Articles with "mitochondrial complex" as a keyword



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Hepatic Suppression of Mitochondrial Complex II Assembly Drives Systemic Metabolic Benefits

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Published in 2022 at "Advanced Science"

DOI: 10.1002/advs.202105587

Abstract: Abstract Alternate day fasting (ADF), the most popular form of caloric restriction, has shown to improve metabolic health in preclinical subjects, while intrinsic network underpinning the process remains unclear. Here, it is found that liver… read more here.

Keywords: complex assembly; hepatic suppression; mitochondrial complex; assembly ... See more keywords
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Genome sequencing and RNA‐seq analyses of mitochondrial complex I deficiency revealed Alu insertion‐mediated deletion in NDUFV2

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Published in 2021 at "Human Mutation"

DOI: 10.1002/humu.24274

Abstract: Isolated complex I deficiency is the most common cause of pediatric mitochondrial disease. Exome sequencing (ES) has revealed many complex I causative genes. However, there are limitations associated with identifying causative genes by ES analysis.… read more here.

Keywords: deletion ndufv2; complex deficiency; genome sequencing; mitochondrial complex ... See more keywords
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A homozygous splice variant in ATP5PO, disrupts mitochondrial complex V function and causes Leigh syndrome in two unrelated families

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Published in 2022 at "Journal of Inherited Metabolic Disease"

DOI: 10.1002/jimd.12526

Abstract: Mitochondrial complex V plays an important role in oxidative phosphorylation by catalyzing the generation of ATP. Most complex V subunits are nuclear encoded and not yet associated with recognized Mendelian disorders. Using exome sequencing, we… read more here.

Keywords: mitochondrial complex; variant atp5po; homozygous splice; atp5po ... See more keywords
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Disruption of Mitochondrial Complex I Induces Progressive Parkinsonism

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Published in 2022 at "Movement Disorders"

DOI: 10.1002/mds.28961

Abstract: Mitochondrial dysfunction is recognized as being an important mechanism through which Parkinson’s disease (PD) can arise. In particular, decrease of mitochondrial complex 1 (MCI) activity in dopaminergic neurons of the substantia nigra (SN) has been… read more here.

Keywords: mitochondrial complex; mci; parkinsonism; dopaminergic neurons ... See more keywords
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Degradation of the mitochondrial complex I assembly factor TMEM126B under chronic hypoxia

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Published in 2018 at "Cellular and Molecular Life Sciences"

DOI: 10.1007/s00018-018-2779-y

Abstract: Cell stress such as hypoxia elicits adaptive responses, also on the level of mitochondria, and in part is mediated by the hypoxia-inducible factor (HIF) 1α. Adaptation of mitochondria towards acute hypoxic conditions is reasonably well… read more here.

Keywords: chronic hypoxia; factor; assembly; hypoxia ... See more keywords
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NDUFB8 Mutations Cause Mitochondrial Complex I Deficiency in Individuals with Leigh-like Encephalomyopathy.

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Published in 2018 at "American journal of human genetics"

DOI: 10.1016/j.ajhg.2018.01.008

Abstract: Respiratory chain complex I deficiency is the most frequently identified biochemical defect in childhood mitochondrial diseases. Clinical symptoms range from fatal infantile lactic acidosis to Leigh syndrome and other encephalomyopathies or cardiomyopathies. To date, disease-causing… read more here.

Keywords: cause mitochondrial; ndufb8 mutations; complex deficiency; mutations cause ... See more keywords
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Anti-aging effects of chlorpropamide depend on mitochondrial complex-II and the production of mitochondrial reactive oxygen species

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Published in 2021 at "Acta Pharmaceutica Sinica B"

DOI: 10.1016/j.apsb.2021.08.007

Abstract: Abstract Sulfonylureas are widely used oral anti-diabetic drugs. However, its long-term usage effects on patients’ lifespan remain controversial, with no reports of influence on animal longevity. Hence, the anti-aging effects of chlorpropamide along with glimepiride,… read more here.

Keywords: reactive oxygen; aging effects; mitochondrial reactive; mitochondrial complex ... See more keywords
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An Essential Role for ECSIT in Mitochondrial Complex I Assembly and Mitophagy in Macrophages

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Published in 2018 at "Cell reports"

DOI: 10.1016/j.celrep.2018.02.051

Abstract: SUMMARY ECSIT is a mitochondrial complex I (CI)-associated protein that has been shown to regulate the production of mitochondrial reactive oxygen species (mROS) following engagement of Toll-like receptors (TLRs). We have generated an Ecsit conditional… read more here.

Keywords: ecsit mitochondrial; ecsit; mitochondrial complex; role ecsit ... See more keywords
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Dissecting the Roles of Mitochondrial Complex I Intermediate Assembly Complex Factors in the Biogenesis of Complex I.

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Published in 2020 at "Cell reports"

DOI: 10.1016/j.celrep.2020.107541

Abstract: Mitochondrial complex I harbors 7 mitochondrial and 38 nuclear-encoded subunits. Its biogenesis requires the assembly and integration of distinct intermediate modules, mediated by numerous assembly factors. The mitochondrial complex I intermediate assembly (MCIA) complex, containing… read more here.

Keywords: assembly; complex intermediate; mcia complex; mitochondrial complex ... See more keywords
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Microglial activation and the nitric oxide/cGMP/PKG pathway underlie enhanced neuronal vulnerability to mitochondrial dysfunction in experimental multiple sclerosis

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Published in 2018 at "Neurobiology of Disease"

DOI: 10.1016/j.nbd.2018.01.002

Abstract: During multiple sclerosis (MS), a close link has been demonstrated to occur between inflammation and neuro-axonal degeneration, leading to the hypothesis that immune mechanisms may promote neurodegeneration, leading to irreversible disease progression. Energy deficits and… read more here.

Keywords: vulnerability; multiple sclerosis; mitochondrial dysfunction; nitric oxide ... See more keywords
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Mitochondrial complex I deactivation is related to superoxide production in acute hypoxia

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Published in 2017 at "Redox Biology"

DOI: 10.1016/j.redox.2017.04.025

Abstract: Mitochondria use oxygen as the final acceptor of the respiratory chain, but its incomplete reduction can also produce reactive oxygen species (ROS), especially superoxide. Acute hypoxia produces a superoxide burst in different cell types, but… read more here.

Keywords: production; deactivation related; hypoxia; acute hypoxia ... See more keywords