• Additionally, exacerbated type I IFN responses triggered by mitochondrial DNA (mtDNA), failures in mitophagy, ER-mitochondria communication and mtROS production promote neurodegeneration. (frontiersin.org)
  • Mitochondria are highly dynamic organelles undergoing coordinated cycles of fission and fusion, referred as 'mitochondrial dynamics', in order to maintain their shape, distribution and size. (cam.ac.uk)
  • Mitochondrial fission is a multi-step process allowing the division of one mitochondrion in two daughter mitochondria. (cam.ac.uk)
  • In this work, we show that the Sel1L-Hrd1 ERAD complex is essential for cold-induced thermogenesis in part by managing mitochondrial dynamics and ER-mitochondria contact in brown adipocytes. (diabetesjournals.org)
  • These dynamic processes are necessary for maintenance of functional mitochondria and mitochondrial DNA, distribution of mitochondria to synapses, energy production, cell death mechanisms, and proper synaptic development and function. (neurodegenerationresearch.eu)
  • Our findings suggest that empagliflozin enters cardiac mitochondria and directly causes these effects by increasing mitochondrial ATP via inhibition of NHE1 and Nav1.5 or their common downstream sites. (nature.com)
  • We demonstrate here that our newly generated tools, including transgenic "MitoFish," can be used to study the in vivo "life cycle" of mitochondria and allows identifying pharmacological and genetic modulators of mitochondrial dynamics. (uni-muenchen.de)
  • Thus, our approach allows detailed studies of the dynamics of mitochondria in their natural environment under normal and disease conditions. (uni-muenchen.de)
  • Glutamate excitotoxicity leads to fragmented mitochondria in neurodegenerative diseases, mediated by nitric oxide and S-nitrosylation of dynamin-related protein 1, a mitochondrial outer membrane fission protein. (omicsdi.org)
  • While OPA1 has been characterized for its role in mitochondrial cristae structure and organelle fusion, possible effects of OPA1 on mitochondrial function have not been determined.Mitochondria from six ADOA patients bearing OPA1 mutations and ten ADOA patients with unidentified gene mutations were studied for respiratory capacity and electron transport complex function. (omicsdi.org)
  • The alteration of the mitochondrial network only when cells are challenged in aerobiosis testifies to the fragility of mitochondria, which are unable to meet the metabolic needs of neurons. (fortuneonline.org)
  • Once within mitochondria , esterases remove the AM ester , trapping RhoVR inside of the mitochondrial matrix, where it can incorporate within the inner membrane and reversibly report on changes in ΔΨm. (bvsalud.org)
  • turn, these repositioned mitochondria supported increased lamellipodia dynamics, faster turnover of focal adhesion complexes, heightened velocity and distance of random cell migration and increased tumor cell invasion. (angiogenesis-blog.com)
  • Fresh mitochondria isolated from homeoplasmic 143B osteosarcoma cybrids containing mitochondrial DNA (mtDNA) derived from health individuals (Mito) or mtDNA with the A8344G mutation (Mito 8344 ) were conjugated with cell-penetrating peptide Pep-1 (P-Mito) or not conjugated prior to cell co-culture. (biomedcentral.com)
  • Furthermore, although there were no differences in energy metabolism after transplantation of normal mitochondria, metabolism was switched to the energetic and glycolytic phenotypes when the mitochondria were replaced with dysfunctional mitochondria, namely, Mito 8344 and P-Mito 8344 , due to dramatically induced glycolysis and reduced mitochondrial respiration, respectively. (biomedcentral.com)
  • We show that mitochondria in the RPE form extensive networks that are destroyed by fixation and discuss important live imaging considerations that can interfere with accurate evaluation of mitochondrial integrity such as RPE differentiation status and acquisition parameters. (figshare.com)
  • Contacts between the mitochondria and organelles such as the ER and lysosomes mediate calcium flux and mitochondrial fission. (figshare.com)
  • We analyzed the organization and function of mitochondrial DNA in a stable human cell line (ECV304, which is also known as T-24) containing mitochondria tagged with the yellow fluorescent protein. (biomedcentral.com)
  • Then, proteins encoded by the nuclear genome and destined for the mitochondria will be made close to mitochondrial-encoded proteins so that they can be assembled efficiently into mitochondrial complexes. (biomedcentral.com)
  • In most human cells, mitochondria contain 10 3 -10 4 copies of a circular genome of 16,569 base-pairs that encodes two ribosomal RNAs, 22 tRNAs, and 13 polypeptides that form parts of the respiratory-chain located in the inner mitochondrial membrane [ 9 , 10 ]. (biomedcentral.com)
  • We now examine the organization of mitochondrial genomes in a human cell line that contains mitochondria tagged with the yellow fluorescent protein (YFP). (biomedcentral.com)
  • Drp1 is thus critical for targeting mitochondria to the nerve terminal, and a disruption in mitochondrial fission can contribute to the preferential death of nigrostriatal DA neurons. (jneurosci.org)
  • First of all, I will examine whether the mitochondrial SPIRE1 actin nucleator regulates the localization of mitochondria in neurons. (uni-regensburg.de)
  • Beyond the organelle dynamics, the constant interaction with the ubiquitin-proteasome-system (UPS) has become an emerging aspect of healthy mitochondria. (hindawi.com)
  • In line with this notion, mitochondria are highly dynamic organelles that undergo fission and fusion and move into the cell along the microtubules to generate the mitochondrial network [ 6 ]. (hindawi.com)
  • Nonetheless their association with mitochondrial dysfunction highlights this organelle as an important player during CNS homeostasis and disease. (frontiersin.org)
  • Mitochondrial function or the ability to generate energy through OXPHOS (oxidative phosphorylation) is vital for cell homeostasis and its dysfunction has been linked to the pathogenesis of nearly all chronic diseases ( Pieczenik and Neustadt, 2007 ). (frontiersin.org)
  • Relevant to the studies presented in this manuscript, mitochondrial dysfunction is a key contributor to the perpetuation of metabolic inflammation caused by nutrient excess. (frontiersin.org)
  • Besides OXPHOS, mitochondrial dysfunction is also present in the examination of mitochondrial morphology, the balance of mitochondrial network formation, and fission and fusion. (frontiersin.org)
  • The mitochondrial cascade hypothesis of dementia assumes mitochondrial dysfunction leading to reduced energy supply, impaired neuroplasticity, and finally cell death as one major pathomechanism underlying the continuum from brain aging over mild cognitive impairment to initial and advanced late onset Alzheimer's disease. (nih.gov)
  • 1,2 The molecular mechanisms underlying striatal vulnerability are still unknown, but growing evidences suggest that mitochondrial dysfunction occurs during the pathogenesis of the disease. (bmj.com)
  • The goal is to define an effective post-exposure, non-invasive intervention strategy to mitigate a primary cause of loud-sound induced hearing loss mitochondrial dysfunction and overproduction of reactive oxygen species. (dtic.mil)
  • While largely indistinguishable from their wildtype littermates, adipocyte-specific Sel1L-deficient mice are cold sensitive associated with mitochondrial dysfunction. (diabetesjournals.org)
  • One of the underlying mechanisms of long COVID may be mitochondrial dysfunction. (mdpi.com)
  • We speculate that PMP22 may promote mitochondrial dysfunction, myelin having a role in mitochondrial metabolism. (fortuneonline.org)
  • Many studies implicate mitochondrial dysfunction as a key contributor to cell loss in Parkinson disease (PD). (nature.com)
  • Cancer cells are generally subjected to high levels of reactive oxygen species (ROS) compared to normal cells because ROS generation is augmented by mtDNA mutations and mitochondrial dysfunction [ 6 ]. (biomedcentral.com)
  • furthermore, it is also becoming evident that mitochondrial dysfunction may play a causative role in determining muscle atrophy. (unipv.it)
  • Mitochondrial dysfunction is strongly implicated in neurodegenerative diseases including age-related macular degeneration (AMD), which causes irreversible blindness in over 50 million older adults worldwide. (figshare.com)
  • Recent studies from our group and others have identified several features of mitochondrial dysfunction in AMD including mitochondrial fragmentation and bioenergetic defects. (figshare.com)
  • We hypothesized that testosterone deprivation aggravates LV dysfunction and cardiac autonomic imbalance via the impairment of cardiac mitochondrial function and dynamics proteins, a reduction in insulin receptor function, and an increase in apoptosis in obese insulin-resistant rats. (konexionsnc.com)
  • Higher levels of SJL have been associated with lower levels of cardiorespiratory fitness, and increased incidence of cardiometabolic disease, which may be due, in part, to mitochondrial dysfunction. (wku.edu)
  • Mitochondrial dysfunction has also been associated with ageing and most of the so-called age-related diseases [ 13 - 17 ]. (hindawi.com)
  • The metabolic enhancer piracetam has been proposed as possible prototype for those compounds by increasing impaired mitochondrial function and related aspects like mechanisms of neuroplasticity. (nih.gov)
  • To recap, the MID51 and MID49 are both similar proteins that recruit Drp1 protein to induce mitochondrial binary fission but they have small differences in structure which allows them to bind Drp1 in different ways. (wikipedia.org)
  • Meanwhile, the mitochondrial function, morphology and expression of dynamic-related proteins suggested that low-dose SFN protected against hypoxia-induced mitochondrial injury and alleviated hypoxia-induced fission Nrf2-dependently without affecting the expression of key effector proteins (Drp1, Fis1, Mfn1/2 and Opa1), while high concentrations (≥10 μM SFN) aggravated hypoxia-induced mitochondrial injury, fission and Drp1 expression, and inhibited Mfn1/2 expression. (uea.ac.uk)
  • In the present study, we extensively investigated the role of mitochondrial dynamics-related proteins and their epigenetic regulation in ischemic liver injury following AHF and explored the possible hepatoprotective role of carvedilol. (uky.edu)
  • Our results indicated that the hepatoprotective effect of carvedilol in ameliorating hepatic ischemic damage could be attributed to its ability to target the mitochondrial dynamics-related proteins (Mtf2, DNM1L and PGC-1α), but also their epigenetic regulator miRNA-17. (uky.edu)
  • Mitochondrial transplantation also consistently decreased Drp-1, which resulted in an enhancement of the tubular mitochondrial network, but a distinct machinery through the increase of parkin and mitochondrial fusion proteins was observed in the Mito and P-Mito groups, respectively. (biomedcentral.com)
  • 0.05), SJL decreased expression of mitochondrial fusion proteins MFN1 and OPA1 (Main Effect - SJL, p (wku.edu)
  • This mitochondrial RNA colocalizes with components of the cytoplasmic machinery that makes and imports nuclear-encoded proteins - that is, a ribosomal protein (S6), a nascent peptide associated protein (NAC), and the translocase in the outer membrane (Tom22). (biomedcentral.com)
  • Differences in mechanical and fatigue properties of diaphragm motor units are the result of expression of different contractile proteins and mitochondrial volume densities in corresponding muscle fiber types. (mayo.edu)
  • Mutations in the core machinery components and defects in mitochondrial dynamics have been associated with numerous human diseases. (cam.ac.uk)
  • We employ mitochondrial single-cell assay for transposase-accessible chromatin with sequencing to profile 163,279 cells from 9 patients with chronic lymphocytic leukemia (CLL) collected across disease course and utilize mitochondrial DNA (mtDNA) mutations as natural genetic markers of cancer clones. (nih.gov)
  • mtDNA mutations thus mirror disease history and provide naturally occurring genetic barcodes to enable patient-specific study of cancer subclonal dynamics. (nih.gov)
  • Single-cell multi-omic profiling of CLL reveals the utility of somatic mtDNA mutations as in vivo barcodes, which mark subclones that can evolve over time along with changes in accessible chromatin and gene expression profiles to capture dynamics of disease evolution. (nih.gov)
  • Autosomal dominant optic atrophy (ADOA), a form of progressive bilateral blindness due to loss of retinal ganglion cells and optic nerve deterioration, arises predominantly from mutations in the nuclear gene for the mitochondrial GTPase, OPA1. (omicsdi.org)
  • Results suggest that the nuclear DNA mutations that give rise to ADOA in our patient population do not alter mitochondrial electron transport.We conclude that the pathophysiology of ADOA likely stems from the role of OPA1 in mitochondrial structure or fusion and not from OPA1 support of oxidative phosphorylation. (omicsdi.org)
  • Charcot-Marie-Tooth disease type 2A (CMT2A) is an inherited axonal peripheral neuropathy mainly caused by mutations in the mitofusin 2 (MFN2) gene encoding for the MFN2 protein, a GTPase involved in the mitochondrial dynamics and bioenergetics. (fortuneonline.org)
  • Mitochondrial DNA (mtDNA) mutations cause severe congenital diseases but may also be associated with healthy aging. (uib.no)
  • The aberrant kinase activity of pathogenic LRRK2 mutations apparently induce neurodegeneration by disturbing intracellular processes such as protein translation, endolysosomal pathway, autophagy, synaptic functions, and cytoskeleton dynamics. (medscape.com)
  • Mutations in the OPA3 gene lead to abnormal mitochondrial function. (medlineplus.gov)
  • Some causative mutations arise in the nuclear genome (for example, in NDUFS4 , 7 , and 8 in Leigh and Leigh-like syndromes), others in the mitochondrial one. (biomedcentral.com)
  • Altogether, our findings support the hypothesis that Cdk5 plays a crucial role in mitochondrial defects involved in the striatal neurodegeneration in HD. (bmj.com)
  • This gene encodes an outer mitochondrial membrane protein that functions in the regulation of mitochondrial morphology. (wikipedia.org)
  • On the other hand, regulation of mitochondrial dynamics is essential for CNS health maintenance and leading to the induction of IL-10 and reduction of TNF-α secretion, increased cell viability and diminished cell injury in addition to reduced oxidative stress. (frontiersin.org)
  • Neuronal regulation of mitochondrial dynamics in models of Parkinsons disease. (neurodegenerationresearch.eu)
  • We and others have found differences in regulation of mitochondrial dynamics in neurons and have found that differences in neuronal bioenergetics may at least in part be responsible, suggesting the metabolic state of the cell is important. (neurodegenerationresearch.eu)
  • Thus, there is a critical need to understand neuron-specific regulation of mitochondrial dynamics and how this is altered in PD. (neurodegenerationresearch.eu)
  • These studies will provide important information on neuronal regulation of mitochondrial dynamics, as well as provide a better understanding of the integrated role of mitochondrial dynamics in PD. (neurodegenerationresearch.eu)
  • This study reveals the antitumour potential of mitochondrial transplantation in breast cancer via distinct regulation of mitochondrial function. (biomedcentral.com)
  • In my master thesis, I investigated the regulation of mitochondrial motility by actin/myosin force generation. (uni-regensburg.de)
  • a transcript generated from one of the promoters also primes mitochondrial DNA (mtDNA) replication. (biomedcentral.com)
  • For example, A to G transitions in nucleotides 3243 and 8344 in mtDNA underlie mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) and myoclonic epilepsy with ragged red fibres (MERFF), respectively. (biomedcentral.com)
  • Importantly, we will also expand our work to include imaging and quantification of mitochondrial dynamics directly, in dopamine (DA) neurons, in an innovative, living vertebrate PD model, thus better dissecting the role of mitochondrial dynamics in early neuropathology in PD. (neurodegenerationresearch.eu)
  • The MID49 peripheral protein will attract the Drp1 to the surface of the mitochondrial membrane which then induces endoplasmic reticulum contact. (wikipedia.org)
  • It can directly recruit the fission mediator dynamin-related protein 1 (Drp1) to the mitochondrial surface. (wikipedia.org)
  • It is regulated by the recruitment of the GTPase Dynamin-related protein 1 (Drp1) by adaptors at actin- and endoplasmic reticulum-mediated mitochondrial constriction sites. (cam.ac.uk)
  • Drp1 oligomerization followed by mitochondrial constriction leads to the recruitment of Dynamin 2 to terminate membrane scission. (cam.ac.uk)
  • Indeed, a link between hypoxia and mitochondrial dynamics, fission via Drp1 mostly, continues to be noted.38 In addition, increased mitochondrial fission by hypoxia was shown to enhance breast cancer invasion.39 Whether a conserved mitochondrial trafficking mechanism reminiscent of the one described in hypoxic neurons40,41 is at play in actively invasive tumor cells, awaits confirmation. (angiogenesis-blog.com)
  • To study one aspect of mitochondrial dynamics-mitochondrial fission-in mouse DA neurons, we deleted the central fission protein dynamin-related protein 1 (Drp1). (jneurosci.org)
  • Without Drp1, mitochondrial mass dramatically decreases, especially in axons, where the mitochondrial movement becomes uncoordinated. (jneurosci.org)
  • It is a dynamic peripheral protein receptor found on the surface of the mitochondrial membrane. (wikipedia.org)
  • Inner mitochondrial membrane constriction has been proposed to be an independent process regulated by calcium influx. (cam.ac.uk)
  • Mitochondrial fusion is driven by a two-step process with the outer mitochondrial membrane fusion mediated by mitofusins 1 and 2 followed by inner membrane fusion, mediated by optic atrophy 1. (cam.ac.uk)
  • Optic atrophy gene 1 (OPA1) is an inner membrane protein important for mitochondrial fusion. (omicsdi.org)
  • OPA1 is a fission-fusion protein involved in mitochondrial inner membrane remodelling. (omicsdi.org)
  • OPA1 localizes to mitochondrial cristae in the inner membrane where electron transport chain complexes are enriched. (omicsdi.org)
  • Imaging Reversible Mitochondrial Membrane Potential Dynamics with a Masked Rhodamine Voltage Reporter. (bvsalud.org)
  • This negative membrane potential across the inner mitochondrial membrane (ΔΨm) represents a fundamental biophysical parameter central to cellular life . (bvsalud.org)
  • SPIRIT RhoVR is compatible with multi- color imaging, enabling simultaneous, real- time observation of cytosolic Ca2+, plasma membrane potential, and reversible ΔΨm dynamics. (bvsalud.org)
  • Characteristics of the movement of K+ across the mitochondrial membrane and the inhibitory action of Tl+. (cdc.gov)
  • Their transient and rapid morphological adaptations are crucial for many cellular processes such as cell cycle, immunity, apoptosis and mitochondrial quality control. (cam.ac.uk)
  • 4 Interestingly, Cdk5 has been shown to act as a mitochondrial regulator during neuronal apoptosis. (bmj.com)
  • Cyclin-dependent kinase 5 is an upstream regulator of mitochondrial fission during neuronal apoptosis. (bmj.com)
  • These mitochondrial defects can be widely rescued by Cdk5 inhibition with roscovitine or Cdk5 knockdown with siRNA transfection. (bmj.com)
  • Utilizing a small molecule mitochondrial fission inhibitor, mitochondrial division inhibitor-1, the results so far have demonstrated that inhibition of the mitochondrial fission process significantly attenuates NIHL and reduces damage to the tissues of the inner ear resulting from a single acute sound exposure. (dtic.mil)
  • We conclude that E2 regulates mitochondrial dynamics to maintain the mitochondrial network promoting mitochondrial fusion and attenuating mitochondrial fission in both the cardiovascular and nervous systems. (ucl.ac.uk)
  • These results support the hypothesis that the mutation in the MFN2 gene altering mitochondrial bioenergetics and fusion causes axonal sensory-motor neuropathy. (fortuneonline.org)
  • We first observed that striatal cells expressing mutant huntingtin (mHtt) present increased mitochondrial fragmentation that worsens after dopaminergic stimuli. (bmj.com)
  • Mitochondrial elongation factor 2 is a protein that in humans is encoded by the MIEF2 gene. (wikipedia.org)
  • The MID49 protein is used to assist in mitochondrial binary fission. (wikipedia.org)
  • To further explore the mode of action of carvedilol, we have investigated, in silico, the ability of carvedilol to target dynamin-1-like protein and mitochondrial dynamics protein (MID51). (uky.edu)
  • Heat generation, mediated by mitochondrial respiration in Brown Adipose Tissue (BAT), is important for defense against cold and maintenance of whole-body homeostasis. (diabetesjournals.org)
  • 6/9) a clear defect in mitochondrial ATP synthesis and reduced respiration rates were observed in patients with poor vision. (omicsdi.org)
  • Previous analyses of dopaminergic (DAergic) neurons from patients with Lewy-body pathology revealed a deficiency in nuclear-encoded genes for mitochondrial respiration, many of which are targets for the transcription factor estrogen-related receptor gamma ( Esrrg /ERRγ). (nature.com)
  • Laser-captured microdissected (LCM) DAergic neurons from postmortem tissue of patients with Lewy-pathology revealed a deficiency in expression for nuclear-encoded genes involved in mitochondrial respiration and function 12 . (nature.com)
  • Live imaging of mouse RPE flatmounts revealed a striking loss of mitochondrial integrity in albino mouse RPE compared to pigmented mice that could have significant functional consequences for cellular metabolism. (figshare.com)
  • Furthermore, I will perform mouse phenotyping experiments to reveal if the loss of mitochondrial SPIRE1 function contributes to the increased fear of SPIRE1-mutant mice. (uni-regensburg.de)
  • At the same time, it significantly reduced the serum albumin level, the activity of hepatic superoxide dismutase (SOD), and the expression of mitochondrial peroxisome proliferator-activated receptor-1α (PGC-1α), and mitofusin 2 (Mtf2). (uky.edu)
  • More recently, it has become apparent that E2 also regulates mitochondrial fusion-fission dynamics, thereby affecting cellular metabolism. (ucl.ac.uk)
  • How mitochondrial shape and substrate-specific metabolism are related has been a difficult question to address. (qxmd.com)
  • Finally, understanding these muscle disorders enables a better understanding of the dynamics of muscle and body metabolism. (medscape.com)
  • In the present study, we examine the specific impacts of MSCs on mitochondrial morphology and function in host tissues. (frontiersin.org)
  • We propose studies that will expand on our initial findings to better characterize alterations in neuronal mitochondrial fission, fusion, biogenesis and mitophagy in both PD-relevant genetic and chronic environmental models, and begin to elucidate possible mechanisms. (neurodegenerationresearch.eu)
  • Here, we investigated whether this aberrant Cdk5 signalling contributes to the striatal neurodegeneration by altering mitochondrial dynamics processes. (bmj.com)
  • This research is focused on understanding the role of changes in mitochondrial homeostasis in the neurodegeneration of Parkinson's disease. (neurodegenerationresearch.eu)
  • Therefore, we propose a new vicious cycle involved in neurodegeneration that includes glutamate excitotoxicity, oxidative stress, and mitochondrial dynamics. (omicsdi.org)
  • In this review, we discuss the functional cross talk of proteostasis and mitostasis in cellular homeodynamics and the impairment of mitochondrial quality control during ageing, cancer, and neurodegeneration. (hindawi.com)
  • Employing in vitro cell culture of inherited mitochondrial disease and an in vivo animal experimental model of low-grade inflammation (high fat feeding), we show human-derived MSCs to alter mitochondrial function. (frontiersin.org)
  • This article will describe an overview of the molecular mechanisms that govern mitochondrial fission and fusion in mammals. (cam.ac.uk)
  • Mitochondrial fusion and fission have emerged as important processes that govern mitochondrial function and PGC-1α may regulate fusion/fission events. (unipv.it)
  • For this program, therapeutic approaches to Alzheimer's disease (AD) and related dementias (ADRD) should have a focus on biological mechanisms related to mitochondrial/bioenergetics and inflammation. (alz.org)
  • Identifying therapies that target mitochondrial/ bioenergetics and inflammation may have the potential to treat complex underlying biology of AD and ADRD, while also gaining a deeper understanding of multiple diseases that affect the brain. (alz.org)
  • Part the Cloud will promote human studies to advance innovative ideas for early phase human trials (Phase 1 or Phase 2) that address therapies (an experimental or repurposed drug) to address mitochondrial/ bioenergetics and inflammation. (alz.org)
  • including biologic or small molecule approaches) targeting mitochondrial/bioenergetics and related biological mechanisms (i.e., inflammation response). (alz.org)
  • At present, little is known about the impact of MSC therapy on mitochondrial function and morphology. (frontiersin.org)
  • Understanding the molecular mechanisms controlling mitochondrial dynamics is crucial to decipher how mitochondrial shape meets the function and to increase the knowledge on the molecular basis of diseases associated with morphology defects. (cam.ac.uk)
  • Accordingly, improving mitochondrial function has become an important strategy to treat the early stages of this continuum. (nih.gov)
  • Impaired fission and fusion balance can also be induced by a reduction of the mitochondrial permeability transition pore (mPTP) function as atractyloside which indicates the mPTP has similar effects on mitochondrial dynamics. (nih.gov)
  • These findings suggest the mPTP as an important target for the beneficial effects of piracetam on mitochondrial function. (nih.gov)
  • To determine whether the degree of vision loss is associated with the level of mitochondrial impairment, we examined mitochondrial function in lymphoblast cell lines obtained from six large Australian OPA1-linked ADOA pedigrees. (omicsdi.org)
  • While ERRγ deletion did not fully recapitulate the transcriptional alterations observed in postmortem tissue, it caused reductions in genes involved in synaptic and mitochondrial function and autophagy. (nature.com)
  • Further evidence suggests that during PD progression, transcriptional programs for the expression of genes involved in mitochondrial structure and function are disrupted 11 . (nature.com)
  • Several studies suggest that targeting PGC-1α to improve mitochondrial function could be a potential therapeutic strategy. (nature.com)
  • Currently, mitochondrial transplantation is an available approach for restoring mitochondrial function in mitochondrial diseases but remains unclear in breast cancer. (biomedcentral.com)
  • After 3 days of treatment, cell viability, proliferation, oxidative stress, drug sensitivity to Doxorubicin/Paclitaxel and mitochondrial function were assessed. (biomedcentral.com)
  • Mitochondrial dynamics are diverse in different cell types, and their function is determined by a delicate shift in the balance of morphological fusion and fission in cells to adapt to physiological stress. (biomedcentral.com)
  • Our studies lay a framework to guide experimental design and selection of model systems for evaluating mitochondrial health and function in the RPE. (figshare.com)
  • These findings suggest that exercise induces adaptations in mitochondrial dynamics, potentially increasing mitochondrial function, and SJL may disrupt mitochondrial dynamics both in the sedentary and exercise trained states. (wku.edu)
  • Dr. Sieck's team is exploring whether the age-related loss of phrenic motor neurons is due to altered mitochondrial function or changes in motor neuron autophagy and mitophagy. (mayo.edu)
  • Moreover, nonreversible mitochondrial damage targets the organelle to a specific autophagic removal, namely, mitophagy. (hindawi.com)
  • Markers of Parkinson (PD) and Alzheimer (AD) diseases are able to induce innate immune pathways induced by alterations in mitochondrial Ca 2+ homeostasis leading to neuroinflammation. (frontiersin.org)
  • In addition, we found early alterations in mitochondrial dynamics in neurons in a chronic neurotoxic PD-relevant model, and we hypothesize that these changes are involved in early neuropathology in PD, and thus, are potential new targets for neuroprotective therapies. (neurodegenerationresearch.eu)
  • We have established an influence of both compromised genomic maintenance (nuclear and mitochondrial), and alterations in external growth control to these processes, which also have direct relevance for the stability and potential reversibility of the aging stem cell state. (lu.se)
  • Mitochondrial dynamics are particularly critical to neurons, and the vulnerable neurons in PD may be especially dependent on these processes. (neurodegenerationresearch.eu)
  • Both genetic and environmental toxin-related models of PD have been linked to dysregulation of mitochondrial dynamics, but less is known about these processes in neurons. (neurodegenerationresearch.eu)
  • We demonstrate that deletion of ERRγ from DAergic neurons in adult mice was sufficient to cause a levodopa-responsive PD-like phenotype with reductions in mitochondrial gene expression and number, that partial deficiency of ERRγ hastens synuclein-mediated toxicity, and that ERRγ overexpression reduces inclusion load and delays synuclein-mediated cell loss. (nature.com)
  • Disruptions in mitochondrial dynamics may contribute to the selective degeneration of dopamine (DA) neurons in Parkinson's disease (PD). (jneurosci.org)
  • However, little is known about the normal functions of mitochondrial dynamics in these neurons, especially in axons where degeneration begins, and this makes it difficult to understand the disease process. (jneurosci.org)
  • Mitochondrial dynamics: overview of molecular mechanisms. (cam.ac.uk)
  • The effect of SFN on angiogenesis and the underlying mechanisms including the roles of Nrf2 and mitochondrial dynamics were investigated using cultured human umbilical vein endothelial cells (HUVECs) in hypoxia. (uea.ac.uk)
  • These findings suggest that SFN biphasically affected the angiogenic capacity of hypoxia challenged HUVECs potentially via mechanisms involving an integrated modulation of Nrf2 and mitochondrial dynamics. (uea.ac.uk)
  • While these studies provide valuable insight at fixed points in time, high-resolution, high-speed live imaging is essential for following mitochondrial injury in real time and identifying disease mechanisms. (figshare.com)
  • In vivo experiments confirmed mitochondrial abundance and mitochondrial oxygen consumption rates were elevated in host tissues following MSC treatment. (frontiersin.org)
  • While cancers evolve during disease progression and in response to therapy, temporal dynamics remain difficult to study in humans due to the lack of consistent barcodes marking individual clones in vivo. (nih.gov)
  • While in vitro studies have greatly expanded our knowledge of mitochondrial dynamics, our understanding in vivo remains limited. (uni-muenchen.de)
  • To address this shortcoming, we developed tools to study mitochondrial dynamics in vivo in optically accessible zebrafish. (uni-muenchen.de)
  • Michael Gurnis investigates local, regional, and global geological and geophysical activity to determine the processes related to plate tectonics and deep mantle dynamics. (caltech.edu)
  • A sub line of our research deals with how proliferation dynamics, at defined cellular stages, associate with these processes. (lu.se)
  • These data suggest that OPA1 deficiency impairs OXPHOS efficiency, but compensation through increases in the distal complexes of the respiratory chain may preserve mitochondrial ATP production in patients who maintain normal vision. (omicsdi.org)
  • To address this issue, we used a mouse model that enabled direct measurement of cytosolic and mitochondrial ATP levels. (nature.com)
  • Empagliflozin treatment significantly increased cytosolic and mitochondrial ATP levels in the hearts of db/db mice. (nature.com)
  • An interconnected highly integrated system of mitochondrial and cytosolic chaperones and proteases along with the fission/fusion machinery represents the surveillance scaffold of mitostasis. (hindawi.com)
  • Collectively, our data indicate that MSC therapy rescues impaired mitochondrial morphology, enhances host metabolic capacity, and induces widespread host gene shifting. (frontiersin.org)
  • Mitochondrial oxidative phosphorylation compensation may preserve vision in patients with OPA1-linked autosomal dominant optic atrophy. (omicsdi.org)
  • Mitochondrial oxidative phosphorylation in autosomal dominant optic atrophy. (omicsdi.org)
  • Disturbances in mitochondrial dynamics may influence many cellular and molecular pathways, as calcium-dependent immune activation, transcription factors phosphorylation, cytokine secretion, organelle transference and even cell death. (frontiersin.org)
  • The role of mitochondrial dynamics in cancer development and progression is still complex and elusive because cancer cells are distinct from normal cells in terms of their ability to survive and proliferate despite their exposure to microenvironmental apoptotic stimuli, such as hypoxia, oxidative stress, nutrient deprivation and inflammation [ 1 ]. (biomedcentral.com)
  • However, no studies to date have evaluated the effects of long term SJL on cardiac mitochondrial dynamics. (wku.edu)
  • in fact, these genes are targets of the transcriptional coactivator and master regulator of transcription of mitochondrial genes peroxisome proliferator-activated receptor gamma coactivator-1 alpha (PGC-1α) 12 . (nature.com)
  • Recent research has revealed important correlation of mitochondrial dynamics and the pathophysiology of brain diseases, as Alzheimer's. (frontiersin.org)
  • MSC co-culture with skin fibroblasts from mitochondrial disease patients rescued aberrant mitochondrial morphology from a fission state to a more fused appearance indicating an effect of MSC co-culture on host cell mitochondrial network formation. (frontiersin.org)
  • We are fascinated by mitochondrial fission, fusion, growth, transport, and network topology, and how they contribute to energy production and mitochondrial genome maintenance. (epfl.ch)
  • Lastly, Sel1L affects mitochondrial biology as a part of the Hrd1 ERAD complex, as adipocyte-specific Hrd1-deficient mouse, phenocopy Sel1L-deficient mice in terms of mitochondrial phenotype. (diabetesjournals.org)
  • Opa1(enu/+) mice show a slow progressive loss of RGCs, activation of astroglia and microglia, and pronounced mitochondrial fission in optic nerve heads as found by electron tomography. (omicsdi.org)
  • By quantifying the dynamic geometry of mitochondrial constrictions, we extract the energies and forces required for division and link them to interactions with the division machinery. (epfl.ch)
  • Our results conclusively demonstrate that not only glutamate excitotoxicity and/or oxidative stress alters mitochondrial fission/fusion, but that an imbalance in mitochondrial fission/fusion in turn leads to NMDA receptor upregulation and oxidative stress. (omicsdi.org)
  • Hence, our data provide novel molecular insights and platform for future studies into ER-mitochondrial interaction and position Sel1L-Hrd1 ERAD at the center of organelle crosstalk. (diabetesjournals.org)
  • Furthermore, we are investigating the metabolic and bioenergetic changes associated with mitochondrial division on a single-organelle level. (epfl.ch)
  • Direct regulators of nuclear-encoded mitochondrial genes are members of the estrogen-related receptor (ERR) family, encoded by Esrra , Esrrb and Esrrg . (nature.com)