Mitochondrial and cellular. Medical search. Frequent questions

Mitochondrial dysfunction inhibits this process and allows ATFS-1 to accumulate in the cytosol and enter the nucleus where it can act as a transcription factor. (wikipedia.org)
The MTS on ATFS-1 is predicted by Mitofates to be substantially weaker than most MTSs which would allow it to be sensitive to subtle mitochondrial dysfunction. (wikipedia.org)
Mitochondrial dysfunction has also been associated with ageing and most of the so-called age-related diseases [ 13 - 17 ]. (hindawi.com)
Mitochondrial diseases include Leigh syndrome, a progressive and fatal disorder characterized by lesions on the brain that may lead to heart, kidney, vision and breathing complications, and Alpers Disease, a neurologic illness that causes seizures, dementia, spasticity, blindness, liver dysfunction and cerebral degeneration. (sciencedaily.com)
Mitochondrial dysfunction has been proven to play a critical role in the pathogenesis of cardiovascular diseases. (frontiersin.org)
Importantly, it has been suggested that mitochondrial dysfunction might precede the metabolic change. (intechopen.com)
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)
Mitochondrial dysfunction has been implicated in the toxicities of certain diseases and many drugs. (fda.gov)
Both α-Synuclein (αSyn) accumulation and mitochondrial dysfunction have been implicated in the pathology of Parkinson's disease (PD). (springer.com)
Although the mechanisms underlying PD remain elusive, α-Synuclein (αSyn) accumulation and mitochondrial dysfunction have been recognized as major contributors [ 1 , 57 ]. (springer.com)
Mitochondrial dysfunction is one of the major intracellular lesions of Alzheimer's disease (AD). (jneurosci.org)
However, the causative factors involved in the mitochondrial dysfunction in human AD are not well understood. (jneurosci.org)
The levels of translocationally arrested mitochondrial APP directly correlated with mitochondrial dysfunction. (jneurosci.org)
Collectively, these results suggest that abnormal accumulation of APP across mitochondrial import channels, causing mitochondrial dysfunction, is a hallmark of human AD pathology. (jneurosci.org)
Progressive mitochondrial accumulation of incompletely translocated APP caused mitochondrial dysfunction and impaired cellular energy metabolism. (jneurosci.org)
Insulin resistance occurs in almost all patients with non-alcoholic fatty liver disease (NAFLD), and mitochondrial dysfunction likely plays a pivotal role in the progression of fatty liver into non-alcoholic steatohepatitis (NASH). (spandidos-publications.com)
Together, these data suggest that curcumin protects hepatocytes from HFFA-induced lipoapoptosis and mitochondrial dysfunction, which partially occurs through the regulation of mitochondrial biogenesis. (spandidos-publications.com)
There is also growing evidence that FFA-mediated oxidative stress contributes significantly to mitochondrial dysfunction in the liver. (spandidos-publications.com)
Therefore, designing therapies that prevent mitochondrial dysfunction stands to be one of the most important strategies in for treating of NAFLD and its complications. (spandidos-publications.com)
Mitochondrial dysfunction is the second process that leads to HSPs. (medscape.com)

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)
Since mitochondrial stress represents a key trigger of cellular senescence, and senescence leads to the accumulation of abnormal mitochondria with impaired dynamics and hindered homeostasis, this review focuses on the beneficial potential of targeting mitochondria, so that strategies can be developed to manage adipose tissue senescence for the treatment of age-related metabolic disorders. (unisannio.it)
The mitochondrial unfolded protein response (UPRmt) is a cellular stress response related to the mitochondria. (wikipedia.org)
ATFS-1 has a nuclear localization sequence that allows it to be imported into the nucleus as well as an N-terminal mitochondrial targeting sequence (MTS) that allows for import into the mitochondria. (wikipedia.org)
In the nucleus, ATFS-1 has a broad transcriptional regulation as it will: attenuate OXPHOS gene expression in both the nucleus and mitochondria, upregulate chaperones and proteases to re-establish mitochondrial proteostasis, increase ROS detoxification, and increase mitochondrial import machinery. (wikipedia.org)
Our PQQ (pyrroloquinoline quinone) supplement is a powerful antioxidant that supports the growth of brand-new mitochondria, to support cellular energy. (pennherb.com)
PQQ (pyrroloquinoline quinone) can support the growth of brand-new mitochondria, which encourages healthy and youthful cellular energy for your entire body. (pennherb.com)
Therefore, maintenance of mitochondria activity is necessary for the proper cellular function and survival. (hindawi.com)
Moreover, mitochondria control cell death through apoptosis and supply Ca 2+ and metabolites required for cellular homeodynamics [ 3 ]. (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)
In addition, the subcellular distribution of mitochondria can affect the cellular transcriptome and transcription rates. (hindawi.com)
The maintenance of "healthy" and fully functional mitochondria is thus essential for cellular homeodynamics. (hindawi.com)
In this review, we summarized the mechanism of mitochondrial transfer in the cardiovascular system and outlined the fate and functional role of donor mitochondria. (frontiersin.org)
Given the complexity of the biological function of mitochondria, researchers have begun to consider rescuing the injured cells through mitochondrial transfer, that is, replacing damaged mitochondria with healthy mitochondria from donor cells. (frontiersin.org)
With the synergy of ubiquinol and PQQ, this supplement supports efficient cellular energy function, helps the creation of new mitochondria, and supports healthy cardiovascular function. (pricepulse.app)
CoQ10 is a component of the energy-producing mitochondria in our cells, playing a critical role in generating ATP (adenosine triphosphate), the body's primary source of cellular energy. (pricepulse.app)
By supporting the creation of new mitochondria, PQQ helps enhance cellular energy production. (pricepulse.app)
[5] The eukaryote Monocercomonoides is known to have completely lost its mitochondria, [6] and the multicellular organism Henneguya salminicola is known to have retained mitochondrion-related organelles in association with a complete loss of their mitochondrial genome. (wikipedia.org)
In addition to supplying cellular energy, mitochondria are involved in other tasks, such as signaling , cellular differentiation , and cell death , as well as maintaining control of the cell cycle and cell growth . (wikipedia.org)
[20] This finding has led to general acceptance of the endosymbiotic hypothesis - that free-living prokaryotic ancestors of modern mitochondria permanently fused with eukaryotic cells in the distant past, evolving such that modern animals, plants, fungi, and other eukaryotes are able to respire to generate cellular energy . (wikipedia.org)
At higher concentrations, ROS induces cell injury and death, which occurs during the aging process, where accelerated generation of ROS and a gradual decline in cellular antioxidant defense mechanisms, especially in the mitochondria. (nih.gov)
They target the factors that damage mitochondria and reverse its effect, thus eliminating the imbalance seen in energy production and restore the normal cellular function, making these antioxidants very powerful alternate strategies for the treatment of cardiovascular cerebrovascular as well as neurodegenerative diseases including AD. (nih.gov)
Mitochondria-related genes are regions on DNA (deoxyribonucleic acid), which encode proteins that are important for mitochondrial structure and function. (fda.gov)
One of the unique features of mitochondria is that they contain their own small, circular DNA (deoxyribonucleic acid) and independent machinery of ribosomal and transfer RNAs (ribonucleic acid) and ribosomes for synthesis of 13 proteins encoded by the mitochondrial DNA essential for oxidative phosphorylation. (fda.gov)
It was therefore assumed that cancer cells were generating energy using glycolysis rather than mitochondrial oxidative phosphorylation, and that the mitochondria were dysfunctional. (researchgate.net)
The generators in the cellular power plants are biological membranes located inside the mitochondria. (sciencedaily.com)
Mitochondria are the organelles providing most of the cellular energy in form of adenosine triphosphate (ATP) in aerobic eukaryotes. (elifesciences.org)
Although studies suggest that αSyn and its missense mutant, A53T, preferentially accumulate in the mitochondria, the mechanisms by which αSyn and mitochondrial proteins regulate each other to trigger mitochondrial and neuronal toxicity are poorly understood. (springer.com)
Despite these findings suggesting that mitochondria might be a key link between αSyn toxicity and neuronal degeneration in PD, the field still lacks an understanding of how αSyn abnormality and mitochondrial functional deficiency influence each other. (springer.com)
Furthermore, in AD brains, mitochondrially associated APP formed stable ∼480 kDa complexes with the translocase of the outer mitochondrial membrane 40 (TOM40) import channel and a super complex of ∼620 kDa with both mitochondrial TOM40 and the translocase of the inner mitochondrial membrane 23 (TIM23) import channel TIM23 in an "N in mitochondria -C out cytoplasm " orientation. (jneurosci.org)

Moreover, this induced mitochondrial stress signaling, which enables the recovery of mitochondrial translation via mitochondrial biogenesis, telomerase expression, and cell proliferation, and thereby normalizes metabolism. (edu.au)
[10] Mitochondrial biogenesis is in turn temporally coordinated with these cellular processes. (wikipedia.org)
Not only did curcumin treatment increase mitochondrial DNA (mtDNA) copy number in hepatocytes, but it also increased levels of transcriptional factors that regulate mitochondrial biogenesis, including peroxisome proliferator-activated receptor-γ coactivator 1α (PGC1α), nuclear respiratory factor 1 (NRF1) and mitochondrial transcription factor A (Tfam). (spandidos-publications.com)
In addition, curcumin contributed to cell survival, as indicated by the restoration of the mitochondrial membrane potential (MMP) and the inhibition of the mitochondrial biogenesis induced by HFFA. (spandidos-publications.com)

However, an accumulation of low molecular weight subcomplexes suggested a complex I assembly/stability defect, which was further demonstrated by reversibly inhibiting mitochondrial protein translation with doxycycline. (nih.gov)

Predicted to be integral component of mitochondrial inner membrane. (nih.gov)

In recent years, a large number of pharmaceutical compounds and nutritional supplements that can boost mitochondrial bioenergetics efficiency have been developed. (frontiersin.org)
This manuscript will be of broad interest to the field of mitochondrial bioenergetics. (elifesciences.org)
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)
ATP-dependent Clp protease (ClpP), a mitochondrial matrix protease, plays an important role in regulating mitochondrial protein turnover and bioenergetics activity. (springer.com)

Analytical solutions for the model point out the cellular- and kinetic aspects of inhibition. (plos.org)
von Kleist M, Metzner P, Marquet R, Schütte C (2012) HIV-1 Polymerase Inhibition by Nucleoside Analogs: Cellular- and Kinetic Parameters of Efficacy, Susceptibility and Resistance Selection. (plos.org)

The moratorium deprives affected American families of the opportunity to prevent inherited, incurable and agonizing mitochondrial disease in their children, the authors contend. (sciencedaily.com)

Following mitochondrial stress, ATFS-1 mitochondrial import efficiency is decreased resulting in a cytoplasmic accumulation of ATFS-1. (wikipedia.org)
growth on ethananol - Extracellular pH affects the accumulation of mitochondrial cytochrome c550. (bio.net)
Overexpression of ClpP reduces αSyn-induced mitochondrial oxidative stress through enhancing the level of Superoxide Dismutase-2 (SOD2), and suppresses the accumulation of αSyn S129 phosphorylation and promotes neuronal morphology in neurons derived from PD patient iPS cells carrying αSyn A53T mutant. (springer.com)
Accumulation of APP across mitochondrial import channels, which varied with the severity of AD, inhibited the entry of nuclear-encoded cytochrome c oxidase subunits IV and Vb proteins, which was associated with decreased cytochrome c oxidase activity and increased levels of H 2 O 2 . (jneurosci.org)
Mitochondrial accumulation of APP was also observed in the cholinergic, dopaminergic, GABAergic, and glutamatergic neuronal types in the category III AD brains. (jneurosci.org)
Hepatocellular lipid accumulation is thought to simultaneously stimulate mitochondrial fatty acid oxidation and the production of ROS, thereby promoting lipid peroxidation and damage of mitochondrial DNA (mtDNA) ( 3 ). (spandidos-publications.com)

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)
Nevertheless, the mechanisms related to the impairment of mitochondrial functions specific to AD remain unclear. (jneurosci.org)

Adashi and Cohen recommend that a coalition of patient and advocacy groups, medical professionals and legislators convene congressional hearings on the prevention of mitochondrial diseases. (sciencedaily.com)
Because there are FDA-regulated drugs (e.g., anti-HIV drugs, anti-cancer drugs) that cause mitochondrial injury, it is important to understand the mechanism of mitochondrial injury and to develop measures for prevention of mitochondrial damage. (fda.gov)

Consequently, specific cellular mechanisms exist that aim to detect cellular stress (causing misfolded/unfolded proteins to accumulate), transduce the signal to the nucleus, and mediate the restoration of protein homeostasis (proteostasis). (wikipedia.org)
Identification of proteins interacting with the mitochondrial small heat shock protein Hsp22 of Drosophila melanogaster: Implication in mitochondrial homeostasis. (nih.gov)
Studies have shown that cell-to-cell mitochondrial transfer plays an essential role in regulating cardiovascular system development and maintaining normal tissue homeostasis under physiological conditions. (frontiersin.org)
In addition to the prime function of energy production, these cellular organelles are involved in fatty acid β-oxidation, the citric acid cycle (TCA cycle), heme and iron-sulfur cluster assembly, amino acids, pyrimidine, and steroid synthesis, and calcium and iron homeostasis. (fda.gov)

The UPRmt can occur either in the mitochondrial matrix or in the mitochondrial inner membrane. (wikipedia.org)
Predicted to be part of TIM23 mitochondrial import inner membrane translocase complex. (nih.gov)
Genetic and structural characterization of the human mitochondrial inner membrane translocase. (nih.gov)
The plasma membrane is an important cellular organelle that is often overlooked in terms of a primary factor in regulating physiology and pathophysiology. (silverchair.com)
The hMiros represent two highly atypical members of the Ras superfamily, and exhibit several unique features: the presence of a GTPase domain at both the N-terminus and C-terminus, the presence of two calcium-binding EF-hand domains and localisation to the mitochondrial outer membrane. (northumbria.ac.uk)
Even minute errors in the composition of the inner mitochondrial membrane can lead to severe metabolic derangements, which can have an especially negative impact on the energy-hungry muscle and nerve cells. (sciencedaily.com)
In order to function, the cellular generators depend on the support of numerous highly specialized membrane proteins in the inner mitochondrial membrane. (sciencedaily.com)
Thus, the scientists were able to demonstrate for the first time that at least two different protein translocases cooperate closely to insert proteins with complex structures into the inner mitochondrial membrane. (sciencedaily.com)
In clarifying this coupled mechanism of membrane insertion, project head Dr. Martin van der Laan and his team have solved a hotly debated scientific problem and made a major contribution to our understanding of the composition and functioning of cellular power plants. (sciencedaily.com)
Mitochondrial membrane protein-associated neurodegeneration (MPAN) is a disorder of the nervous system. (medlineplus.gov)
Dogu O, Krebs CE, Kaleagasi H, Demirtas Z, Oksuz N, Walker RH, Paisan-Ruiz C. Rapid disease progression in adult-onset mitochondrial membrane protein-associated neurodegeneration. (medlineplus.gov)
Hartig M, Prokisch H, Meitinger T, Klopstock T. Mitochondrial membrane protein-associated neurodegeneration (MPAN). (medlineplus.gov)
The molecular machinery responsible for energy transformation is the oxidative phosphorylation (OXPHOS) system, which is canonically composed of five multiprotein complexes embedded in the inner mitochondrial membrane. (elifesciences.org)
Prohibitin 1 and prohibitin 2 form a ring-shaped complex in the INNER MITOCHONDRIAL MEMBRANE. (bvsalud.org)
Impaired cellular membrane trafficking, more particularly, axonal transport of macromolecules and organelles, is the best-characterized genetic mechanism of HSP. (medscape.com)
It is part of the m-AAA protease, an adenosine triphosphate (ATP) ̶ dependent proteolytic complex located at the mitochondrial inner membrane, which controls protein quality and regulates ribosome assembly. (medscape.com)

Super Ubiquinol CoQ10 with Enhanced Mitochondrial Support™ is formulated with a highly bioavailable form of coenzyme Q10 called ubiquinol and augmented with the adaptogen PrimaVie® shilajit, which has been shown in preclinical models to promote mitochondrial energy production. (lifeextension.com)

In mammals, this effect is compensated for by increased mitochondrial protein stability and upregulation of the citric acid cycle. (edu.au)
Conversely, we show that increased fidelity of mitochondrial translation reduces the rate of protein synthesis without eliciting a mitochondrial stress response. (edu.au)
In summary, our findings reveal mammalian-specific signaling pathways that respond to changes in the fidelity of mitochondrial protein synthesis and affect metabolism. (edu.au)
The ER UPR is induced by a variety of cellular stressors that inhibit protein folding or exit of the ER. (wikipedia.org)
Using this approach, several components of the mammalian UPRmt have been identified including the mitochondrial chaperone heat shock protein 60 (Hsp60), the mitochondrial caseinolytic peptidase ClpP, the transcription factor Chop and the kinases c-Jun N-terminal kinase (JNK) and the interferon-induced, double-stranded RNA-activated protein kinase (Pkr). (wikipedia.org)
The AAA-ATPase (ATPases associated with diverse cellular activities) valosin-containing protein (VCP), is essential for many cellular pathways including but not limited to endoplasmic reticulum-associated degradation (ERAD), DNA damage responses, and cell cycle regulation. (portlandpress.com)
Predicted to be involved in protein import into mitochondrial matrix. (nih.gov)
Tim50 is a subunit of the TIM23 complex that links protein translocation across the outer and inner mitochondrial membranes. (nih.gov)
Analyzing 5′-upstream non-protein-encoding regions of the human mitochondrial function-associated genes, we speculate that mitochondrial functions could be recovered or improved at a transcriptional level. (intechopen.com)

The rest of approximately 1,900 proteins (in humans) required for the performance of various mitochondrial functions are encoded by the nuclear DNA. (fda.gov)
This group includes lysosomal storage disorders, various mitochondrial diseases, other neurometabolic disorders, and several other miscellaneous disorders. (medscape.com)

This responses restores proteostasis by upregulating chaperones and proteases, increasing reactive oxygen species (ROS) detoxification, and increasing mitochondrial import machinery. (wikipedia.org)
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)

In healthy cells, ATFS-1 is preferentially targeted to the mitochondrial matrix where it is degraded by the Lon protease. (wikipedia.org)
Treatment with the purified protease increased cellular and mitochondrial reactive oxygen species (ROS) generation. (cdc.gov)

Oxygen-containing charged particles are common byproducts of cellular metabolism. (mda.org)

Leber's hereditary optic neuropathy (LHON), the most frequent mitochondrial disorder, is mostly due to three mitochondrial DNA (mtDNA) mutations in respiratory chain complex I subunit genes: 3460/ND1, 11778/ND4 and 14484/ND6. (nih.gov)
We propose that hypometabolism, coupled with oxidative stressors, is responsible for most Alzheimer disease (AD) and cerebrovascular accidents (CVAs) and appears to be a central initiating factor for vascular abnormalities, mitochondrial damage and an imbalance in the activity of vasoactive substances, such as different isoforms of nitric oxide synthase (NOS), endothelin-1 (ET-1), oxidative stress markers, mtDNA and mitochondrial enzymes in the vascular wall and in brain parenchymal cells. (nih.gov)

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)

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)

Publication from the Danial and Shirihai Labs reported that fatty acid utilization is selectively coupled to mitochondrial morphology. (harvard.edu)
In the present study, we examine the specific impacts of MSCs on mitochondrial morphology and function in host tissues. (frontiersin.org)
Collectively, our data indicate that MSC therapy rescues impaired mitochondrial morphology, enhances host metabolic capacity, and induces widespread host gene shifting. (frontiersin.org)
At present, little is known about the impact of MSC therapy on mitochondrial function and morphology. (frontiersin.org)

Key role in aerobic respiration, in which mitochondrial enzymes accept electrons from electron carriers reduced in glycolysis and the tricarboxylic acid cycle. (yeastgenome.org)
that affects cellular respiration. (msdmanuals.com)
We are currently studying white fat browning at the cellular level, by measuring mitochondrial respiration and aerobic enzyme activity, in Japanese quail. (lu.se)

Thus, as an example (in addition to neuronal targets or other cellular types), there are opportunities to target cellular mechanisms of astrocytes, oligodendrocytes, microglia and even endothelial cells on blood vessels in order to halt, diminish and/or reverse the brain cell degeneration seen in AD or ADRD. (alz.org)

Due to its abundance and versatile function, VCP participates in many cellular pathways including ERAD, endolysosomal trafficking, selective autophagy, cell cycle regulation, and DNA damage signaling [ 1 ]. (portlandpress.com)
Furthermore, microarray profiling identified 226 genes with differential expression in the liver of animals treated with MSC, with cellular signaling, and actin cytoskeleton regulation as key upregulated processes. (frontiersin.org)

A majority of cellular proteins are translated and folded in the cytosol with the help of molecular chaperones. (wikipedia.org)
Cells express a pool of thousands of different proteins that need to be tightly controlled for proper cellular structure, organization, and function. (hindawi.com)
Deficiency in ClpP induces an overload of mitochondrial misfolded/unfolded proteins, suppresses mitochondrial respiratory activity, increases mitochondrial oxidative damage and causes cell death. (springer.com)
Cellular component GO terms and their p-values for co-expressed proteins. (lu.se)

During electron transport, complexes I, III, and IV pump protons from the mitochondrial matrix to the intermembrane space, generating a proton gradient that provides the protonmotive force exploited by complex V to synthesize ATP. (elifesciences.org)

But a much-studied form of mitochondrial replacement (MR) could prevent the transmission of such diseases from mothers to children, researchers say. (sciencedaily.com)
We hope this review will provide perspectives on mitochondrial-targeted therapeutics in cardiovascular diseases. (frontiersin.org)
Therefore, patients with cardiovascular diseases would fail to achieve the desired outcomes by using these mitochondrial-targeted drugs ( 5 ). (frontiersin.org)
Therefore, mitochondrial DNA abnormalities such as overproliferation and or deletion can be used as a key marker for diseases differentiation and effectiveness of the treatment. (nih.gov)

Mitochondrial electron transport chain mutations that extend the life span of Caenorhabditis elegans (nematode worms) also activate the UPRmt. (wikipedia.org)
Mothers with mitochondrial DNA mutations often give birth to children who face incurable and fatal illnesses. (sciencedaily.com)

Tim50, a component of the mitochondrial translocator, regulates mitochondrial integrity and cell death. (nih.gov)

The binding of αSyn to ClpP further promotes a distribution of ClpP from soluble to insoluble cellular fraction in vitro and in vivo, leading to reduced solubility of ClpP. (springer.com)

This phenomenon was not accompanied by an increase in mitochondrial respiratory activity. (elifesciences.org)

On this page you will find links to comprehensive gene information for five species and a listing of NCTR publications on understanding the mechanism of mitochondrial toxicity and development of biomarkers to predict toxicity. (fda.gov)
NCTR has published several papers illustrating the mechanism of mitochondrial toxicity in response to different drugs in the mouse and rat. (fda.gov)
While mitochondrial energy is vital for the survival of cells, these organelles also play an important role in the execution of apoptosis (programmed cell death), a process that serves as a major defense mechanism to remove unwanted and potentially dangerous cells. (fda.gov)
The aim of this study was to analyze the ability of curcumin to protect against the mitochondrial impairment induced by high free fatty acids (HFFAs) and to determine the underlying mechanism for this cytoprotection. (spandidos-publications.com)

In mammals, UPRmt has mostly been studied using transfection with a truncated, dysfunctional mitochondrial enzyme (OTCΔ) that does not fold correctly after translocation into the mitochondrial matrix. (wikipedia.org)
Interestingly, mitochondrial translocation of APP was incomplete because of an internal acidic domain present between 220 and 290 amino acids. (jneurosci.org)

Cellular Physiology and Biochemistry (2012) 29 (3-4): 583-594. (karger.com)
Carotenogenesis in the green alga Haematococcus pluvialis: Cellular physiology and stress response. (bio.net)
Cellular Physiology and Biochemistry (2018) 49 (5): 1943-1958. (karger.com)

The human Miro GTPases (hMiros) have recently emerged as important mediators of mitochondrial transport and may significantly contribute to the development of disorders such as Alzheimer's and schizophrenia. (northumbria.ac.uk)

Molecular and Cellular Biochemistry , 451 (1-2), 21-35. (northumbria.ac.uk)

Compensating for the loss of ClpP in the substantia nigra of αSyn A53T mice by viral expression of ClpP suppresses mitochondrial oxidative damage, and reduces αSyn pathology and behavioral deficits of mice. (springer.com)

Mitochondrial respiratory chain organization does not seem to be conserved in all organisms. (elifesciences.org)

The combination of ubiquinol and PQQ in QH-Absorb + PQQ offers a comprehensive approach to supporting mitochondrial health. (pricepulse.app)
This ubiquinol form of CoQ10 is 8x more bioavailable than standard ubiquinone varieties, and we've combined it with the adaptogen shilajit to augment CoQ10's cellular energy benefits. (lifeextension.com)

Gene Ontology (GO) terms that describe the function of a complex, the biological process in which it participates, or its cellular location. (yeastgenome.org)
MitoChip is a tool to evaluate the change in mitochondrial gene expression in response to various agents including drugs. (fda.gov)

Cellular senescence modifies many functional aspects of adipose tissue, leading to metabolic alterations through defective adipogenesis, inflammation, and aberrant adipocytokine production, and in turn, it triggers systemic inflammation and senescence, as well as insulin resistance in metabolically active tissues, leading to premature declined physiological features. (unisannio.it)

If a chemical compound targeting one of these hubs is used, it can not only modify the anticipated biological pathways but also change other unexpected mitochondrial processes ( 5 ). (frontiersin.org)

We generated yeast and mouse models with error-prone and hyper-accurate mitochondrial translation, and found that translation rate is more important than translational accuracy for cell function in mammals. (edu.au)
This potent antioxidant not only supports mitochondrial function but also helps protect against oxidative stress, a key factor in aging. (pricepulse.app)
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)
In this work, we present a novel sensitivity analysis method, called molecular density function perturbation (MDFP), for the dynamical analysis of cellular heterogeneity. (mdpi.com)
The proposed analysis is based on introducing perturbations to the density or distribution function of the cellular state variables at specific time points, and quantifying how such perturbations affect the state distribution at later time points. (mdpi.com)
This NCTR MitoChip webpage is created to provide comprehensive knowledge and resources on mitochondrial function, mitochondrial injury, and mitchondrial toxicity, while also reporting progress on NCTR's contributions to mitochondrial research. (fda.gov)
Substantial evidence has shown that αSyn toxicity may directly disrupt mitochondrial function. (springer.com)

CELL PROLIFERATION, regulations of various immune and mitochondrial functions, and cellular stress responses. (bvsalud.org)

Dysfunction22

Mitochondria30

Biogenesis4

Inhibiting mitochondrial protein1

Integral component of mitochondrial inner me1

Bioenergetics7

Inhibition2

Agonizing mitochondrial disease1

Accumulation6

Impairment of mitochondrial2

Prevention of mitochondrial2

Homeostasis4

Membrane16

Promote mitochondrial1

Protein9

Various mitochondrial2

Chaperones and proteases2

Protease2

Metabolism1

MtDNA2

Cytosolic2

Fission1

Morphology4

Respiration3

Mechanisms1

Regulation2

Proteins4

Intermembrane space1

Diseases4

Mutations2

Regulates1

Promotes1

Respiratory activity1

Mechanism4

Translocation2

Physiology3

Significantly1

Biochemistry1

ClpP1

Organization1

Ubiquinol2

Gene2

Senescence1

Processes1

Function7

Cell proliferation1