Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy [PDF]
Human Molecular Genetics, 2010 Mitochondrial dysfunction and perturbed degradation of proteins have been implicated in Parkinson's disease (PD) pathogenesis. Mutations in the Parkin and PINK1 genes are a cause of familial PD. PINK1 is a putative kinase associated with mitochondria, and loss of PINK1 expression leads to mitochondrial dysfunction, which increases with time.
Matthew E, Gegg +5 more
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Mitofusin 2 ablation increases endoplasmic reticulum–mitochondria coupling [PDF]
Proceedings of the National Academy of Sciences, 2015 Significance The privileged interrelationship between mitochondria and the endoplasmic reticulum (ER) plays a key role in a variety of physiological functions, from lipid metabolism to Ca 2+ signalling, and its modulation influences apoptotic susceptibility, mitophagy, and ...
Filadi Riccardo +5 more
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Redox Biology, 2020 UCH-L1 is a deubiquitinating enzyme (DUB), highly abundant in neurons, with a sub-cellular localization dependent on its farnesylation state. Despite UCH-L1′s association with familial Parkinson's Disease (PD), the effects on mitochondrial bioenergetics ...
Fernanda M. Cerqueira +9 more
doaj +1 more source
Differentiation state-specific mitochondrial dynamic regulatory networks are revealed by global transcriptional analysis of the developing chicken lens. [PDF]
, 2014 The mature eye lens contains a surface layer of epithelial cells called the lens epithelium that requires a functional mitochondrial population to maintain the homeostasis and transparency of the entire lens.
Anastas, Sara +9 more
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iScience, 2023 Summary: Defects in mitochondrial fusion are at the base of many diseases. Mitofusins power membrane-remodeling events via self-interaction and GTP hydrolysis.
Ira Buntenbroich +8 more
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Natural and Induced Mitochondrial Phosphate Carrier Loss: DIFFERENTIAL DEPENDENCE OF MITOCHONDRIAL METABOLISM AND DYNAMICS AND CELL SURVIVAL ON THE EXTENT OF DEPLETION. [PDF]
, 2016 The relevance of mitochondrial phosphate carrier (PiC), encoded by SLC25A3, in bioenergetics is well accepted. However, little is known about the mechanisms mediating the cellular impairments induced by pathological SLC25A3 variants.
Acoba, Michelle G. +9 more
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Protein & Cell, 2017 Hypertonia is a neurological dysfunction associated with a number of central nervous system disorders, including cerebral palsy, Parkinson’s disease, dystonia, and epilepsy.
Crystal A. Lee, Lih-Shen Chin, Lian Li
doaj +1 more source
Mitofusin 2 Deficiency Affects Energy Metabolism and Mitochondrial Biogenesis in MEF Cells. [PDF]
PLoS ONE, 2015 Mitofusin 2 (Mfn2), mitochondrial outer membrane protein which is involved in rearrangement of these organelles, was first described in pathology of hypertension and diabetes, and more recently much attention is paid to its functions in Charcot-Marie ...
Maria Kawalec +5 more
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Manipulation of Mitochondria Dynamics Reveals Separate Roles for Form and Function in Mitochondria Distribution [PDF]
, 2018 Mitochondria shape is controlled by membrane fusion and fission mediated by mitofusins, Opa1, and Drp1, whereas mitochondrial motility relies on microtubule motors.
Bova, Sergio +5 more
core +2 more sources
Calcium Signalling: Mitofusins Promote Interorganellar Crosstalk [PDF]
Current Biology, 2009 Juxtaposition of the endoplasmic reticulum with mitochondria facilitates Ca(2+) fluxes between the organelles. However, the molecular basis of interorganellar tethering remains unknown. A recent report has identified a fundamental role for the dynamin-related mitofusins in the tethering mechanism, thereby ensuring rapid and high fidelity Ca(2 ...
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