Mammalian mitochondrial. Medical search. Frequent questions

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)
Here we employ spatially restricted proximity labelling and immunodetection to demonstrate that NSUN2 is imported into the matrix of mammalian mitochondria. (radygenomics.org)
Here we demonstrate that mammalian mitochondria can take up intact NAD + , and identify SLC25A51 (also known as MCART1)-an essential 6,7 mitochondrial protein of previously unknown function-as a mammalian mitochondrial NAD + transporter. (princeton.edu)
Loss of SLC25A51 decreases mitochondrial-but not whole-cell-NAD + content, impairs mitochondrial respiration, and blocks the uptake of NAD + into isolated mitochondria. (princeton.edu)
Conversely, overexpression of SLC25A51 or SLC25A52 (a nearly identical paralogue of SLC25A51) increases mitochondrial NAD + levels and restores NAD + uptake into yeast mitochondria lacking endogenous NAD + transporters. (princeton.edu)
Together, these findings identify SLC25A51 as a mammalian transporter capable of importing NAD + into mitochondria. (princeton.edu)
MAVS is located in the outer membrane of the mitochondria, peroxisomes, and mitochondrial-associated endoplasmic reticulum membrane (MAM). (wikipedia.org)
Then, having established the full complement of mitochondria in the fully grown oocyte, there is no further increase of the mitochondrial population during early development. (nih.gov)
If the participation of mitochondria in the regulation of sperm-triggered Ca2+ oscillations is now well established, the role of other properties of mitochondrial function during development remain largely unexplored probably due to the difficulty of accessing the mitochondrial compartment in an embryo. (nih.gov)
Mitochondrial energetic adaptations encompass a plethora of conserved processes that maintain cell and organismal fitness and survival in the changing environment by adjusting the respiratory capacity of mitochondria. (nature.com)
In a new study, Rockefeller scientists identify a key molecule that ferries glutathione, the body's major antioxidant, into the cell's mitochondria, where free radicals are produced en masse. (sciencedaily.com)
We primarily focus on a intracellular signaling pathway known as the mitochondrial unfolded protein response (or UPR mt ), which allows cells to monitor the function of the entire cellular pool of mitochondria and adapt transcription accordingly. (umassmed.edu)
I use a variety of techniques to examine mitochondrial function (isolated mitochondria, permeabilized fibres, whole muscle incubations), use molecular biological approaches to up-and down-regulate mitochondrial proteins, as well exercise, altered nutrition and aerobic training to study novel regulation in mitochondrial bioenergetics. (uoguelph.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)
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 DNA (mtDNA) is DNA that is located in mitochondria. (dadamo.com)
However many proteins found in the mitochondria are encoded by nuclear DNA: some, if not most, are thought to have been originally part of the mitochondrial DNA but have since been transferred to the nucleus during evolution. (dadamo.com)
The existence of separate mitochondrial DNA suggests that, at one point, mitochondria were separate entities from their current host cells. (dadamo.com)
Mitochondria in mammalian sperm are usually destroyed by the egg cell after fertilization. (dadamo.com)
The ACSF3 enzyme is found only in mitochondria and is involved in mitochondrial fatty acid synthesis. (medlineplus.gov)

The mitochondrial genome encodes 22 tRNAs, 2 rRNAs and 11 mRNAs and their post-transcriptional modification constitutes one of the key regulatory steps during mitochondrial gene expression. (radygenomics.org)
The BN rat sequence is the third complete mammalian genome to be deciphered, and three-way comparisons with the human and mouse genomes resolve details of mammalian evolution. (nih.gov)
This first comprehensive analysis includes genes and proteins and their relation to human disease, repeated sequences, comparative genome-wide studies of mammalian orthologous chromosomal regions and rearrangement breakpoints, reconstruction of ancestral karyotypes and the events leading to existing species, rates of variation, and lineage-specific and lineage-independent evolutionary events such as expansion of gene families, orthology relations and protein evolution. (nih.gov)
Our results imply that rare 6mA in the mammalian genome is introduced by RNA m 6 A machinery via a non-targeted mechanism. (nature.com)
Lin YF, Schulz AM, Pellegrino MW, Lu Y, Shaham S, Haynes CM. (2016) Maintenance and propagation of a deleterious mitochondrial genome by the mitochondrial unfolded protein response . (umassmed.edu)
In multiple species, certain tissue types are prone to acquiring greater loads of mitochondrial genome (mtDNA) mutations relative to others, however the mechanisms that drive these heteroplasmy differences are unknown. (biorxiv.org)
We found that the conserved PTEN-induced putative kinase (PINK1/PINK-1) and the E3 ubiquitin-protein ligase parkin (PDR-1), which are required for mitochondrial autophagy (mitophagy), underlie stereotyped differences in heteroplasmy of a deleterious mitochondrial genome mutation (ΔmtDNA) between major somatic tissues types in Caenorhabditis elegans . (biorxiv.org)
Variants within the high copy number mitochondrial genome (mtDNA) can disrupt organelle function and lead to severe multisystem disease. (jci.org)

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)
I hypothesized that this pattern of rapid suppression of liver mitochondrial metabolism with gradual reversal, was related to changes in the phosphorylation state of Ictidomys tridecemlineatus ' mitochondrial proteins in different stages of torpor bouts. (uwo.ca)
Mammalian mitochondrial ribosomal proteins are encoded by nuclear genes and help in protein synthesis within the mitochondrion. (nih.gov)
Mammalian mitochondrial ribosomal proteins (4). (nih.gov)
Proteins of indi- mitochondrial enzymes, in Ixodes scapularis and Ambly- vidual ticks were reduced with 100 mmol/L dithiothreitol, omma americanum ticks for months after molting. (cdc.gov)
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)
As with other mitochondrial carrier family proteins, the ornithine carrier is composed of 300 amino acids that constitute 3 repeated motifs of approximately 100 amino acids each. (medscape.com)

Cytosine-5 methylation (m5C) has been detected in mitochondrial transcriptome, however its biogenesis has not been investigated in details. (radygenomics.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)
The Haynes Laboratory is interested in how cells and organisms adapt to mitochondrial dysfunction in a number of physiologic and pathologic scenarios including during aging, oocyte biogenesis, cancer cell growth, stem cell maintenance and bacterial infection. (umassmed.edu)
We elucidated biogenesis of 5-formylcytidine in human mitochondrial tRNA. (nii.ac.jp)
Exercise-Induced Reductions in Mitochondrial ADP Sensitivity Contribute to the Induction of Gene Expression and Mitochondrial Biogenesis Through Enhanced Mitochondrial H 2 O 2 Emission. (uoguelph.ca)
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)

Mitochondrial DNA (mtDNA) is a small circular double-stranded DNA molecule that contains information that is essential for efficient energy production. (umu.se)
The composition of the mitochondrial DNA (mtDNA) in each cell is in constant flux through processes of mutation, replication, and degradation. (biorxiv.org)
Background Mitochondrial DNA (mtDNA) diseases are rare disorders whose prevalence is estimated around 1 in 5000. (bmj.com)
Methods We analysed the whole mtDNA in a cohort of 743 patients suspected of manifesting a mitochondrial disease, after excluding deletions and common mutations. (bmj.com)
The wide range of manifestations observed in patients with mitochondrial disease results from varying fractions of abnormal mtDNA molecules in different cells and tissues, a phenomenon termed heteroplasmy. (jci.org)
Here, we identify nonrandom distribution of a pathogenic mtDNA variant across a complex tissue using single-cell RNA-Seq, mitochondrial single-cell ATAC sequencing, and multimodal single-cell sequencing. (jci.org)

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

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

Molecular typing of Cyclospora cayetanensis in produce and clinical samples using targeted enrichment of complete mitochondrial genomes and next-generation sequencing. (cdc.gov)

Changes in protein phosphorylation among hibernation-specific metabolic stages do not correlate with mitochondrial respiration. (uwo.ca)
We also found, in separate studies, that microRNA (miRNA) expression in PBMo and mitochondrial respiration in peripheral blood mononuclear cells (PBMCs) differed in the IL-1ß/IL-10-based ASD subgroups. (frontiersin.org)
This study explored whether serum miRNAs are associated with both altered innate immune responses and changes in mitochondrial respiration as a link of regulatory mechanisms for these two common abnormalities in ASD subjects. (frontiersin.org)
Serum miRNA levels were examined by high-throughput deep sequencing in ASD and non-ASD control sera with concurrent measurement of PBMo cytokine production and mitochondrial respiration by PBMCs. (frontiersin.org)
ASD subgroup-specific associations were observed between serum miRNA expression and IL-1ß/IL-10 ratios, mitochondrial respiration, and monocyte cytokine profiles (IL-10, CCL2, and TNF-α). (frontiersin.org)
In summary, our results indicate that serum levels of select miRNAs may serve as promising biomarkers for screening and monitoring changes in innate immunity and mitochondrial respiration in ASD. (frontiersin.org)
My research during my post-doctoral training, which has continued during my faculty position, has been focused on using exercise and nutritional approaches to identify novel regulation in mitochondrial bioenergetics (aerobic respiration), and apply this knowledge to the study of various diseases. (uoguelph.ca)

Its role in the regulation of normal mitochondrial function and in different disease states attributed to mitochondrial dysfunction is not known. (nih.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)

Mitochondrial translation is crucial for maintaining mitochondrial function and mutations in this system lead to a breakdown in the respiratory chain-oxidative phosphorylation system and to impaired maintenance of mitochondrial DNA. (nih.gov)

A primary goal of our research is to both exploit and develop tools to understand mitochondrial homeostasis in health and disease. (helsinki.fi)
Alpha-Linolenic Acid Supplementation Prevents Exercise-Induced Improvements in White Adipose Tissue Mitochondrial Bioenergetics and Whole-Body Glucose Homeostasis in Obese Zucker Rats. (uoguelph.ca)

Fig. 4: Post-translational mechanisms governing respiratory control and the role of mitochondrial membrane dynamics. (nature.com)

Recent advances have made it possible to visualise mitophagy in mammalian tissues. (helsinki.fi)

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)
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)

The formation of this MAVS signaling complex is aided by augmented levels of mitochondrial reactive oxygen species (mROS), independent of the RNA sensing. (wikipedia.org)

Mitochondrial dysfunction is a common factor to many human diseases, however the intrinsic means cells employ to survive and ultimately recover from mitochondrial perturbations are only beginning to be understood. (umassmed.edu)
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)

Mutations of mitochondrial DNA can lead to a number of illnesses including exercise intolerance and Kearns-Sayre syndrome (KSS), which causes a person to lose full function of their heart, eye, and muscle movements. (dadamo.com)

Using three genetic models for NSUN2 inactivation-knockout mice, patient-derived fibroblasts and CRISPR/Cas9 knockout in human cells-we show that NSUN2 is necessary for the generation of m5C at positions 48, 49 and 50 of several mammalian mitochondrial tRNAs. (radygenomics.org)
Finally, we show that inactivation of NSUN2 does not have a profound effect on mitochondrial tRNA stability and oxidative phosphorylation in differentiated cells. (radygenomics.org)
Using this method, we examined the genomic positions of 6mA in bacteria ( E. coli ), green algae ( C. reinhardtii ), and mammalian cells (HEK239T, Huh7, and HeLa cells). (nature.com)
Here, we present a simplified metabolite extraction (SiMeEx) method, to efficiently and quickly extract metabolites from adherent mammalian cells. (frontiersin.org)
For adherent mammalian cells in culture the normal procedure involves their detachment from the surface. (frontiersin.org)
In this current study, we present a new and shorter method, termed as simplified metabolite extraction (SiMeEx), to extract metabolites from adherent mammalian cells. (frontiersin.org)
Consequently, our cells have evolved multiple mechanisms to cope with mitochondrial meltdown. (helsinki.fi)
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)
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)
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)
We profiled the transcriptome, chromatin accessibility state, and heteroplasmy in cells from the eyes of a patient with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) and from healthy control donors. (jci.org)
Figure 1 summarises the major steps of carbohydrate oxidation in mammalian cells. (bmj.com)

I lead a research group that focuses on mitochondrial DNA and the molecular mechanisms that mediate the signaling of its instability. (umu.se)
A thorough understanding of the molecular mechanisms that mediate these signals relates to various hereditary mitochondrial diseases, but also to neurodegenerative disease and various types of cancers. (umu.se)
Although the mechanisms underlying PD remain elusive, α-Synuclein (αSyn) accumulation and mitochondrial dysfunction have been recognized as major contributors [ 1 , 57 ]. (springer.com)

It is not surprising, therefore that mitochondrial damage and aberrant mitochondrial metabolism are associated with a diverse array of human diseases. (helsinki.fi)
Sodium Nitrate Supplementation Alters Mitochondrial H 2 O 2 Emission but Does Not Improve Mitochondrial Oxidative Metabolism in the Heart of Healthy Rats. (uoguelph.ca)
The term congenital lactic acidosis (CLA) refers to a group of inborn errors of mitochondrial metabolism variably characterised by progressive neuromuscular deterioration and accumulation of lactate and hydrogen ions in blood, urine and/or cerebrospinal fluid, frequently resulting in early death. (bmj.com)
Hyperlactataemia is the defining biochemical abnormality in children with CLA and, in the absence of hypoxia, should be considered a surrogate marker for underlying failure of mitochondrial energy metabolism. (bmj.com)

My research is primarily focused on understanding the regulation of mitochondrial bioenergetics, with a particular interest in studying fatty acid oxidation (breakdown of fat yielding energy) in skeletal and cardiac muscle. (uoguelph.ca)
Nutrition and Training Influences on the Regulation of Mitochondrial Adenosine Diphosphate Sensitivity and Bioenergetics. (uoguelph.ca)
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)
ATP-dependent Clp protease (ClpP), a mitochondrial matrix protease, plays an important role in regulating mitochondrial protein turnover and bioenergetics activity. (springer.com)

Fig. 1: Regulation of mitochondrial respiratory capacity. (nature.com)
Fig. 3: Translational control of mitochondrial respiratory chain assembly. (nature.com)
Sex-Differences in Mitochondrial Respiratory Function in Human Skeletal Muscle. (uoguelph.ca)
The team identified an allosteric inhibitory site buried inside mammalian mitochondrial heme-copper oxidases (mtHCOs), essential respiratory enzymes. (scienceboard.net)

This reaction is catalysed by PDC, a series of linked enzymes located in the inner mitochondrial membrane (fig 2 ). (bmj.com)
A total of 5 enzymes in 2 subcellular compartments (mitochondrial matrix and cytosol) convert ammonia into urea, which is excreted by the kidney (see image below). (medscape.com)

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)

Initiation factor 3 (IF3 mt ) promotes the dissociation of the 55S mitochondrial ribosome into subunits and may play additional, less-well-understood, roles in initiation complex formation. (elsevierpure.com)
Structures of the human mitochondrial ribosome bound to EF-G1 reveal distinct features of mitochondrial translation elongation. (nih.gov)

MtFAS is an essential pathway in mammalian skeletal myoblasts but does not contribute to synthesis of major cellular lipids. (elifesciences.org)
A ) Schematic of the mitochondrial fatty acid synthesis pathway and downstream lipoic acid synthesis. (elifesciences.org)

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)

This is accomplished in humans by sequencing one or more of the hypervariable control regions (HVR1 or HVR2) of the mitochondrial DNA. (dadamo.com)

Witkowski A, Thweatt J, Smith S. Mammalian ACSF3 protein is a malonyl-CoA synthetase that supplies the chain extender units for mitochondrial fatty acid synthesis. (medlineplus.gov)

Such maternally inherited differences in mitochondrial distribution are thought to play roles in defining the long-term viability of the blastomere in some cases and embryonic axes and patterning in others. (nih.gov)
The fact that mitochondrial DNA is maternally inherited enables researchers to trace uterine lineage far back in time. (dadamo.com)

Mitochondrial NAD + transporters have been identified in yeast and plants 1,2 , but their existence in mammals remains controversial 3-5 . (princeton.edu)

Can we apply strategies used by extremophiles to improve mammalian cell and tissue storage? (researchgate.net)

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)

This gene encodes one of the mitochondrial translation elongation factors. (nih.gov)

5 This concept is most readily appreciated by considering mitochondrial enzyme deficiencies. (bmj.com)
An inner mitochondrial membrane transporter directs ornithine to the transcarbamoylase enzyme to keep intramatrix ornithine levels low. (medscape.com)

Expression of human mitochondrial DNA is indispensable for proper function of the oxidative phosphorylation machinery. (radygenomics.org)
Binding of the human homolog of the Drosophila discs large tumor suppressor protein to the mitochondrial ribosomal protein MRP-S34. (nih.gov)
Going forward, we hope to gain further insight into the mitochondrial contribution to human pathology and aging, and ultimately, manipulate the UPR mt therapeutically. (umassmed.edu)
Age-Associated Impairments in Mitochondrial ADP Sensitivity Contribute to Redox Stress in Senescent Human Skeletal Muscle. (uoguelph.ca)

Substantial evidence has shown that αSyn toxicity may directly disrupt mitochondrial function. (springer.com)

This review summarizes the literature on mammalian toxicity of ZnO nanoparticles (NPs) published between 2009 and 2011. (dovepress.com)

B ) Crude isolated mitochondrial fractions from duplicate single cell clones of Mcat , Oxsm , and Mecr mutants, compared with GFP control clonal cell lines, were separated via SDS-PAGE and immunoblotted for the indicated targets. (elifesciences.org)

Whole cell lysate (WCL), post mitochondrial supernatant (PMS), and mitochondrial lysate (Mito) was isolated from each cell line indicated, normalized for total protein via BCA assay, and immunoblotted for Mecr or VDAC as a mitochondrial marker control. (elifesciences.org)

Mitochondrial ribosomes (mitoribosomes) consist of a small 28S subunit and a large 39S subunit. (nih.gov)
The 28S subunit of the mammalian mitoribosome may play a crucial and characteristic role in translation initiation. (thermofisher.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)

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)

Ischaemic accumulation of succinate controls reperfusion injury through mitochondrial ROS. (nature.com)
Both α-Synuclein (αSyn) accumulation and mitochondrial dysfunction have been implicated in the pathology of Parkinson's disease (PD). (springer.com)

The transporter was identified by probing a mammalian-expressed sequence tag database with 2 fungal mitochondrial ornithine carrier protein sequences. (medscape.com)
In hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, the mitochondrial ornithine transporter ORNT1 is defective. (medscape.com)
In hyperornithinemia-hyperammonemia-homocitrullinemia syndrome, the mitochondrial ornithine transporter ORNT1 is defective. (medscape.com)

Problems with mitochondrial DNA can therefore lead to a cellular energy defect and mitochondrial disease. (umu.se)

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)

Heteroplasmy and mosaicism are therefore important determinants of mitochondrial disease pathophysiology. (biorxiv.org)

Anatomy37

Organisms35

Chemicals and Drugs66

Analytical, Diagnostic and Therapeutic Techniques and Equipment32

Phenomena and Processes86

Disciplines and Occupations1

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Health Care2

Mitochondria20

Genome8

Proteins7

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Insights into mammalian1

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Genomes1

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Normal mitochondrial function2

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Homeostasis2

Role of mitochondrial1

Mitophagy1

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Dysfunction is a common2

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Humans1

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20201

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Heteroplasmy1