Mitochondrial cardiolipin. Medical search. Frequent questions

Thus, decreasing glycolysis and augmenting mitochondrial function could be beneficial to tackle the disease. (nature.com)

Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (avivasysbio.com)

Nonetheless their association with mitochondrial dysfunction highlights this organelle as an important player during CNS homeostasis and disease. (frontiersin.org)
One of the underlying mechanisms of long COVID may be mitochondrial dysfunction. (mdpi.com)
Cardiolipin as a biomarker of mitochondrial dysfunction associated with Parkinson's disease. (cdc.gov)
Both α-Synuclein (αSyn) accumulation and mitochondrial dysfunction have been implicated in the pathology of Parkinson's disease (PD). (springer.com)
Although the mechanisms underlying PD remain elusive, α-Synuclein (αSyn) accumulation and mitochondrial dysfunction have been recognized as major contributors [ 1 , 57 ]. (springer.com)
Mitochondrial dysfunction includes heritable diseases, acquired pathologies, and age-related declines in health. (biorxiv.org)
Researchers have known for some time that mitochondrial dysfunction has something to do with the onset and progression of nonalcoholic fatty liver. (eurekalert.org)
Cardiolipin peroxidation can lead to mitochondrial dysfunction. (eurekalert.org)

Before fusion, curving of the outer membranes is promoted by the phospholipase D-dependent hydrolysis of cardiolipin. (centrodinoferrari.com)
PGPs have a net charge of -1 at physiological pH and are found in high concentration in mitochondrial membranes and as components of pulmonary surfactant. (hmdb.ca)
Szeto-Schiller (SS) peptides comprise a class of amphipathic tetrapeptides that have demonstrated efficacy in treating a wide array of mitochondrial disorders, and are believed to target mitochondrial membranes due to their enrichment in the anionic phospholipid cardiolipin (CL). (biorxiv.org)
In this study, we have analyzed the interactions of the lead compound SS-31 (Elamipretide) with model and mitochondrial membranes using biophysical and computational approaches. (biorxiv.org)
Most notably, SS-31 modulates the surface electrostatic properties of model and mitochondrial membranes, which could play a significant role in the mitoprotective properties of this compound. (biorxiv.org)
As a proof of concept, we show that SS-31 alters ion distribution at the membrane interface with implications for maintaining mitochondrial membranes subject to divalent cation (calcium) stress. (biorxiv.org)
Cardiolipin promotes pore-forming activity of alpha-synuclein oligomers in mitochondrial membranes. (mpg.de)
The space between inner and outer mitochondrial membranes is called intermembrane space. (notesbard.com)

The increase in the heart respiratory chain proteome suggests an increase in mitochondrial oxidative capacity, which could compensate for the energy deficit caused by the inhibition of glycolysis. (nature.com)
In several models of lymphocyte apoptosis, two alterations of mitochondrial function precede advanced DNA fragmentation: (1) a reduction of mitochondrial transmembrane potential (delta psi m) and (2) an increase in mitochondrial generation of superoxide anion. (nih.gov)
This phenomenon was not accompanied by an increase in mitochondrial respiratory activity. (elifesciences.org)

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

The tafazzin protein is involved in altering a fat (lipid) called cardiolipin, which plays critical roles in the mitochondrial inner membrane. (medlineplus.gov)
Specifically binds to cardiolipin (in vitro) but not to the precursor lipid phosphatidylglycerol. (avivasysbio.com)
Membrane Medicine is a targeted neurometabolic approach for neurological disorders including autism, seizures, pervasive developmental delay (PDD), and mitochondrial using bioactive lipid therapy. (autismone.org)
Our optimization of membrane and neurometabolic function with the use of targeted bioactive lipids, utilized both orally and intravenously, has has yielded marked clinical neurological improvement in patients over the past 21 years following 3 months of a targeted phospholipid regime corresponding with significant normalization in red cell lipid analysis, cardiolipin, peroxisomal/mitochondrial respiration, epigenetic status, cellular structure, cell leaflets, and membrane phospholipid arrangement. (autismone.org)
Cardiolipin (also known as PDL6) is an indispensable lipid required for mitochondrial respiration that is generated through de novo synthesis and remodeling. (wustl.edu)

Cardiolipin is the distinctive inner mitochondrial membrane phospholipid coupling structure with metabolism and signaling function. (barthsyndrome.org)
The multi-subunit mitochondrial contact site and cristae organizing system (MICOS) is a conserved protein complex of the inner mitochondrial membrane that is essential for maintenance of cristae architecture. (rug.nl)
Eth)high cells display a reduced incorporation of the cardiolipin-specific dye nonyl-acridine orange (NAO), showing a structural defect of the cardiolipin-containing inner mitochondrial membrane. (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)
The molecular machinery responsible for energy transformation is the oxidative phosphorylation (OXPHOS) system, which is canonically composed of five multiprotein complexes embedded in the inner mitochondrial membrane. (elifesciences.org)
Cardiolipin remodeling enables protein crowding in the inner mitochondrial membrane. (bvsalud.org)
Tau-induced mitochondrial membrane perturbation is dependent upon cardiolipin. (mpg.de)
The membrane in the inner side or which is facing the cytoplasm is called the inner mitochondrial membrane. (notesbard.com)
The membrane in the outside is called outer mitochondrial membrane. (notesbard.com)
And the outer mitochondrial membrane is fairly smooth. (notesbard.com)
Many solutes can't pass this membrane due to very high content of cardiolipin. (notesbard.com)
Cardiolipin is a phospholipid molecule present in inner mitochondrial membrane. (notesbard.com)

This manuscript will be of broad interest to the field of mitochondrial bioenergetics. (elifesciences.org)

Briefly, CD8+ T cells, responsible for fighting infections and safeguarding our organism from cancer, rely on the presence of cardiolipin and on its regulated synthesis and remodeling to properly activate, expand and eliminate pathogens or tumor cells. (barthsyndrome.org)
In synthesis, it appears that mitochondrial alterations occur in a significant percentage of circulating T lymphocytes from HIV-1 carriers. (nih.gov)
PGP also serves as a precursor for the synthesis of cardiolipin. (hmdb.ca)

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

SARS-CoV-2 infection of cultured cells is accompanied by elevated glycolysis and decreased mitochondrial function, whereas 2DG represses glycolysis and stimulates respiration, and restricts viral replication. (nature.com)
Although this phenomenon has long been identified and has been suggested that weight loss may alter skeletal muscle mitochondrial respiration, the mechanisms are not fully understood and direct evidence is lacking. (news-medical.net)
Progression of autism, seizures and PDD are hallmarked by impaired peroxisomal and mitochondrial respiration and phospholipid instability due to altered gene expression following toxic exposure. (autismone.org)

The mitochondrial signature phospholipid cardiolipin shows a stabilizing function on Mic10 oligomers. (rug.nl)

Once altered by tafazzin, cardiolipin is key in maintaining mitochondrial shape, energy production, and protein transport within cells. (medlineplus.gov)
QIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphology. (avivasysbio.com)
AAIR8193;Apolipoprotein O-like;CXorf33;FAM121A;family with sequence similarity 121A;Mic27;MICOS complex subunit MIC27;MICOS27;mitochondrial contact site and cristae organizing system subunit 27;Protein FAM121A;UNQ8193. (avivasysbio.com)
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)

In addition, changes in mitochondrial energy metabolism, mitochondrial proteomes, and mitochondrial lipidomes were examined in weight-loss mice. (news-medical.net)
Many of the mitochondrial phenomena we investigate exert effects on these processes and thus manifest as changes in mitochondrial network morphology. (brocku.ca)

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

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)

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

Our findings indicate that the Mic10 core machinery of MICOS is regulated by several mechanisms, including interaction with cardiolipin and antagonistic actions of Mic26 and Mic27. (rug.nl)
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)
This study provides new insights into the mitochondrial mechanisms underlying DR intervention in obesity. (myjournals.org)
To learn about basic mitochondrial and cellular biology, understand mechanisms of disease, and develop drugs, cell culture is indispensable. (brocku.ca)

Evolutionary biologists view the presence of the diminutive mitochondrial genome as a vestige of this organelle's evolutionary history. (reasons.org)
[5] The eukaryote Monocercomonoides is known to have completely lost its mitochondria, [6] and the multicellular organism Henneguya salminicola is known to have retained mitochondrion-related organelles in association with a complete loss of their mitochondrial genome. (wikipedia.org)

In April 2023, Professor Katsuhiko Funai at University of Utah published a study in Life Metabolism entitled 'Weight loss increases skeletal muscle mitochondrial energy efficiency in obese mice'(DOI: 10.1093/lifemeta/load014). (news-medical.net)
Funai and colleagues found that during weight loss in obese mice, the efficiency of skeletal muscle mitochondrial oxidative phosphorylation increased, resulting in a reduction in energy expenditure throughout the body, which in turn contributes to weight loss rebound. (news-medical.net)
The results showed that there was no significant change in mitochondrial proteomes or respiratory chain supercomplex formation in skeletal muscle of weight-loss mice, but oxidative phosphorylation efficiency significantly increased. (news-medical.net)
Finally, by analyzing the lipidome of skeletal muscle in weight-loss mice, the authors found that weight loss accelerated the remodeling of mitochondrial cardiolipin (CL) acyl-chains to increase tetralinoleoyl CL (TLCL) content, a species of lipids thought to be functionally critical for the respiratory enzymes. (news-medical.net)
Altogether, the evidence suggests that weight loss leads to an increase in skeletal muscle mitochondrial energy production efficiency, thereby reducing energy expenditure throughout the body. (news-medical.net)
2023). Weight loss increases skeletal muscle mitochondrial energy efficiency in obese mice. (news-medical.net)

Eth conversion is generated during mitochondrial electron transport. (nih.gov)
relevant publication: Cardiolipin promotes electron transport between ubiquinone and complex I to rescue PINK1 deficiency, Vos et al. (pressebox.com)

How does mitochondrial Grx2 protect from doxorubicin toxicity, cardiolipin peroxidation and apoptosis? (mpg.de)
For example, one of the two lipids, cardiolipin, is vulnerable to a chemical reaction called peroxidation with reactive oxygen byproducts of energy production. (eurekalert.org)

Adult-onset obesity is triggered by impaired mitochondrial gene expression. (avivasysbio.com)

Whether this might be mediated by the reduction or loss of cardiolipin or the accumulation of specific cardiolipin species, how this could happen and whether this could exacerbate or worsen muscle condition in Barth syndrome patients will be the core of this project. (barthsyndrome.org)
Despite the impressive accumulation of knowledge of mitochondrial function, the models could not explain the symptoms of these diseases, and researchers were unable to predict who would develop these diseases or how severe they would be, or explain their origin and mechanism. (sciencedaily.com)

Substantial evidence has shown that αSyn toxicity may directly disrupt mitochondrial function. (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)
Mitochondrial Oxygen Toxicity. (brocku.ca)
Mitochondrial zinc toxicity. (brocku.ca)

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)
Mitochondrial oxidative stress plays a crucial role in the occurrence and development of atherosclerosis. (eurekaselect.com)

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

[10] Mitochondrial biogenesis is in turn temporally coordinated with these cellular processes. (wikipedia.org)

Other biochemical and morphological mitochondrial abnormalities also exist. (bvsalud.org)

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)

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)
Molecular docking analysis for different enzymes involved in aging (collagenase, elastase and hyaluronidase), antioxidant enzymes (superoxide dismutase, glutathione-s-transferase, glutathione peroxidase and catalase) and mitochondrial enzymes (nicotinamide adenine dinucleotide (NAD)+hydrogen (H) dehydrogenase, succinate dehydrogenase, cytochrome c oxidase and adenosine triphosphate synthase) was carried out for epigallocatechin gallate alone (1), withaferin A alone (2), epigallocatechin gallate and withaferin A in combination (3) and a reference molecule. (ijpsonline.com)
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)
Their findings, published in the journal Molecular & Cellular Proteomics , show that many proteins involved in mitochondrial function, especially those directly involved in making ATP, are broken down more quickly than usual in a fatty liver. (eurekalert.org)
Exposure to LPS selectively decreased levels of carbon 20 (C 20 )-containing cardiolipin molecular species, whereas the content of C 18 or C 16 species was not significantly altered, consistent with decreased levels of Alcat1. (wustl.edu)
Exposure to LPS selectively decreased levels of carbon 20 (C20)-containing cardiolipin molecular species, whereas the content of C18 or C16 species was not significantly altered, consistent with decreased levels of Alcat1. (wustl.edu)

They see the biochemical similarities between mitochondrial and bacterial genomes as further evidence for the evolutionary origin of these organelles. (reasons.org)

In addition to being considered a potent antioxidant, melatonin has also been found to play an important role in mitochondrial homeostasis. (eurekaselect.com)

The importance of cardiolipin in mitochondrial function is evidenced by the X-linked inherited pathology Barth syndrome a result of cardiolipin remodeling deficiency. (barthsyndrome.org)
Defects in the adaptive branch of immunity might also further deteriorate muscle function if inflammation is modulated by cardiolipin deficiency. (barthsyndrome.org)
The discovery confirms the model proposed by the team in 2008 to account for observations that could not be explained by the established model of mitochondrial function. (sciencedaily.com)
This situation revealed that our knowledge of mitochondrial function was much less complete than had been believed and that our models were inadequate. (sciencedaily.com)
Plays a crucial role in crista junction formation and mitochondrial function (PubMed:23704930), (PubMed:25764979). (avivasysbio.com)
Diet restriction (DR) ameliorates obesity by regulating mitochondrial function. (myjournals.org)
Cardiolipin (CL), a mitochondrial phospholipid, is closely associated with mitochondrial function. (myjournals.org)
Source-Omega ( www.source-omega.com ) today asserts the newest DHA omega-3 structure-function claim filed with the FDA to demonstrate how their exclusive product, SOURCE OIL® - Water Extracted Chromista Oil - best supports mitochondrial health and maintenance. (source-omega.com)
SwissProt ID, Q6UWP7) is destabilized in epithelia by lipopolysaccharide (LPS) impairing mitochondrial function. (wustl.edu)

In addition, systemic effects of cardiolipin deficiency like neutropenia, T cell defects or other immune-related pathologies are important comorbidity factors in Barth syndrome patients in particular in respect to their higher susceptibility to recurrent infections. (barthsyndrome.org)
Prof. Patrik Verstreken (VIB-KU Leuven): "My team recently made groundbreaking discoveries related to mitochondrial defects in Parkinson's Disease. (pressebox.com)

Anti-cardiolipin antibodies (ACA) are antibodies often directed against cardiolipin and found in several diseases, including syphilis, antiphospholipid syndrome, livedoid vasculitis, vertebrobasilar insufficiency, Behçet's syndrome, idiopathic spontaneous abortion, and systemic lupus erythematosus (SLE). (wikipedia.org)
Recent research has revealed important correlation of mitochondrial dynamics and the pathophysiology of brain diseases, as Alzheimer's. (frontiersin.org)
This view was overturned by the description of mitochondrial diseases. (sciencedaily.com)

Mammalian mitochondrial respiratory chain (MRC) complexes are able to associate into quaternary structures named supercomplexes (SCs), which normally coexist with non-bound individual complexes. (elifesciences.org)

Review/Rese a: Novel insights into the mechanism of electron transfer in mitochondrial cytochrome c. (us.es)
Review/Rese a: Mitochondrial cytochrome c shot towards histone chaperone condensates in the nucleus. (us.es)

Mitochondrial fusion and fission are fundamental processes underlying cellular dynamics [1]. (centrodinoferrari.com)

To facilitate this we developed the Mitochondrial Network Analysis (MiNA) toolset as a simple set of macros making use of existing ImageJ plug-ins that allows a semi-automated analysis of mitochondrial networks in cultured mammalian cells. (brocku.ca)

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)
Recent high-throughput studies of lipids and proteins in liver mitochondria from patients and mice with nonalcoholic steatohepatosis (NASH) or its precursor, nonalcoholic fatty liver disease (NAFLD), investigate how mitochondrial energy. (eurekalert.org)
As a result, the authors observed mitochondrial and ATP shortages in the cells of mice with fatty liver. (eurekalert.org)

Phosphatidylglycerolphosphate may be present in animal tissues merely as a precursor for diphosphatidylglycerol (cardiolipin). (hmdb.ca)

The syndrome is characterized by muscular weakness, growth retardation, DILATED CARDIOMYOPATHY, variable NEUTROPENIA, 3-methylglutaconic aciduria (type II) and decreases in mitochondrial CARDIOLIPIN level. (bvsalud.org)

This is in contrast to rheumatoid arthritis with systemic sclerosis (scleroderma) because anti-cardiolipin antibodies are present in both conditions, and therefore may tie the two conditions together. (wikipedia.org)

We also review the literature describing the main disorders associated with the disruption of mitochondrial fusion. (centrodinoferrari.com)

My previous work started to shed light on the effects of cardiolipin deficiency in CD8+ T cell immune response (Corrado et al. (barthsyndrome.org)
Of note, investigating further the effects of cardiolipin deficiency or modification (oxidation/saturation) in the adaptive immune response is pivotal in the context of current (Tazpower) or future trials impacting cardiolipin remodeling and/or oxidation. (barthsyndrome.org)

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)
Mitochondrial dynamics in health and disease. (brocku.ca)

QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease. (avivasysbio.com)
This study aimed to evaluate the anti-obesity effects of graded levels of DR based on mitochondrial CL levels in the liver. (myjournals.org)
The altered profile of mitochondrial CL in the liver was explored using a targeted metabolomics strategy by ultra-high-pressure liquid chromatography MS/MS coupled with quadrupole time-of-flight mass spectrometry. (myjournals.org)
The first two studies illuminate how mitochondrial energy production stutters and fails as fatty liver disease progresses. (eurekalert.org)
Researchers at Northeast Ohio Medical University studied the lifespan of mitochondrial proteins in a mouse model of fatty liver disease. (eurekalert.org)
More detailed study will be needed to determine whether, as the authors hypothesize, mitochondrial overwork contributes to mitochondrial failure and liver disease progression. (eurekalert.org)

among them, mitochondrial fission 1 and mitochondrial fission factor have been proposed to be involved in DRP1 recruitment, although recent in vitro studies seem to not support this hypothesis [6, 7]. (centrodinoferrari.com)

The mitochondrial matrix contain dsDNA, mitochondrial ribosomes, tRNAs and various proteins. (notesbard.com)

Mitochondrial dsDNA is normally circular and has similarities with bacteria. (notesbard.com)

As a result, tafazzin cannot alter cardiolipin, and levels of functional cardiolipin are reduced. (medlineplus.gov)
A lack of functional cardiolipin and an excess of MLCL are thought to impair normal mitochondrial shape and functions. (medlineplus.gov)

Augmenting mitochondrial function1

Outer membrane1

Dysfunction8

Membranes8

Increase in mitochondrial3

Important role in regulating mi1

Lipid5

Membrane13

Bioenergetics1

Synthesis3

Intermembrane space1

Respiration3

Phospholipid cardiolipin1

Protein4

Changes in mitochondrial2

Morphology3

Trigger mitochondrial1

Mitophagy1

Mechanisms4

Genome2

Skeletal muscle6

Electron transport2

Peroxidation2

Triggered by impaired1

Accumulation2

Toxicity4

Oxidative stress2

Alterations2

Biogenesis1

Morphological1

Respiratory activity1

Molecular6

Biochemical1

Homeostasis1

Function9

Defects2

Diseases3

Complexes1

Cytochrome2

Cellular1

Mammalian1

Mice3

Precursor1

Neutropenia1

Systemic1

Disorders1

Deficiency2

Dynamics2

Liver6

Hypothesis1

Matrix1

Bacteria1

Functional2