Mitochondrial protein. Medical search. Frequent questions

Mitochondrial trifunctional protein (MTP) is a protein attached to the inner mitochondrial membrane which catalyzes three out of the four steps in beta oxidation. (wikipedia.org)
This is especially interesting, because up until then it was unknown exactly how MTP was associated with the inner mitochondrial membrane, and this discovery may provide the explanation. (wikipedia.org)
Using a novel drug analog photo-catalyzable affinity probe and mass spectrometry-based proteomics, MSDC scientists identified two phylogenetically-conserved proteins in the inner mitochondrial membrane, meaning these proteins are present in, and play an important role in the development of, organisms from yeast and fruit flies to humans. (news-medical.net)
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)
We will illustrate this approach by using the urea cycle enzymes that localize in the mitochondrial matrix, and form clusters at the inner mitochondrial membrane . (bvsalud.org)

Several hundred different proteins are imported from the cytosol into the mitochondria. (nih.gov)
To generate this inventory, we performed mass spectrometry of mitochondria isolated from fourteen tissues, assessed protein localization through large-scale GFP tagging/microscopy, and integrated these results with six other genome-scale datasets of mitochondrial localization, using a Bayesian approach. (broadinstitute.org)
Proteins encoded by the mitochondrial genome or proteins encoded by the nuclear genome that are imported to and resident in the MITOCHONDRIA. (harvard.edu)
Thus, although Txnip deletion suppresses mitochondrial function, protection from myocardial ischemia is enhanced as a result of a coordinated shift to enhanced anaerobic metabolism, which provides an energy source outside of mitochondria. (jci.org)
Here we report that nonglycosylated full-length and C-terminal truncated amyloid precursor protein (APP) accumulates exclusively in the protein import channels of mitochondria of human AD brains but not in age-matched controls. (jneurosci.org)
Eukaryotes contain two protein translational systems, one in the cytoplasm and one in the mitochondria. (nih.gov)
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)
Here, we show that formation of mitochondrial diacylglycerol (DAG) and its binding to PKD1 is the means by which PKD1 is localized to the mitochondria in response to ROS. (biologists.com)
Recent studies suggest that the mitochondria contain fidelity proteins and MnSOD constitutes an integral part of the nucleoid complex. (duke.edu)
Mitochondrial energetic adaptations encompass a plethora of conserved processes that maintain cell and organismal fitness and survival in the changing environment by adjusting the respiratory capacity of mitochondria. (nature.com)
In mammalian mitochondria, post-transcriptional regulation of gene expression is conducted by mitochondrial RBPs (mt-RBPs) at various stages of mt-RNA metabolism, including polycistronic transcript production, its processing into individual transcripts, mt-RNA modifications, stability, translation and degradation. (mpg.de)
When unfolded/misfolded proteins accumulate in mitochondria, the mitochondria release signals that regulate the transcription of certain proteins in the nucleus, thereby inducing the correct folding or degradation of proteins in mitochondria. (currentmedicinalchemistry.com)
Mitochondria could be targeted in the development of novel antidepressant drugs, and specific forms of mitochondrial dysfunction could be identified as biomarkers to personalize treatment and aid in early diagnosis by differentiating between disorders with overlapping symptoms. (frontiersin.org)
This chapter describes a combination of approaches to study clustered proteins including co-immunoprecipitation , biochemical co- localization in purified mitochondria , and super resolution imaging of endogenous proteins in situ. (bvsalud.org)

Mitochondrial trifunctional protein deficiency is a rare condition that prevents the body from converting certain fats to energy, particularly during periods without food (fasting). (medlineplus.gov)
Signs and symptoms of mitochondrial trifunctional protein deficiency may begin during infancy or later in life. (medlineplus.gov)
Signs and symptoms of mitochondrial trifunctional protein deficiency that may begin after infancy include hypotonia, muscle pain, a breakdown of muscle tissue, and a loss of sensation in the extremities (peripheral neuropathy). (medlineplus.gov)
Problems related to mitochondrial trifunctional protein deficiency can be triggered by periods of fasting or by illnesses such as viral infections. (medlineplus.gov)
Mutations in the HADHA and HADHB genes cause mitochondrial trifunctional protein deficiency. (medlineplus.gov)
Mutations in the HADHA or HADHB genes that cause mitochondrial trifunctional protein deficiency disrupt all three functions of this enzyme complex. (medlineplus.gov)
This abnormal buildup causes the other signs and symptoms of mitochondrial trifunctional protein deficiency. (medlineplus.gov)
Mitochondrial trifunctional protein deficiency (MTPD), a rare condition that leads to failure of converting certain fats to energy is characterized by decreased activity of three enzymes in the enzyme complex. (trdizin.gov.tr)
Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations. (trdizin.gov.tr)
Mitochondrial trifunctional protein deficiency: a severe fatty acid oxidation disorder with cardiac and neurologic involvement. (trdizin.gov.tr)
Mitochondrial trifunctional protein deficiency due to HADHB gene mutation in a Chinese family. (trdizin.gov.tr)
Trifunctional protein deficiency is characterized by decreased activity of all 3 enzymes. (nih.gov)
2003). Genetic Heterogeneity of Mitochondrial Trifunctional Protein Deficiency See also MTPD2 (620300), caused by mutation in the HADHB gene, the beta subunit of the mitochondrial trifunctional protein. (nih.gov)

Deletion of Txnip in the myocardium results in mitochondrial dysfunction with decreased expression of transcripts encoding mitochondrial metabolism. (jci.org)
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)
Mitochondrial dysfunction is putatively central to glioblastoma (GBM) pathophysiology but there has been no systematic analysis in GBM of the proteins which are integral to mitochondrial function. (gla.ac.uk)
Its role in the regulation of normal mitochondrial function and in different disease states attributed to mitochondrial dysfunction is not known. (nih.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)
Thus, mitochondrial adaptive cellular processes are important for physiological responses, including to nutrient availability, temperature and physical activity, and their failure leads to diseases associated with mitochondrial dysfunction such as metabolic and age-associated diseases and cancer. (nature.com)
One of the underlying mechanisms of long COVID may be mitochondrial dysfunction. (mdpi.com)
In this paper, we make the case that mitochondrial dysfunction could play an important role in the pathophysiology of depression. (frontiersin.org)
However, the data in relation to antidepressant drug effects are contradictory: some studies reveal they have no effect on mitochondrial function or even potentiate dysfunction, whereas other studies show more beneficial effects. (frontiersin.org)
The focus of this review is the link between mitochondrial dysfunction and major depression. (frontiersin.org)
In this review, we summarize some of the latest knowledge on mitochondrial dysregulation in major depression (depicted in Figure 1 ) and also discuss how mitochondrial dysfunction could instigate downstream changes in extracellular matrix proteins such as reelin, neuronal nitric oxide (nNOS), oxidative stress, and inflammation, and finally adult hippocampal neurogenesis. (frontiersin.org)
Organic dust-induced mitochondrial dysfunction could be targeted via cGAS-STING or cytoplasmic NOX-2 inhibition using microglial cells and brain slice culture models. (cdc.gov)
mitochondrial dysfunction may impair urea production and result in hyperammonemia . (medscape.com)

The unfolded protein response of the endoplasmic reticulum supports mitochondrial biogenesis by buffering nonimported proteins. (harvard.edu)
These changes can promote mitochondrial biogenesis and membrane dynamics that in turn support mitochondrial respiration. (nature.com)
Fig. 2: Transcriptional control of mitochondrial biogenesis through PGC1α. (nature.com)
One study demonstrated that although lifelong CR preserved muscle mitochondrial function in mice, it did so by protecting the integrity and function of existing mitochondrial cellular components rather than by increasing biogenesis. (medscape.com)
[ 11 ] In the CALERIE phase 1 study, however, we demonstrated that 6 months of 25% CR in healthy human s increased the expression of genes involved in mitochondrial biogenesis and mitochondrial DNA (mtDNA) copy number. (medscape.com)

Coordinated with reduced mitochondrial function, deletion of Txnip enhanced anaerobic glycolysis. (jci.org)

Oncometabolic role of mitochondrial sirtuins in glioma patients. (harvard.edu)
Fig. 4: Post-translational mechanisms governing respiratory control and the role of mitochondrial membrane dynamics. (nature.com)
Further, by investigating the role of mitochondrial cell death proteins it was determined that a distinct approach will be required to improve kidney mitochondrial health in diabetes, due to their unique metabolic profile. (monash.edu)

Nevertheless, the mechanisms related to the impairment of mitochondrial functions specific to AD remain unclear. (jneurosci.org)
This review provides an overview of the basic molecular mechanisms involved in mitochondrial fusion and focuses on the alteration in mitochondrial DNA amount resulting from impairment of mitochondrial dynamics. (centrodinoferrari.com)
Long-chain hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency and trifunctional protein (TFP) deficiency are caused by impairment of mitochondrial TFP. (nih.gov)

It has been reported that C1QBP is mainly localized in the mitochondrial matrix and is less expressed in the cytoplasm, nucleus and on the cell surface [ 9 - 11 ]. (jcancer.org)

Genotoxic agents, such as ultraviolet (UV) radiation, generates oxidative stress and cause mitochondrial DNA (mtDNA) damage. (duke.edu)
The mtDNA polymerase (Polγ), a major constituent of nucleoids, is responsible for the replication and repair of the mitochondrial genome. (duke.edu)
The results suggest that these proteins form a complex for the repair of UVB-associated mtDNA damage. (duke.edu)
They mapped the nuclear reads they generated to the human reference genome and the mtDNA reads to the Denisova 3 mitochondrial genome. (genomeweb.com)
The number of nucleotide differences among the Denisovan mtDNA samples also suggests, the researchers reported, that Denisova 8 mtDNA is more diverged and has accumulated fewer substitutions than the other Denisovan mitochondrial genomes. (genomeweb.com)
Damaged mitochondrial DNA (mtDNA) initiates and spreads Parkinson's disease (PD) pathology, potentially opening new avenues for early diagnosis, disease monitoring, and drug development. (medscape.com)
Our proteomic analysis of extracellular vesicles containing damaged mtDNA identified the TLR4 activator, ribosomal protein S3, as a key protein involved in recognizing and extruding damaged mtDNA," the investigators write. (medscape.com)
Looking ahead, they plan to investigate how mtDNA damage can serve as a predictive marker for different disease stages and progression and to explore potential therapeutic strategies aimed at restoring normal mitochondrial function to rectify the mitochondrial dysfunctions implicated in PD. (medscape.com)

2012) demonstrated that thyroid hormone stimulates mitochondrial metabolism in a pathway mediated by MTP. (wikipedia.org)
The mTOT complex functions as a molecular 'sensor switch' connecting mitochondrial metabolism to important cellular activities, such as carbohydrate, lipid, and amino acid metabolism, that are out of balance in patients with type 2 diabetes. (news-medical.net)
Liver mitochondrial cristae organizing protein MIC19 promotes energy expenditure and pedestrian locomotion by altering nucleotide metabolism. (harvard.edu)
Thioredoxin-interacting protein (Txnip) is a regulator of metabolism and an inhibitor of the antioxidant thioredoxins, but little is known about its roles in the myocardium. (jci.org)
both methods indicated suppression of genes and proteins participating in mitochondrial metabolism. (jci.org)
A and B ) PMAGE demonstrated transcriptional changes in mitochondrial metabolism-related pathways in Txnip-KO hearts. (jci.org)
Protein-protein interaction analysis highlighted a reduction in multiple proteins coupled to energy metabolism (in particular respiratory chain proteins, including 23 complex-I proteins). (gla.ac.uk)
1. Morris AAM, Spiekerkoetter U. Disorders of mitochondrial fatty acid oxidation and riboflavin metabolism. (trdizin.gov.tr)
In mice PD models that were deficient in IFNβ signaling, the investigators showed that neuronal IFNβ is required to maintain mitochondrial homeostasis and metabolism. (medscape.com)
Overall, the hepatic urea cycle is the major route for waste nitrogen disposal, generation of which is chiefly from protein and amino acid metabolism. (medscape.com)
An aldehyde dehydrogenase expressed in the mitochondrial matrix that is essential for the metabolism of ETHANOL. (bvsalud.org)

The first three components are present in a multi-subunit complex that catalyses recognition and membrane insertion of precursor proteins. (nih.gov)
Mitochondrial ribosomes (mitoribosomes) consist of a small 28S subunit and a large 39S subunit. (nih.gov)
This gene encodes a 39S subunit protein. (nih.gov)
Multi-omics identifies large mitoribosomal subunit instability caused by pathogenic MRPL39 variants as a cause of pediatric onset mitochondrial disease. (harvard.edu)
This is the expected M(r) for the fusion protein subunit. (lu.se)
Mutations in HADHB, which encodes the β-subunit of mitochondrial trifunctional protein, cause infantile onset hypoparathyroidism and peripheral polyneuropathy. (trdizin.gov.tr)

MitoCarta3.0, released 2020, uses manual literature curation to revise the previous MitoCarta2.0 inventory (78 added and 100 removed genes), provide annotation of sub-mitochondrial localization, and assign genes to a custom ontology of 149 mitochondrial pathways. (broadinstitute.org)
Protein import into this mitochondrial sub-compartment is underpinned by an intriguing variety of pathways, many of which are still poorly understood. (uni-koeln.de)
We discuss the main import pathways into the IMS, but also how IMS proteins are degraded or even retro-translocated to the cytosol in an integrated network of interactions that is necessary to maintain a healthy balance of IMS proteins under physiological and cellular stress conditions. (uni-koeln.de)
We conclude this review by highlighting new and exciting perspectives in this area with a view to develop a better understanding of yet unknown, likely unconventional import pathways, how presequence-less proteins can be targeted and the basis for dual localisation in the IMS and the cytosol. (uni-koeln.de)
Zhou L, Guo X, Chen M, Fu S, Zhou J, Ren G, Yang Z and Fan W: [Retracted] Inhibition of δ‑opioid receptors induces brain glioma cell apoptosis through the mitochondrial and protein kinase C pathways. (spandidos-publications.com)
Currently, we aim to gain further mechanistic and regulatory insight into how these pathways support cellular protein homeostasis, and uncover novel interactions of mitochondrial stress responses with other organelles. (dillinlab-berkeley.org)
mutants will be broadly useful as a method for identifying multiple types of post-translationally modified proteins and analyzing their synthesis pathways with the power of C. elegans genetic analysis.The second method that I developed is an experimental strategy for identifying proteins that contain different post-translational modifications between samples (Chapter 5). (unr.edu)

Overall, our results demonstrate an modified phosphopattern in CB2R-IN-1 the glutamatergic synapse, together with the identification of a protein kinase/phosphatase biochemical signature in the Ts65Dn murine model, which may represent novel restorative focuses on for DS synaptopathy. (logic2010.org)
The serine/threonine kinase protein kinase D1 (PKD1) is a stress-responsive kinase and sensor for reactive oxygen species, which can initiate cell survival through NF-κB signaling. (biologists.com)
Intrinsic protein kinase activity in mitochondrial. (analyteguru.com)
The detection of mitochondrial protein kinases is complicated by the low concentration of kinase relative to that of the target protein, the lack of specific antibodies, and contamination from associated, but nonmatrix, proteins. (analyteguru.com)
In this study, we use blue native gel electrophoresis (BN-PAGE) to isolate rat and porcine heart mitochondrial complexes for screening of protein kinase activity. (analyteguru.com)
The nearly ubiquitous (32)P protein labeling demonstrates protein kinase activity within each mitochondrial protein complex. (analyteguru.com)
Studies on purified Complex V and creatine kinase confirmed that these proteins undergo autophosphorylation and, to a lesser degree, tenacious (32)P-metabolite association. (analyteguru.com)
Image analysis of the matched maps identified 7 proteins that were either over- or down-expressed: activated protein kinase c receptor (LACK), alpha tubulin (×2), prostaglandin f2-alpha synthase, protein disulfide isomerase, vesicular transport protein and a hypothetical protein. (who.int)

Whereas mitochondrial ATP synthesis was minimally decreased by Txnip deletion, cellular ATP content and lactate formation were higher in Txnip-KO hearts after ischemia-reperfusion injury. (jci.org)
While these disorders can still be considered rare, defective mitochondrial dynamics seem to play a significant role in the molecular and cellular pathogenesis of more common neurodegenerative diseases, for example, Alzheimer's and Parkinson's diseases. (centrodinoferrari.com)
Mitochondrial fusion and fission are fundamental processes underlying cellular dynamics [1]. (centrodinoferrari.com)
Complement C11 binding protein (C1QBP, also named p32 and HABP1) is a multifunctional protein which plays a pivotal role in diverse cellular processes such autophagy and cell apoptosis [ 7 , 8 ]. (jcancer.org)
Fig. 3: Accumulated mitochondrial succinate regulates cellular αKG-dependent dioxygenases. (nature.com)
Richter-Dennerlein R, Dennerlein S, Rehling P (2015) Integrating mitochondrial translation into the cellular context. (uni-goettingen.de)
Cellular component GO terms and their p-values for co-expressed proteins. (lu.se)

MFN2 is also present in the endoplasmic reticulum, controlling its morphology and facilitating mitochondrial calcium influx from endoplasmic reticulum stores [8]. (centrodinoferrari.com)

Recent studies have identified the mitochondrial outer membrane proteins MOM19, MOM72, MOM38 (approximately ISP42) and p32 which have a role in initial steps of protein import. (nih.gov)
The most relevant proteins involved in the mitochondrial fusion process are three GTPase dynamin-like proteins: mitofusin 1 (MFN1) and 2 (MFN2), located in the outer mitochondrial membrane, and optic atrophy protein 1 (OPA1), in the inner membrane. (centrodinoferrari.com)
Fusion of the outer mitochondrial membrane depends on two GTPase family members: mitofusin 1 (MFN1) and mitofusin 2 (MFN2). (centrodinoferrari.com)

This gene encodes one of the mitochondrial translation elongation factors. (nih.gov)
These mitochondrial responses are governed by general principles of regulatory biology exemplified by changes in gene expression, protein translation, protein complex formation, transmembrane transport, enzymatic activities and metabolite levels. (nature.com)
In all biological systems, RNAs are associated with RNA-binding proteins (RBPs), forming complexes that control gene regulatory mechanisms, from RNA synthesis to decay. (mpg.de)
the FXN gene codes for the mitochondrial protein frataxin. (msdmanuals.com)

Mammalian mitochondrial ribosomal proteins are encoded by nuclear genes and help in protein synthesis within the mitochondrion. (nih.gov)
We determined myofibrillar and mitochondrial protein fractional synthesis rates (FSR), intramuscular signaling protein phosphorylation, and mRNA expression responses after isolated bouts of resistance exercise (RE), aerobic exercise (AE), or in combination [termed concurrent exercise (CE)] in sedentary middle-aged men. (roehampton.ac.uk)
These results occurred without an interference effect on muscle protein subfractional synthesis rates, protein signaling, or mRNA expression. (roehampton.ac.uk)
In vivo mitochondrial function [maximal ATP synthesis rate (ATPmax), ATPflux/O 2 (P/O)] was determined by 31 P-magnetic resonance spectroscopy and optical spectroscopy, and body composition was determined by dual-energy X-ray absorptiometry. (medscape.com)

Structures of the human mitochondrial ribosome bound to EF-G1 reveal distinct features of mitochondrial translation elongation. (nih.gov)

These include changes affecting mitochondrial DNA, membrane permeability, and increased formation of reactive oxygen species (ROS). (frontiersin.org)
Various samples were processed to quantify mitochondrial reactive oxygen species (mt-ROS), mt-DNA, cytochrome c, TFAM, mitochondrial stress markers and mt-DNA-induced signaling via cGAS-STING and TLR9. (cdc.gov)

Alterations in mitochondrial dynamics due to mutations in proteins involved in the fusion-fission machinery represent an important pathogenic mechanism of human diseases. (centrodinoferrari.com)
Mitochondrial translation is crucial for maintaining mitochondrial function and mutations in this system lead to a breakdown in the respiratory chain-oxidative phosphorylation system and to impaired maintenance of mitochondrial DNA. (nih.gov)
DynaMut: predicting the impact of mutations on protein conformation, flexibility and stability. (trdizin.gov.tr)

In order to collect information on nuclear coded mitochondrial proteins we developed MitoNuc and MitoAln, two related databases containing, respectively, detailed information on sequenced nuclear genes coding for mitochondrial proteins in Metazoa and yeast, and the multiple alignments of the relevant homologous protein coding regions. (nih.gov)
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)
Manganese superoxide dismutase is a nuclear encoded primary antioxidant enzyme localized exclusively in the mitochondrial matrix. (duke.edu)
NEW YORK (GenomeWeb) - Nuclear and mitochondrial DNA sequences from two Denisovan individuals suggest that the ancient hominin population was more diverse genetically than Neanderthals, but not as diverse as modern humans, according to the Max Planck Institute for Evolutionary Anthropology's Svante Pääbo and his colleagues. (genomeweb.com)
By sequencing DNA obtained from these teeth, Pääbo and his colleagues found that mitochondrial DNA diversity among Denisovans was higher than it was among Neanderthals, though nuclear DNA diversity between the groups was comparable, as they reported today in the Proceedings of the National Academy of Sciences . (genomeweb.com)
Pääbo and his colleagues extracted DNA from the samples and sequenced both the nuclear and mitochondrial genomes of these Denisovans. (genomeweb.com)

[ 12 ] The activity of key mitochondrial enzymes of the tricarboxylic acid cycle, β-oxidation, and the electron transport system, conversely, were unchanged, [ 12 ] leaving many unanswered questions regarding the effects of CR on muscle-specific mitochondrial function in humans. (medscape.com)

Structural insights into mammalian mitochondrial translation elongation catalyzed by mtEFG1. (nih.gov)

Here, we show that the protein level of ClpP is selectively decreased in αSyn-expressing cell culture and neurons derived from iPS cells of PD patient carrying αSyn A53T mutant, and in dopaminergic (DA) neurons of αSyn A53T mice and PD patient postmortem brains. (springer.com)
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)
Mice lacking mitochondrial uncoupling protein are cold-sensitive but not obese. (nature.com)

The newly identified mitochondrial protein complex is being referred to as the mitochondrial Target of Thiazolidinediones (TZDs), or mTOT ™ (ADA Abstract #1096-P). (news-medical.net)
ABSTRACT In order to define the protein expressional changes related to the process of meglumine antimoniate resistance in anthroponotic cutaneous leishmaniasis (CL), we performed a comparative proteomics analysis on sensitive and resistant strains of Leishmania tropica isolated from Iranian CL patients. (who.int)

This suggests a possible link between mitochondrial membrane cardiolipin content and beta oxidation. (wikipedia.org)
Human and animal studies suggest an intriguing link between mitochondrial diseases and depression. (frontiersin.org)
Overall, the data suggest an intriguing link between mitochondrial function and depression that warrants further investigation. (frontiersin.org)

Mitochondrial genomes have been extensively sequenced and analysed and the data collected in several specialised databases. (nih.gov)

The mitochondrial intermembrane space (IMS) houses a large spectrum of proteins with distinct and -critical functions. (uni-koeln.de)

Finally, we suggested that C1QBP is correlated with genes encoding ribosome RPL-related proteins and mitochondrial MRPL-related proteins in HCC patients. (jcancer.org)

This study highlights the complex mitochondrial proteomic adjustments which occur in GBM pathophysiology. (gla.ac.uk)

Antimycin A and FCCP cause mitochondrial depolarization. (elifesciences.org)

Calpain inhibition decreases oxidative stress via mitochondrial regulation in a swine model of chronic myocardial ischemia. (harvard.edu)
This basic two-step process has been confirmed in most mammalian cells, although its regulation and the repertoire of specific accessory proteins are likely to be highly context dependent. (centrodinoferrari.com)
Mitochondrial uncoupling proteins allow dissipation of the mitochondrial proton gradient associated with thermogenesis and regulation of radical formation. (guidetopharmacology.org)
Fig. 1: Regulation of mitochondrial respiratory capacity. (nature.com)
Mitochondrial protein phosphorylation is a well-recognized metabolic control mechanism, with the classical example of pyruvate dehydrogenase (PDH) regulation by specific kinases and phosphatases of bacterial origin. (analyteguru.com)
Collectively, this study proposes that many of the mitochondrial complexes contain an autophosphorylation mechanism, which may play a functional role in the regulation of these multiprotein units. (analyteguru.com)

Fig. 2: Succinate controls mitochondrial superoxide production through mitochondrial complex I. (nature.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)

Dozens of mitochondrial proteins were labeled with (32)P in this setting, including all five complexes of oxidative phosphorylation and several citric acid cycle enzymes. (analyteguru.com)
Traditional genetic, biochemical , and biophysical approaches for studying protein - protein interactions in complexes with defined stoichiometry yield inconclusive results when applied to clustered proteins . (bvsalud.org)

As the name suggests, mitochondrial trifunctional protein contains three enzymes that each perform a different function. (medlineplus.gov)
The kinetics of CS1 and MDH1 activities of the fusion protein were compared to those of the free enzymes. (lu.se)
It was observed that AAT was a less effective competitor for OAA when the CS1/MDH1 fusion protein is used than when the separate enzymes are employed. (lu.se)
In addition, the transient time for the coupled reaction sequence was less for the fusion protein than for the free enzymes. (lu.se)
Assessing Protein Interactions for Clustering of Mitochondrial Urea Cycle Enzymes. (bvsalud.org)

Knockout of C6orf203 leads to a decrease in mitochondrial translation and consequent OXPHOS deficiency, without affecting mitochondrial RNA levels. (mpg.de)

Interestingly, mitochondrial translocation of APP was incomplete because of an internal acidic domain present between 220 and 290 amino acids. (jneurosci.org)
Saal KA, Richter F, Rehling P, Rizzoli SO (2018) Combined Use of Unnatural Amino Acids Enables Dual-Color Super-Resolution Imaging of Proteins via Click Chemistry. (uni-goettingen.de)

Alterations in proteins in mitochondrial enriched fractions from patients with GBM were defined with label-free liquid chromatography mass spectrometry. (gla.ac.uk)
Alterations in mitochondrial functions such as oxidative phosphorylation (OXPHOS) and membrane polarity, which increase oxidative stress and apoptosis, may precede the development of depressive symptoms. (frontiersin.org)
As detailed in the right side of the image, there are a series of mitochondrial alterations that have been observed both in depressed patients and in animal models of depression (red lines). (frontiersin.org)

Mitochondrial trifunctional protein defects: molecular basis and novel therapeutic approaches. (medlineplus.gov)

The mitochondrial unfolded protein response (mtUPR) is a newly discovered mechanism for regulating mitochondrial homeostasis. (currentmedicinalchemistry.com)

A protein complex that is part of a mitochondrion. (planteome.org)

We evaluated muscle mitochondrial function and associated transcriptional profiles in nonobese humans after 12 months of CR. (medscape.com)

Activated ERK1/2 also phosphorylates the GTPase dynamin-related protein (Drp1), which then stimulates mitochondrial fission. (cdc.gov)

Proteins associated with oxidative damage (including catalase, superoxide dismutase 2, peroxiredoxin 1 and peroxiredoxin 4) were increased in GBM. (gla.ac.uk)

The human mitochondrial ribosomal protein genes: mapping of 54 genes to the chromosomes and implications for human disorders. (nih.gov)

Each group refers to a group of proteins with common function or process. (jci.org)
Loss of vacuole function triggers mitochondrial protein degradation. (elifesciences.org)
A ) Schematic illustration showing that loss of vacuolar acidity (2) through aging or concanamycin A (conc A)-mediated inhibition of the Vacuolar H + -ATPase (1) leads to loss of mitochondrial function (3) through an unknown mechanism. (elifesciences.org)
The relatively short intracellular domains interact with a variety of cytoplasmic proteins,such as b-catenin, to regulate cadherin function. (logic2010.org)
Substantial evidence has shown that αSyn toxicity may directly disrupt mitochondrial function. (springer.com)
Furthermore, the data gained from experiments using these methods have contributed new insights into our understanding of the glycosylation of proteins with mitochondrial function.The first such method is a chemical biology approach for the discovery of glycoproteins in Caenorhabditis elegans (C. elegans). (unr.edu)
Namely, I have determined that multiple proteins with established and essential mitochondrial function have low-abundance glycosylated isoforms (Chapters 3 and 4). (unr.edu)
Enzyme clustering is a phenomenon that involves partitioning of proteins that function together in a common subcellular or sub-organellar compartment. (bvsalud.org)
The normal function of N -acetylglutamate (NAG), the reaction product, is to act as an activator of carbamyl phosphate synthetase (CPS), which is also a mitochondrial enzyme. (medscape.com)
Effects of antimony on mitochondrial function and protein thiol and adenine nucleotide status in cultured cardiac myocytes. (cdc.gov)
The effects of caloric restriction (CR) on in vivo muscle mitochondrial function in humans are controversial. (medscape.com)
however, having a "more coupled" (versus "less coupled") phenotype enables CR-induced improvements in muscle mitochondrial function. (medscape.com)
Mitochondrial changes (content and function) are at the heart of a wide range of age-related diseases. (medscape.com)
Taken together, these data lead to the question of whether duration (chronic versus short-term) is a key determinant of CR-induced changes in muscle mitochondrial mass and function. (medscape.com)
To date, there have been no randomized controlled trials that have examined the effects of long-term CR on muscle mitochondrial function in humans. (medscape.com)
Decreased frataxin levels lead to mitochondrial iron overload and impaired mitochondrial function. (msdmanuals.com)

Lipid Biosynthesis Coordinates a Mitochondrial-to-Cytosolic. (dillinlab-berkeley.org)
Stefely, J. A. & Pagliarini, D. J. Biochemistry of mitochondrial coenzyme Q biosynthesis. (nature.com)

MitoCarta3.0 is an inventory of 1136 human and 1140 mouse genes encoding proteins with strong support of mitochondrial localization, now with sub-mitochondrial compartment and pathway annotations. (broadinstitute.org)
The Parkinson's disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization. (alzforum.org)
Canet-Avilés RM, Wilson MA, Miller DW, Ahmad R, McLendon C, Bandyopadhyay S, Baptista MJ, Ringe D, Petsko GA, Cookson MR . The Parkinson's disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization . (alzforum.org)

The mitochondrial trifunctional protein, composed of 4 alpha and 4 beta subunits, catalyzes 3 steps in mitochondrial beta-oxidation of fatty acids: long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), long-chain enoyl-CoA hydratase, and long-chain thiolase activities. (nih.gov)

Recent work from our lab identified two histone demethylases that regulate the mitochondrial stress response (1). (dillinlab-berkeley.org)
Two Conserved Histone Demethylases Regulate Mitochondrial Stress-Induced Longevity. (dillinlab-berkeley.org)

2012) used 2D gel electrophoresis and mass spectrometry to identify MTP as one of the proteins that interacts with ER alpha, a receptor triggered by estrogen. (wikipedia.org)
Cell proteins were analysed with 2-dimensional electrophoresis and differentially expressed proteins were identified by matrix-assisted laser desorption/ ionization time-of-flight mass spectrometry. (who.int)
Our preliminary study using 2D isolates were recovered from liq- new cases throughout the world cu- gel electrophoresis in drug sensitive/ uid nitrogen, and sub-cultured in taneous leishmaniasis (CL) remains resistant strains of L. tropica showed RPMI1640 medium (Gibco/BRL) a serious public health problem in that some proteins were differentially supplemented with 10% fetal bovine numerous countries [1,2]. (who.int)

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)
Scholars@Duke publication: Manganese superoxide dismutase is a mitochondrial fidelity protein that protects Polγ against UV-induced inactivation. (duke.edu)

Fig. 3: Translational control of mitochondrial respiratory chain assembly. (nature.com)

Although a variety of studies have shown that Complement C11 binding protein (C1QBP) may play a tumor-promoting or tumor-suppressive role in cancer, the functions and mechanisms of C1QBP in HCC progression are under-investigating. (jcancer.org)

Antimony induction of heat shock proteins in neonatal rat cardiac myocytes. (cdc.gov)

We conducted an ancillary study to the CALERIE 2 randomized controlled trial to examine the effects of 12 months of 25% CR on in vivo skeletal muscle mitochondrial energetics, mitochondrial content, and markers of oxidative stress. (medscape.com)

Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Rat Uncoupling Protein 2, Mitochondrial (UCP2) in tissue homogenates, cell lysates, cell culture supernates and other biological fluids. (thescientistconnect.com)
Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Rat Uncoupling Protein 2, Mitochondrial (UCP2) in samples from tissue homogenates, cell lysates, cell culture supernates and other biological fluids with no significant corss-reactivity with analogues from other species. (thescientistconnect.com)
Description: Quantitativesandwich ELISA kit for measuring Rat Mitochondrial uncoupling protein 2 (UCP2) in samples from serum, plasma, tissue homogenates. (thescientistconnect.com)
Description: A competitive ELISA for quantitative measurement of Rat Mitochondrial uncoupling protein 2(UCP2) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. (thescientistconnect.com)
Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Mitochondrial Uncoupling Protein 2 (UCP2) were tested 20 times on one plate, respectively. (biomatik.com)
Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Mitochondrial Uncoupling Protein 2 (UCP2) were tested on 3 different plates, 8 replicates in each plate. (biomatik.com)
This assay has high sensitivity and excellent specificity for detection of Mitochondrial Uncoupling Protein 2 (UCP2). (biomatik.com)
No significant cross-reactivity or interference between Mitochondrial Uncoupling Protein 2 (UCP2) and analogues was observed. (biomatik.com)

Anatomy22

Organisms14

Diseases9

Chemicals and Drugs132

Analytical, Diagnostic and Therapeutic Techniques and Equipment17

Phenomena and Processes56

Disciplines and Occupations2

Information Science4

Inner mitochon5

Mitochondria15

Trifunctional protein de13

Dysfunction18

Biogenesis5

Reduced mitochondrial function1

Role of mitochondrial3

Impairment of mitochondrial3

Localized in the mitochondrial matrix1

MtDNA8

Metabolism11

Subunit6

Pathways7

Kinase8

Cellular7

Endoplasmic reticulum1

Outer3

Gene4

Synthesis4

Human mitochondrial ribosome1

Reactive oxygen2

Mutations3

Nuclear6

Tricarboxylic acid1

Mammalian mitochondrial1

Mice3

Abstract2

Link between mitochondrial3

Genomes1

Intermembrane space1

Ribosome1

Complex mitochondrial1

Cause mitochondrial1

Regulation6

Controls mitochondrial1

Suppresses mitochondrial1

Complexes2

Enzymes5

Deficiency1

Amino Acids2

Alterations3

Molecular1

Homeostasis1

Mitochondrion1

Transcriptional1

Stimulates1

Oxidative damage1

Ribosomal protein1

Function16

Biosynthesis2

Localization3

Dehydrogenase1

Regulate2

Electrophoresis3

Superoxide2

Respiratory chain assembly1

Mechanisms1

Heat-Shock Pr1

Skeletal muscle1

UCP28