Clinical and Biochemical Features in a Patient With Mitochondrial Fission Factor Gene Alteration. [PDF]
Mitochondrial Fission Factor (MFF) is part of a protein complex that promotes mitochondria and peroxisome fission. Hitherto, only 5 patients have been reported harboring mutations in MFF, all of them with the clinical features of a very early onset Leigh-like encephalopathy. We report on an 11-year-old boy with epileptic encephalopathy.
Nasca A +7 more
europepmc +5 more sources
Nanobodies as novel tools to monitor the mitochondrial fission factor Drp1. [PDF]
In cells, mitochondria undergo constant fusion and fission. An essential factor for fission is the mammalian dynamin-related protein 1 (Drp1). Dysregulation of Drp1 is associated with neurodegenerative diseases including Parkinson’s, cardiovascular diseases and cancer, making Drp1 a pivotal biomarker for monitoring mitochondrial status and potential ...
Froehlich T +12 more
europepmc +5 more sources
microRNA-200a-3p enhances mitochondrial elongation by targeting mitochondrial fission factor. [PDF]
Mitochondria play pivotal roles in the ATP production, apoptosis and generation of reactive oxygen species. Although dynamic regulation of mitochondria morphology is a critical step to maintain cellular homeostasis, the regulatory mechanisms are not yet fully elucidated.
Lee H +5 more
europepmc +5 more sources
Mitochondrial Fission Factor (MFF) Inhibits Mitochondrial Metabolism and Reduces Breast Cancer Stem Cell (CSC) Activity. [PDF]
Elevated mitochondrial biogenesis and metabolism represent key features of breast cancer stem cells (CSCs), whose propagation is conducive to disease onset and progression. Therefore, interfering with mitochondria biology and function may be regarded as a useful approach to eradicate CSCs.
Sánchez-Alvarez R +4 more
europepmc +8 more sources
Mitochondrial fission factor drives an actionable metabolic vulnerability in multiple myeloma. [PDF]
Proliferating multiple myeloma (MM) cells in the bone marrow fluctuate across various metabolic states to resist cancer treatments. Herein, we investigate how mitochondrial dynamics, which controls mitochondrial fitness via coordinated fission and fusion events, shapes MM cell metabolism impacting growth, survival and drug sensitivity. We identify MFF (
Cantafio MEG +19 more
europepmc +4 more sources
Parkin-mediated ubiquitination contributes to the constitutive turnover of mitochondrial fission factor (Mff). [PDF]
The mitochondrial outer membrane protein Mitochondrial Fission Factor (Mff) plays a key role in both physiological and pathological fission. It is well established that at stressed or functionally impaired mitochondria, PINK1 recruits the ubiquitin ligase Parkin which ubiquitinates Mff and other mitochondrial outer membrane proteins to facilitate the ...
Lee L +4 more
europepmc +8 more sources
Non-alcoholic fatty liver disease in mice with hepatocyte-specific deletion of mitochondrial fission factor. [PDF]
Abstract Aims/hypothesis Mitochondria are highly dynamic organelles continuously undergoing fission and fusion, referred to as mitochondrial dynamics, to adapt to nutritional demands. Evidence suggests that impaired mitochondrial dynamics leads to metabolic abnormalities such as non-alcoholic steatohepatitis (NASH ...
Takeichi Y +13 more
europepmc +4 more sources
miR-27 regulates mitochondrial networks by directly targeting the mitochondrial fission factor [PDF]
Mitochondrial morphology is dynamically regulated by forming small, fragmented units or interconnected networks, and this is a pivotal process that is used to maintain mitochondrial homeostasis. Although dysregulation of mitochondrial dynamics is related to the pathogenesis of several human diseases, its molecular mechanism is not fully elucidated.
Tak, Hyosun +9 more
openaire +2 more sources
Interaction of mitochondrial fission factor with dynamin related protein 1 governs physiological mitochondrial function in vivo. [PDF]
AbstractMitochondria form a dynamic network governed by a balance between opposing fission and fusion processes. Because excessive mitochondrial fission correlates with numerous pathologies, including neurodegeneration, the mechanism governing fission has become an attractive therapeutic strategy.
Kornfeld OS +5 more
europepmc +5 more sources
Control of mitochondrial morphology through differential interactions of mitochondrial fusion and fission proteins. [PDF]
Mitochondria in mammals are organized into tubular networks that undergo frequent shape change. Mitochondrial fission and fusion are the main components mediating the mitochondrial shape change.
Pinwei Huang +2 more
doaj +1 more source

