Mitochondrial proteins. 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)

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)

The unfolded protein response of the endoplasmic reticulum supports mitochondrial biogenesis by buffering nonimported proteins. (harvard.edu)
MiRNA-378a-3p acts as a negative regulator of mitochondrial metabolic and biogenesis pathways. (cdc.gov)

These genes each provide instructions for making part of an enzyme complex called mitochondrial trifunctional protein. (medlineplus.gov)
Mammalian mitochondrial ribosomal proteins are encoded by nuclear genes and help in protein synthesis within the mitochondrion. (nih.gov)
The human mitochondrial ribosomal protein genes: mapping of 54 genes to the chromosomes and implications for human disorders. (nih.gov)
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)
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)
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)
both methods indicated suppression of genes and proteins participating in mitochondrial metabolism. (jci.org)
Finally, we suggested that C1QBP is correlated with genes encoding ribosome RPL-related proteins and mitochondrial MRPL-related proteins in HCC patients. (jcancer.org)
We systematically screened the C. elegans genome and identified 171 genes that, when knocked down, induce the expression of an hsp-6 HSP70 reporter and encode mitochondrial proteins. (ucl.ac.uk)
These genes represent many, but not all, mitochondrial processes (e.g., mitochondrial calcium homeostasis and mitophagy are not represented). (ucl.ac.uk)
Knockdown of these genes leads to reduced mitochondrial membrane potential and, hence, decreased protein import into mitochondria. (ucl.ac.uk)

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)

MiRNA-378a regulated proteins involved in mitochondrial fusion, transcription, and fatty acid metabolism. (cdc.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)
Reducing a protein found in the mitochondria of cardiac muscle cells initiates cardiac dysfunction and heart failure, a finding that could provide insight for new treatments for cardiovascular diseases, a study led by Georgia State University has shown. (medicalxpress.com)
The researchers discovered that reducing an outer mitochondrial membrane protein, FUN14 domain containing 1 (FUNDC1), in cardiac muscle cells , also known as cardiomyocytes, activates and worsens cardiac dysfunction . (medicalxpress.com)
Also, disrupting how FUNDC1 binds to a particular receptor inhibited the release of calcium from another cell structure, the endoplasmic reticulum (ER), into the mitochondria of these cells and resulted in mitochondrial dysfunction, cardiac dysfunction and heart failure . (medicalxpress.com)
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)
Proapoptotic BAX and BAK: a requisite gateway to mitochondrial dysfunction and death. (nature.com)
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)

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)
These precursor proteins are translocated into mitochondria by the TOM complex, a protein-conducting channel in the mitochondrial outer membrane. (escholarship.org)

A and B ) PMAGE demonstrated transcriptional changes in mitochondrial metabolism-related pathways in Txnip-KO hearts. (jci.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)

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)
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)
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 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)
Nano-titanium dioxide (nano -TiO2), though one of the most utilized and produced engineered nanomaterial s (ENMs), diminishes cardiovascular function through dysregulation of metabolism and mitochondrial bioenergetics following inhalation exposure. (cdc.gov)

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)

identified a human mitochondrial protein, monolysocardiolipin acyltransferase (MLCL AT-1), that is identical in amino acid sequence to the 59-kDa C-terminal end of MTP, linking MTP to the remodeling of cardiolipin from monolysocardiolipin. (wikipedia.org)
Here, we describe a putative human mitochondrial protein, C6orf203, that contains an S4-like domain-an evolutionarily conserved RNA-binding domain previously identified in proteins involved in translation. (mpg.de)

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)

Mitochondrial trifunctional protein defects: molecular basis and novel therapeutic approaches. (medlineplus.gov)
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)
Our study found the formation of MAMs mediated by the mitochondrial membrane protein FUNDC1 was significantly suppressed in patients with heart failure, which provides evidence that FUNDC1 and MAMs actively participate in the development of heart failure," said Dr. Ming-Hui Zou, director of the Center for Molecular and Translational Medicine at Georgia State and a Georgia Research Alliance Eminent Scholar in Molecular Medicine. (medicalxpress.com)
Our study reveals the detailed molecular organization of the TOM complex and provides new insights about the mechanism of protein translocation into mitochondria. (escholarship.org)
The study sheds light on the molecular underpinnings of mitochondrial stress, contributing to a deeper comprehension of its impact on cellular health. (techexplores.org)
Unveiling the molecular intricacies of this pathway not only enhances our understanding but also paves the way for innovative treatments aimed at mitigating the effects of mitochondrial stress on overall well-being. (techexplores.org)
Workplace exposures equivalent to no or low observable proteins and other molecular chaperones (valosin-containing pro- adverse effect concentrations in animals: Step by step tein or VCP) are up-regulated to handle the increase of misfolded approach and damaged proteins which are causing oxidative stress. (cdc.gov)

This gene encodes a 39S subunit protein. (nih.gov)
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 induction of the mitochondrial unfolded protein response (UPRmt) results in increased transcription of the gene encoding the mitochondrial chaperone HSP70. (ucl.ac.uk)
the FXN gene codes for the mitochondrial protein frataxin. (msdmanuals.com)
Homo sapiens uracil-DNA glycosylase (UNG), nuclear gene encoding mitochondrial protein, transcript variant 2, mRNA. (lu.se)

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

Oncometabolic role of mitochondrial sirtuins in glioma patients. (harvard.edu)
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)

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)
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)

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)
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)
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)

Dynamin-related protein 1 (DRP1), a cytosolic dynamin-related GTPase, plays a central role in fission by promoting mitochondrial division through its oligomerization into multimeric spiral structures [5]. (centrodinoferrari.com)
Nearly all mitochondrial proteins are encoded by the nuclear genome and imported into mitochondria after synthesis on cytosolic ribosomes. (escholarship.org)
In another line of work, we found that the mitochondrial and cytosolic stress responses are coordinated by lipids (2). (dillinlab-berkeley.org)
Lipid Biosynthesis Coordinates a Mitochondrial-to-Cytosolic. (dillinlab-berkeley.org)
Cytosolic and mitochondrial forms the cancer phenotype. (who.int)

Interestingly, mitochondrial translocation of APP was incomplete because of an internal acidic domain present between 220 and 290 amino acids. (jneurosci.org)
By scrutinizing the positions of various amino acids-the essential components of proteins-within the structure, they successfully pinpointed the amino acids involved in binding and assembling the octamer. (techexplores.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)

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)

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

Antimycin A and FCCP cause mitochondrial depolarization. (elifesciences.org)
Genotoxic agents, such as ultraviolet (UV) radiation, generates oxidative stress and cause mitochondrial DNA (mtDNA) damage. (duke.edu)

Proteins encoded by the mitochondrial genome or proteins encoded by the nuclear genome that are imported to and resident in the MITOCHONDRIA. (harvard.edu)
The mtDNA polymerase (Polγ), a major constituent of nucleoids, is responsible for the replication and repair of the mitochondrial genome. (duke.edu)

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)
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)

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)
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)
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)

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)
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)
This research zeroes in on a pivotal mitochondrial protein known as DELE1, which holds the key to triggering the cell's integrated stress response (ISR)-a fundamental pathway for cellular maintenance. (techexplores.org)
Richter-Dennerlein R, Dennerlein S, Rehling P (2015) Integrating mitochondrial translation into the cellular context. (uni-goettingen.de)

The mitochondrial trifunctional protein (MTP) is a multienzyme complex of the fatty acid beta- oxidation cycle. (trdizin.gov.tr)
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)

Loss of vacuole function triggers mitochondrial protein degradation. (elifesciences.org)
Mitochondrial protein degradation is not triggered by loss of mitochondrial membrane potential or oxidative stress. (elifesciences.org)
C ) Loss of mitochondrial membrane potential or oxidative stress does not activate autophagy-dependent Tom70-GFP degradation. (elifesciences.org)
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)
Normothermic Machine Perfusion of the Liver Supports Protein Translation and Mitochondrial Function While Reducing Protein Degradation and Metabolic Imbalance: A Proteomics Study. (ox.ac.uk)
P104-012 stopped protein degradation. (cdc.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)

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)
DynaMut: predicting the impact of mutations on protein conformation, flexibility and stability. (trdizin.gov.tr)

The results suggest that these proteins form a complex for the repair of UVB-associated mtDNA damage. (duke.edu)

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

We previously developed stabilized α-helix of BCL-2 domains (SAHBs) that directly initiate BAX-mediated mitochondrial apoptosis. (nature.com)
BCL-2, BCL-X(L) sequester BH3 domain-only molecules preventing BAX- and BAK-mediated mitochondrial apoptosis. (nature.com)

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

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)

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

respiratory burst (the rapid release of Reactions of oxygen radicals with Mitochondrial oxidative phosphor- reactive oxygen species from cells) cel ular components can deplete an- ylation is a major source of oxy- that involves nicotinamide adenine tioxidants, can cause direct oxidative gen radicals of endogenous origin. (who.int)

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

These data suggest that CE-induced acute stimulation of myofibrillar and mitochondrial FSR, protein signaling, and mRNA expression are equivalent to either isolate mode (RE or AE). (roehampton.ac.uk)

A groundbreaking study by Scripps Research scientists, published in August 2023, has unveiled a significant advancement in comprehending the intricate process of mitochondrial stress signaling. (techexplores.org)

A mitochondrial uncoupling protein that is expressed in heart and skeletal muscle tissues. (online-medical-dictionary.org)

This assay has high sensitivity and excellent specificity for detection of Mitochondrial Uncoupling Protein 2 (UCP2). (biomatik.com)

Manganese superoxide dismutase is a nuclear encoded primary antioxidant enzyme localized exclusively in the mitochondrial matrix. (duke.edu)
Mitochondrial Triglyceride (TG) transfer protein (MTP) is a key enzyme for apoB-containing lipoprotein assembly and secretion. (current-pharmaceutical-design.com)
Enzyme clustering is a phenomenon that involves partitioning of proteins that function together in a common subcellular or sub-organellar compartment. (bvsalud.org)

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)

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

Assessing Protein Interactions for Clustering of Mitochondrial Urea Cycle Enzymes. (bvsalud.org)
These approaches can be used to study interactions among proteins that form clusters. (bvsalud.org)

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)

Researchers at Metabolic Solutions Development Company, LLC (MSDC) have identified a mitochondrial protein complex through which anti-diabetic drugs exert their insulin sensitizing effects when used to treat patients diagnosed with type 2 diabetes . (news-medical.net)
2008). A mitochondrial protein compendium elucidates complex I disease biology. (broadinstitute.org)
This study highlights the complex mitochondrial proteomic adjustments which occur in GBM pathophysiology. (gla.ac.uk)
Recent studies suggest that the mitochondria contain fidelity proteins and MnSOD constitutes an integral part of the nucleoid complex. (duke.edu)
In-gel Complex IV activity was shown to be inhibited by ATP, and partially reversed by phosphatase activity, consistent with an inhibitory role for protein phosphorylation in this complex. (analyteguru.com)
The unique structure of DELE1, which forms an octamer, or an eight-fold complex, consisting of identical protein fragments, has emerged as a central element in this exploration. (techexplores.org)
Link to all annotated objects annotated to mitochondrial protein complex. (planteome.org)
Link to all direct and indirect annotations to mitochondrial protein complex. (planteome.org)
Cardiac function, utilizing the Vevo 2100 Imaging System, electron transport chain complex activities, and mitochondrial respiration assessed cardiac and mitochondrial function. (cdc.gov)

Although MLCL AT-1 and MTP are different proteins, in 2012 the same lab discovered that MTP did indeed have cardiolipin remodeling capabilities. (wikipedia.org)
This suggests a possible link between mitochondrial membrane cardiolipin content and beta oxidation. (wikipedia.org)

The specific targeting of precursor proteins synthesized in the cytosol to various cell organelles is a central aspect of intracellular protein traffic. (nih.gov)

ATP-dependent Clp protease (ClpP), a mitochondrial matrix protease, plays an important role in regulating mitochondrial protein turnover and bioenergetics activity. (springer.com)

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

Alterations in proteins in mitochondrial enriched fractions from patients with GBM were defined with label-free liquid chromatography mass spectrometry. (gla.ac.uk)

Wiedemann N, van der Laan M, Hutu DP, Rehling P, Pfanner N (2007) Sorting switch of mitochondrial presequence translocase involves coupling of motor module to respiratory chain. (uni-goettingen.de)

Among different species, the proteins comprising the mitoribosome differ greatly in sequence, and sometimes in biochemical properties, which prevents easy recognition by sequence homology. (nih.gov)
In addition, the transient time for the coupled reaction sequence was less for the fusion protein than for the free enzymes. (lu.se)
We propose that compromised mitochondrial protein import signals the induction of UPRmt and that the mitochondrial targeting sequence of ATFS-1 functions as a sensor for this signal. (ucl.ac.uk)

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)

Anatomy22

Organisms14

Diseases9

Chemicals and Drugs132

Analytical, Diagnostic and Therapeutic Techniques and Equipment17

Phenomena and Processes56

Disciplines and Occupations2

Information Science4

Inner mitochon5

Trifunctional protein de13

Biogenesis2

Genes11

Impairment of mitochondrial3

Involved in mitochondrial fusion1

Dysfunction13

Outer4

Pathways6

Metabolism9

Kinase7

Human mitochondrial protein2

Abstract2

Molecular7

Gene5

Reduced mitochondrial function1

Role of mitochondrial2

Subunit5

Localization4

Cytosolic5

Amino Acids3

Trigger mitochondrial1

Intermembrane space1

Cause mitochondrial2

Genome2

Suppresses mitochondrial2

Complexes3

Cellular5

Fatty2

Degradation6

Synthesis2

Mutations2

MtDNA1

Endoplasmic reticulum1

Apoptosis2

Mitochondrion1

Mammalian1

Oxidative damage1

Reactive oxygen1

Deficiency2

MRNA1

20231

Skeletal Muscle1

Specificity1

Enzyme3

Pyruvate1

Heat-Shock Pr1

Interactions2

Regulate2

Complex9

Cardiolipin2

Precursor proteins1

Bioenergetics1

Homeostasis1

Alterations1

Respiratory1

Sequence3

Electrophoresis3