are dynamic organelles engaged in quality control and aging processes. They
constantly undergo fusion, fission, transport, and anchoring events, which empower …