Protein mitochondrial membranes. Medical search. Frequent questions

The outer membrane mitochondrial proteins carry out functions for mitochondrial biogenesis and integration between mitochondria and the cellular system. (wikipedia.org)
[10] Mitochondrial biogenesis is in turn temporally coordinated with these cellular processes. (wikipedia.org)
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)
Recent evidence indicates that SLP-2 is involved in the organization of cardiolipin-enriched microdomains in mitochondrial membranes and the regulation of mitochondrial biogenesis and function. (ox.ac.uk)
Two AAA proteases with catalytic sites at opposite membrane surfaces form a membrane-integrated quality control system and exert crucial functions during the biogenesis of mitochondria. (silverchair.com)
Altered mitochondrial recycling, resulting from abnormal biogenesis/mitophagy cycles, coupled to decreased antioxidant capacity and hence unmitigated reactive oxygen species (ROS) production, all result in impaired ATP synthesis and trigger energy deficits largely responsible for the progressive cellular dysfunction characteristic of aging [ 3 , 4 ]. (aging-us.com)
The biogenesis of most proteins in the mitochondrial matrix and the inner membrane relies on the function of the TIM23 complex. (frontiersin.org)
For instance, the association of mitochondria with the endoplasmic reticulum (ER), in a juxtaposition known as Mitochondria-Associated Membrane (MAM), has an important role in controlling mitochondria biogenesis, Ca 2+ release, and lipid synthesis and apoptosis [ 10 , 11 ]. (hindawi.com)

The TOM complex forms two exit sites for precursor proteins-Tom40, Tom7, and the intermembrane space domain of Tom22-promote the transfer of presequence-containing precursors to the TIM23 complex. (wikipedia.org)
The SAM Complex is essential for sorting and assembling beta-barrel proteins from the intermembrane space side into the outer membrane. (wikipedia.org)
These compartments or regions include the outer membrane, intermembrane space , inner membrane , cristae , and matrix . (wikipedia.org)
We now include manually curated annotations of sub-mitochondrial localization (matrix, inner membrane, intermembrane space, outer membrane) as well as assignment to 149 hierarchical 'MitoPathways' spanning seven broad functional categories relevant to mitochondria. (nih.gov)
The mitochondrial intermembrane space (IMS) houses a large spectrum of proteins with distinct and -critical functions. (uni-koeln.de)

It is regulated by the recruitment of the GTPase Dynamin-related protein 1 (Drp1) by adaptors at actin- and endoplasmic reticulum-mediated mitochondrial constriction sites. (cam.ac.uk)
Jan. 17, 2023 Aging and related diseases are associated with alterations in oxidative status and low-grade inflammation, as well as a decreased endoplasmic reticulum (ER) unfolded protein response (UPR). (sciencedaily.com)
part of these networks is also established by contacts of the mitochondria with organelles (e.g., with endoplasmic reticulum, plasma membrane, and peroxisomes) [ 7 - 9 ]. (hindawi.com)
Several proteins, such as spastin ( SPG4 ) and atlastin-1 ( SPG3A ), which shape membranes of the endoplasmic reticulum or endosomes, are known as such candidates. (medscape.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)
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)
Reduced mitochondrial function in the liver and brain lead to the liver failure and neurological dysfunction associated with MPV17 -related hepatocerebral mitochondrial DNA depletion syndrome. (medlineplus.gov)
Nonetheless their association with mitochondrial dysfunction highlights this organelle as an important player during CNS homeostasis and disease. (frontiersin.org)
Yan W , Zhang T , Kang Y , Zhang G , Ji X , Feng X , Shi G , . Testosterone ameliorates age-related brain mitochondrial dysfunction. (aging-us.com)
Brain mitochondrial dysfunction and reduced testosterone levels are common features of aging in men. (aging-us.com)
Although evidence suggests that the two phenomena are interrelated, it is unclear whether testosterone supplementation ameliorates mitochondrial dysfunction in the aging male brain. (aging-us.com)
These findings suggest that testosterone supplementation may be a viable approach to ameliorating brain mitochondrial dysfunction and thus prevent or treat cognitive-behavioral deficits and neurodegenerative conditions associated with aging. (aging-us.com)
Mitochondrial dysfunction is a common feature of normal aging and is closely associated with the development of age-related neurodegenerative disease [ 1 - 3 ]. (aging-us.com)
Notwithstanding, there is a paucity of research on the effects of testosterone supplementation on age-related mitochondrial dysfunction in the brain. (aging-us.com)
Mitochondrial dysfunction is associated with many aging-related neuromuscular degenerative diseases and metabolic disorders. (upstate.edu)
Ageing leads to a gradual dysfunction of the proteostasis network and thus to proteome instability due to accumulation of damaged and/or misfolded proteins [ 2 ]. (hindawi.com)
Mitochondrial dysfunction has also been associated with ageing and most of the so-called age-related diseases [ 13 - 17 ]. (hindawi.com)
In contrast to the results for BEAS-2B and A549 cells, in RKO colon carcinoma cells ZnO NPs and not Zn2 + induce cytotoxicity and mitochondrial dysfunction. (dovepress.com)
Lysosomal function is critical for organismal homeostasis-mutations in genes encoding lysosomal proteins cause severe human disorders known as lysosomal storage diseases, and lysosome dysfunction is implicated in age-associated diseases including cancer, neurodegeneration and metabolic syndrome. (stanford.edu)
Mitochondrial dysfunction is the second process that leads to HSPs. (medscape.com)

Their transient and rapid morphological adaptations are crucial for many cellular processes such as cell cycle, immunity, apoptosis and mitochondrial quality control. (cam.ac.uk)
This proadifen-MTX synergism was also mediated by the inhibition of various cellular proteins engaged in apoptosis, including Mc-1, Bcl-xL, survivin and activation of procaspase-3. (spandidos-publications.com)
These include regulation of cell cycle, activation of mitochondrial pathway, induction of mitochondrial outer membrane permeabilization, induction of apoptosis, modulation of oxidative stress, amelioration of inflammation, modulation of insulin signaling and inhibition of angiogenesis. (mdpi.com)
Dr Czabotar said activation of the protein Bax had long been known to be an important event leading to apoptosis, but until now it was not known how this activation occurred. (edu.au)
Short-term exposure of skin cells results in apoptosis but not in an inflammatory response, while long-term exposure leads to increased ROS generation, decreased mitochondrial activity, and formation of tubular intercellular structures. (dovepress.com)
Western blotting indicated that both TiO2 NPs and Fe3O4-TiO2 NPs could induce apoptosis, inflammation, and carcinogenesis related signal protein alterations. (cdc.gov)
Membrane proteins encoded by the BCL-2 GENES and serving as potent inhibitors of cell death by APOPTOSIS. (bvsalud.org)

Effect of ischemia and reperfusion on antioxidant enzymes and mitochondrial inner membrane proteins in perfused rat heart. (unich.it)
We have also studied the mitochondrial inner membrane proteins for both molecular weight, with Coomassie blue, and thiol status, with monobromobimane stain, using a sodium dodecyl sulfate polyacrylamide gel electrophoresis technique. (unich.it)
Neither ischemia nor reperfusion effected any relevant modification of the molecular weight of the mitochondrial inner-membrane proteins either in the presence or absence of a reducing agent. (unich.it)
An ubiquitous and conserved proteolytic system regulates the stability of mitochondrial inner membrane proteins. (silverchair.com)
This result and other findings suggested that Mgr2p controls the lateral release of inner membrane proteins harboring a stop-transfer signal that follows an N-terminal amino acid signal. (frontiersin.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)
Gilkerson, R. W., Selker, J. M. L. & Capaldi, R. A. The cristal membrane of mitochondria is the principal site of oxidative phosphorylation. (nature.com)
On the other hand, regulation of mitochondrial dynamics is essential for CNS health maintenance and leading to the induction of IL-10 and reduction of TNF-α secretion, increased cell viability and diminished cell injury in addition to reduced oxidative stress. (frontiersin.org)
Besides impairing ATP production by compromising the normal function of the respiratory chain machinery, excessive ROS generation and the ensuing oxidative stress cause oxidative damage to proteins, lipids, and DNA, accelerating the aging phenotype [ 5 , 6 ]. (aging-us.com)
This possibility is indeed supported by animal studies that showed that orchiectomy-induced testosterone deficiency reduces mitochondrial function and increases oxidative damage in the substantia nigra (SN) and the hippocampus (HIPP) of adult male rats [ 16 - 18 ]. (aging-us.com)
In animals engineered to lack this protein, red blood cells quickly die by oxidative stress due to their failure to bring glutathione into mitochondria. (sciencedaily.com)
June 23, 2022 Researchers describe how they have improved the ability of a protein to repair oxidative DNA damage and created a new protein function. (sciencedaily.com)
In vitro exposure of BEAS-2B bronchial epithelial cells and A549 alveolar adenocarcinoma cells results in cytotoxicity, increased oxidative stress, increased intracellular [Ca2 + ], decreased mitochondrial membrane potential, and interleukin (IL)-8 production. (dovepress.com)
exposure results in cytotoxicity, oxidative stress, intracellular Ca2 + flux, decreased mitochondrial membrane potential, and production of IL-1β and chemokine CXCL9. (dovepress.com)

Tim50 is a subunit of the TIM23 complex that links protein translocation across the outer and inner mitochondrial membranes. (nih.gov)
Interestingly, mitochondrial translocation of APP was incomplete because of an internal acidic domain present between 220 and 290 amino acids. (jneurosci.org)
The TIM23 complex is a hub for translocation of preproteins into or across the mitochondrial inner membrane. (frontiersin.org)
Deletion of Mgr2p has been found to delay protein translocation into the matrix and accumulation in the inner membrane. (frontiersin.org)
The core of the TIM23 complex contains a pore that is essential for protein insertion into or translocation across the inner membrane. (frontiersin.org)
Overexpression of bcl-2 proteins, due to a translocation of the gene, is associated with follicular lymphoma. (bvsalud.org)
Studying the mechanisms underlying increased VDAC1 expression and membrane translocation in pancreatic beta cells, vascular endothelial cells, adipocytes, and macrophages in pathological conditions such as long-lasting hyperglycemia or low-grade inflammation. (lu.se)
Therefore, in the lab we working with molecular biological approaches such as making mutants and fusion proteins but also purely biochemical/biophysical projects, purifying proteins for reconstitution into liposomes, where the ion translocation activities can be measured by Na+ NMR using a shift reagent or by a pH sensitive, membrane-impermeable dye denoted Glu3. (lu.se)

It functions in promoting mitochondrial fission, and prevents the formation of hyperfilamentous mitochondria. (nih.gov)
The role of the mitochondrial outer membrane protein SLC25A46 in mitochondrial fission and fusion. (nih.gov)
Mitochondria are highly dynamic organelles undergoing coordinated cycles of fission and fusion, referred as 'mitochondrial dynamics', in order to maintain their shape, distribution and size. (cam.ac.uk)
Mitochondrial fission is a multi-step process allowing the division of one mitochondrion in two daughter mitochondria. (cam.ac.uk)
This article will describe an overview of the molecular mechanisms that govern mitochondrial fission and fusion in mammals. (cam.ac.uk)
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 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)
Activated ERK1/2 also phosphorylates the GTPase dynamin-related protein (Drp1), which then stimulates mitochondrial fission. (cdc.gov)

The constricted volume of the IMS and the topological segregation by the inner membrane cristae into a bulk area surrounded by the boundary inner membrane and the lumen within the cristae is an important factor that adds to the complexity of the protein import, folding and assembly processes. (uni-koeln.de)

The outer mitochondrial membrane forms the border of mitochondria towards the cellular environment. (wikipedia.org)
Fig. 3: Accumulated mitochondrial succinate regulates cellular αKG-dependent dioxygenases. (nature.com)
Disturbances in mitochondrial dynamics may influence many cellular and molecular pathways, as calcium-dependent immune activation, transcription factors phosphorylation, cytokine secretion, organelle transference and even cell death. (frontiersin.org)
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 investigate how mitochondrial function deteriorates during aging and how mitochondrial damage induces cellular degeneration and neuromuscular degenerative diseases. (upstate.edu)
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)
Cells express a pool of thousands of different proteins that need to be tightly controlled for proper cellular structure, organization, and function. (hindawi.com)
Lysosomes are membrane-bound compartments that degrade macromolecules and clear damaged organelles to enable cellular adaptation to various metabolic states. (stanford.edu)
Cellular component GO terms and their p-values for co-expressed proteins. (lu.se)
Impaired cellular membrane trafficking, more particularly, axonal transport of macromolecules and organelles, is the best-characterized genetic mechanism of HSP. (medscape.com)

There are 53 discovered human mitochondrial membrane transporters, with many others that are known to still need discovered. (wikipedia.org)

The gene mutations that cause this condition lead to an altered C19orf12 protein that likely has little or no function. (medlineplus.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)
However, an 11th protein, called gene. (cdc.gov)
This ing to Kozak's analysis (5), which explains why PB1-F2 novel protein is translated from an alternative open reading can be translated when an alternative start codon is used at frame (ORF) in the PB1 gene. (cdc.gov)
This gene encodes a mitochondrial solute carrier protein family member. (nih.gov)
The MPV17 gene provides instructions for making a protein whose function is largely unknown. (medlineplus.gov)
More than 30 mutations in the MPV17 gene have been found to cause MPV17 -related hepatocerebral mitochondrial DNA depletion syndrome, a condition characterized by liver disease and neurological problems that begin in infancy. (medlineplus.gov)
MitoNEET belongs to the CDGSH Iron-Sulfur Domain (cisd)-gene family of proteins and is a [2Fe-2S] cluster-containing protein found on the outer membrane of mitochondria. (researchgate.net)
A rare type of X-linked HSP, however, has been associated with a myelin protein gene mutation. (medscape.com)

Fig. 1: Regulation of mitochondrial respiratory capacity. (nature.com)
Link to all annotated objects annotated to positive regulation of protein insertion into mitochondrial outer membrane. (planteome.org)
We examined not just the effect of proadifen and MTX on the expression of BCRP, but we were also interested in other molecular mechanisms involved in the possible antitumour activity of proadifen alone and in combination with MTX, such as the expression of anti-apoptotic proteins, proteins involved in the regulation of BCRP and proteins involved in the reparation of chemotherapeutic drug-induced DNA damage. (spandidos-publications.com)

The TOM complex, part of the TOM/TIM supercomplex, is essential for the translocase of almost all mitochondrial proteins which consists of at least 7 different subunits. (wikipedia.org)
Sam35 caps the Sam50 β-barrel, stabilizing the core of the protein translocase. (wikipedia.org)
Predicted to be part of TIM23 mitochondrial import inner membrane translocase complex. (nih.gov)
The TIM17.23 preprotein translocase of mitochondria: composition and function in protein transport into the matrix. (nih.gov)
Genetic and structural characterization of the human mitochondrial inner membrane translocase. (nih.gov)
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)
Mgr2p/Romo1 bridges TIM23 to the presequence translocase-associated motor (PAM) and is thought to regulate the lateral release of proteins harboring stop-transfer sequences. (frontiersin.org)

Using a human cortical neuronal (HCN) culture system, we showed that full-length APP is targeted to both mitochondria and plasma membrane (PM) by virtue of N-terminal chimeric signals. (jneurosci.org)
Mitochondrial and plasma membrane pools of stomatin-like protein 2 coalesce at the immunological synapse during T cell activation. (ox.ac.uk)
Although the major pool of SLP-2 is associated with mitochondria, we show here that there is an additional pool of SLP-2 associated with the plasma membrane of T cells. (ox.ac.uk)
Both plasma membrane-associated and mitochondria-associated pools of SLP-2 coalesce at the immunological synapse (IS) upon T cell activation. (ox.ac.uk)
Based on these results, we propose that SLP-2 facilitates the compartmentalization not only of mitochondrial membranes but also of the plasma membrane into functional microdomains. (ox.ac.uk)
Our data also suggest that there may be a net exchange of membrane material between mitochondria and plasma membrane, explaining the presence of some mitochondrial proteins in the plasma membrane. (ox.ac.uk)

[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)
[22] A mitochondrion contains outer and inner membranes composed of phospholipid bilayers and proteins . (wikipedia.org)

We previously developed MitoCarta, a catalogue of over 1000 genes encoding the mammalian mitochondrial proteome. (nih.gov)
The NADH binding site, FMN and eight FeS clusters are located in the promontory part, whereas the seven membrane-spanning proteins are all encoded by mitochondrial DNA in eukaryotes, in fact the Complex I encoding genes make up half of the mtDNA in mammals. (lu.se)

Mitochondrial membrane transport proteins, also known as mitochondrial carrier proteins, are proteins which exist in the membranes of mitochondria. (wikipedia.org)
Mitochondria contain their own DNA, known as mitochondrial DNA (mtDNA), which is essential for the normal function of these structures. (medlineplus.gov)
A dysfunctional or absent MPV17 protein leads to problems with the maintenance of mtDNA, which can cause a reduction in the amount of mtDNA (known as mitochondrial DNA depletion). (medlineplus.gov)

The MPV17 protein is located in the inner membrane of cell structures called mitochondria. (medlineplus.gov)

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)
Additionally, exacerbated type I IFN responses triggered by mitochondrial DNA (mtDNA), failures in mitophagy, ER-mitochondria communication and mtROS production promote neurodegeneration. (frontiersin.org)

This dual sorting mechanism is currently being investigated, and in yeast appears to be regulated by a recently discovered subunit, the Mgr2 protein. (frontiersin.org)

These factors play a role in the function of mitochondrial membrane transport proteins. (wikipedia.org)

The mammalian mitochondrial proteome is under dual genomic control, with 99% of proteins encoded by the nuclear genome and 13 originating from the mitochondrial DNA (mtDNA). (nih.gov)
It is likely that the MPV17 protein is involved in the maintenance of mtDNA. (medlineplus.gov)
3)We investigate the mechanism of mitochondrial DNA recombination, replication and repair in healthy cells and mtDNA instability in human diseases. (upstate.edu)

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

Mitochondrial membrane ATP synthase (F1F0 ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. (joplink.net)

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

Although its function is unknown, researchers suggest that the C19orf12 protein plays a role in the maintenance of fat (lipid) molecules, a process known as lipid homeostasis. (medlineplus.gov)
In addition to the role of membrane lipid composition, several members of the machinery can undergo post-translational modifications modulating these processes. (cam.ac.uk)
We show the first near-atomic structure of yeast complex IV and the protein-protein, and lipid-protein interaction implicated in supercomplex formation. (lu.se)

Drp1 oligomerization followed by mitochondrial constriction leads to the recruitment of Dynamin 2 to terminate membrane scission. (cam.ac.uk)

Influenza A virus contains eight RNA segments and from an alternative open reading frame (ORF) in the PB1 encodes 10 viral proteins. (cdc.gov)
Typing of the RNA genome and encodes 10 viral proteins (3). (cdc.gov)

Tom40 is the protein-conducting channel of the complex with beta-barrel structure, which forms a cation-selective channel. (wikipedia.org)
Three small proteins Tom5, Tom6, Tom7 interact closely with Tom40 to assemble and stabilize the complex. (wikipedia.org)
The TOM complex also consists of a dimer of Tom40 or small Tom proteins that are held together by two Tom22 subunits. (wikipedia.org)
Protein sorting into the mitochondrial compartments always starts at the TOM complex. (wikipedia.org)
The SAM complex consists of three subunits: The β-barrel protein Sam50 and two peripheral subunits Sam35 and Sam37. (wikipedia.org)
Sam37 accommodates the release of the folded β-barrel proteins from the SAM complex. (wikipedia.org)
Tom70 transfers the precursor proteins to the MIM Complex. (wikipedia.org)
Their activity is modulated by another membrane-protein complex that is composed of prohibitins. (silverchair.com)
Fig. 2: Succinate controls mitochondrial superoxide production through mitochondrial complex I. (nature.com)
Crystal structure of mitochondrial respiratory membrane protein complex II. (nature.com)
These effects were consistent with improved mitochondrial function, reflected by testosterone-induced increases in mitochondrial membrane potential (MMP), antioxidant enzyme (GSH-PX, catalase, and Mn-SOD) expression/activity, and mitochondrial respiratory complex activities in both brain regions. (aging-us.com)
Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. (joplink.net)
It belongs to a multimeric complex and is associated with other proteins.The pleiotropic actions of TSPO render this protein an interesting target in the nervous system. (uni-regensburg.de)
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)
The classical Complex I enzyme is composed of seven membrane-spanning protein subunits and seven protein subunits protruding into the cytoplasm (in bacteria) or into the mitochondrial matrix (in eukaryotes). (lu.se)

Fig. 4: Post-translational mechanisms governing respiratory control and the role of mitochondrial membrane dynamics. (nature.com)
Here, we discuss the importance of these mitochondrial dynamics during neuroinflammation, and how they correlate either with the amelioration or worsening of CNS disease. (frontiersin.org)
Recent research has revealed important correlation of mitochondrial dynamics and the pathophysiology of brain diseases, as Alzheimer's. (frontiersin.org)
Mitochondrial dynamics: overview of molecular mechanisms. (cam.ac.uk)
Mutations in the core machinery components and defects in mitochondrial dynamics have been associated with numerous human diseases. (cam.ac.uk)
Understanding the molecular mechanisms controlling mitochondrial dynamics is crucial to decipher how mitochondrial shape meets the function and to increase the knowledge on the molecular basis of diseases associated with morphology defects. (cam.ac.uk)

The yeast metal resistance proteins, which are 850-900 amino acyl residues in length, also exhibit two or three putative TMSs. (tcdb.org)
Overexpression of the yeast proteins, Al R 1p and Mn R 2p, overcomes toxicity to aluminum and manganese, respectively. (tcdb.org)
The yeast proteins appear to exhibit broad specificity transporting a wide range of di- and trivalent metal cations. (tcdb.org)

The specific functions of mitoNEET/CISD1 remain to be fully elucidated, but the protein is involved in regulating mitochondrial bioenergetics in several metabolic diseases. (researchgate.net)

Moreover, nonreversible mitochondrial damage targets the organelle to a specific autophagic removal, namely, mitophagy. (hindawi.com)

A sensitive mass spectrometric assay for mitochondrial CoQ pool redox state in vivo. (nature.com)
This fluorescence assay (IFA) by type-specific monoclonal new protein product was discovered serendipitously dur- antibody (Dako, Cambridgeshire, UK). (cdc.gov)
Mitochondria JC-1 staining assay showed that mitochondrial membrane permeability injury occurred in both NPs treated cells. (cdc.gov)

VDAC plays a crucial role in facilitating energy metabolism by transporting ADP and ATP in and out of the outer membrane. (wikipedia.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 receptor proteins Tom70 and Tom20 recognize incoming precursor proteins, in which Tom70 is responsible for docking of precursors of hydrophobic proteins accompanied by cytosolic chaperones and Tom 20 recognizes precursor proteins of the presequence pathways. (wikipedia.org)

Transmission electron microscopy demonstrated that Ery PDT altered the C. albicans cell morphology by inducing cell wall/membrane rupture. (thieme-connect.de)

Ischaemic accumulation of succinate controls reperfusion injury through mitochondrial ROS. (nature.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)
1)Mitochondrial Precursor Over-accumulation Stress (mPOS) - We discovered that mitochondrial protein import is readily saturable within the cell and that the cytosol has a limited capacity in degrading unimported proteins. (upstate.edu)
Various mitochondrial stressors, with or without directly targeting the core protein import machinery, can cause the over-accumulation of unimported proteins in the cytosol. (upstate.edu)
This leads to a cytosolic stress that we named mitochondrial Precursor Over-accumulation Stress (mPOS). (upstate.edu)

However, two of these proteins with an apparent molecular weight of 52,0000 and 12,000 showed a decrease in the monobromobimane stain, probably due to the oxidation of their thiol groups. (unich.it)
Molecular architecture of the inner membrane of mitochondria from rat liver: a combined biochemical and stereological study. (harvard.edu)
Dr Peter Czabotar (left) and Dr Dana Westphal have visualised the molecular changes in a critical cell death protein that force cells to die. (edu.au)
Scientists at the Walter and Eliza Hall Institute have for the first time visualised the molecular changes in a critical cell death protein that force cells to die. (edu.au)
The proteostasis network (PN) is an assembly of distinct dynamic molecular pathways that control the functionality of the proteome (proteome homeodynamics) during protein synthesis, folding, trafficking, and degradation. (hindawi.com)

This protein has been mistargeted to the cell membrane, causing ATP loss that results in cell dysfunctionality. (lu.se)

The explanation for individual differences in susceptibility to lead poisoning may lie in the genetic variability of the lead-binding proteins. (medscape.com)

The major constituent of a senile plaque is β-amyloid (Αβ), which is a 40-43 amino acid peptide produced by the action of secretory pathway-associated proteases, namely β and γ secretases, at the C terminus of a type I membrane-spanning glycoprotein termed amyloid precursor protein (APP). (jneurosci.org)

Inner mitochondrial membrane constriction has been proposed to be an independent process regulated by calcium influx. (cam.ac.uk)

AIMS: To review the role played by the mitochondrial permeability transition pore (MPTP) in different physiological and pathological processes. (lu.se)
This being the case, the action would be mediated by the permeability of its membranes, through the formation of the mitochondrial permeability transition pore, and would involve phenomena such as the dissipation of the mitochondrial electrochemical potential and the release of substances from within it. (lu.se)

It was previously reported that proadifen (SKF-525A), a well-known cytochrome P450 monooxygenase inhibitor, not only has anti-proliferative potential in some cancer cell lines, but it is also able to inhibit BCRP and MRP1 transporter proteins ( 8 ). (spandidos-publications.com)

With acute lead nephrotoxicity, these lead-protein complexes are observed as typical intracellular inclusions. (medscape.com)

Loss of vacuole function triggers mitochondrial protein degradation. (elifesciences.org)
The changes in the MPV17 protein that cause MPV17 -related hepatocerebral mitochondrial DNA depletion syndrome, including the R50Q mutation, impair protein function and reduce the amount of protein that is available. (medlineplus.gov)
Mitochondrial DNA depletion impairs mitochondrial function in many of the body's cells and tissues, particularly the brain, liver, and other tissues that have high energy requirements. (medlineplus.gov)
Thus, ensuring normal mitochondrial function is critical for delaying aging and reducing the risk of age-related neurodegenerative disease [ 4 , 7 , 8 ]. (aging-us.com)
Although testosterone supplementation in male patients was shown to improve cognitive function in AD [ 12 , 13 ], and to relieve motor and nonmotor symptoms in PD [ 14 , 15 ], it remains unclear whether testosterone beneficially influences mitochondrial function in the aging brain. (aging-us.com)
The effects include mitochondrial swelling and inhibition of respiratory function and energy (adenosine triphosphate) production. (medscape.com)
The analysis pointed to SLC25A39, a protein in the mitochondrial membrane whose function was hitherto unknown. (sciencedaily.com)
which underlies the importance of TIM23 to mitochondrial function. (frontiersin.org)
Highly conserved acidic residues found in the short periplasmic loop are not essential for CorA function or Mg 2+ selectivity but may be required for proper protein folding and stability. (tcdb.org)
We have recently shown that it is possible to restore the normal function of beta-cells by blocking the protein VDAC1 (Voltage-Dependent Anion Channel 1), which is dramatically increased in beta cells of people with T2D. (lu.se)

The proteins are found on mitochondrial, microsomal, and NUCLEAR MEMBRANE sites within many cell types. (bvsalud.org)

Most of the mutations that cause this condition change single protein building blocks (amino acids) in the MPV17 protein. (medlineplus.gov)

MitoCarta3.0, including sub-mitochondrial localization and MitoPathway annotations, is freely available at http://www.broadinstitute.org/mitocarta and should serve as a continued community resource for mitochondrial biology and medicine. (nih.gov)

Mice lacking mitochondrial uncoupling protein are cold-sensitive but not obese. (nature.com)

Fig. 1: Mitochondrial bioenergetics and the coenzyme Q (CoQ) pool. (nature.com)

F-type ATPases consist of two structural domains, F1 - containing the extramembraneous catalytic core, and F0 - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. (joplink.net)

Markers of Parkinson (PD) and Alzheimer (AD) diseases are able to induce innate immune pathways induced by alterations in mitochondrial Ca 2+ homeostasis leading to neuroinflammation. (frontiersin.org)

MPV17-associated hepatocerebral mitochondrial DNA depletion syndrome: new patients and novel mutations. (medlineplus.gov)

Mitochondrial superoxide dismutase (EC 1.15.1.1) activity was depressed in ischemic as well as in reperfused hearts, though the cytoplasmic form was unmodified. (unich.it)

Fig. 3: Translational control of mitochondrial respiratory chain assembly. (nature.com)
Rich, P. R. & Maréchal, A. The mitochondrial respiratory chain. (nature.com)

Coyne, L. and X.J. Chen (2019) Consequences of inner mitochondrial membrane protein misfolding. (upstate.edu)

Mitochondrial enzymes, such as aminolevulinic acid synthase and ferrochelatase, are also inhibited by lead. (medscape.com)
The mitochondria have their own chaperones and proteolytic enzymes that remove damaged or unfolded proteins [ 18 - 20 ]. (hindawi.com)

Anatomy43

Organisms10

Diseases2

Chemicals and Drugs124

Analytical, Diagnostic and Therapeutic Techniques and Equipment20

Phenomena and Processes54

Disciplines and Occupations2

Technology, Industry, Agriculture5

Information Science3

Health Care1

Biogenesis9

Intermembrane space5

Endoplasmic reticulum4

Dysfunction20

Apoptosis7

Inner-membrane proteins5

Oxidative10

Translocation8

Fission8

Cristae1

Cellular10

Human mitochondrial membrane1

Gene9

Regulation3

Translocase7

Plasma membrane6

Mitochondrion2

Genes2

Known as mitochondrial3

Located in the inner membrane1

Neurodegeneration4

Subunit1

Transport proteins1

MtDNA3

Impairment of mitochondrial1

F1F0 ATP1

Regulates mitochondrial1

Lipid3

Drp11

Encodes2

Complex15

Dynamics6

Yeast3

Metabolic1

Mitophagy1

Assay3

Metabolism1

ATPase1

Cytosolic chaperones1

Morphology1

Accumulation6

Molecular5

Cell Membrane1

Genetic1

Proteases1

Constriction1

Permeability2

Cytochrome1

Intracellular1

Function10

Nuclear1

Amino acids1

Localization1

Mice1

Bioenergetics1

Structural1

Induce1

Mutations1

Superoxide1

Respiratory chain2

20191

Enzymes2