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16+ results
Field: Mitochondrial Function and Pathology

Disorders of carnitine transport and the carnitine cycle

Verified

Nicola Longo, Cristina Amat Di San Filippo, Marzia Pasquali

Journal: American Journal of Medical Genetics Part C Seminars in Medical Genetics
Year: 2006
Citations: 540

Carnitine plays an essential role in the transfer of long-chain fatty acids across the inner mitochondrial membrane. This transfer requires enzymes and transporters that accumulate carnitine within the cell (OCTN2 carnitine transporter), conjugate it with long chain fatty acids (carnitine palmitoyl ...

Life SciencesBiochemistry, Genetics and Molecular BiologyClinical BiochemistryOpen Access
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Function of the<scp>SIRT</scp>3 mitochondrial deacetylase in cellular physiology, cancer, and neurodegenerative disease

Verified

Aneesa Ansari, Md. Shahedur Rahman, Subbroto Kumar Saha, Forhad Karim Saikot et al.

Journal: Aging CellYear: 2016Citations: 299

In mammals, seven members of the sirtuin protein family known as class III histone deacetylase have been identified for their characteristic features. These distinguished characteristics include the tissues where they are distributed or located, enzymatic activities, molecular functions, and involve...

Health SciencesMedicineGeriatrics and GerontologyOpen Access
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Nutraceuticals in Neurological Disorders

Verified

Rashita Makkar, Tapan Behl, Simona Bungău, Gökhan Zengin et al.

Journal: International Journal of Molecular SciencesYear: 2020Citations: 174

Neurological diseases are one of the major healthcare issues worldwide. Posed lifestyle changes are associated with drastically increased risk of chronic illness and diseases, posing a substantial healthcare and financial burden to society globally. Researchers aim to provide fine treatment for aili...

Life SciencesBiochemistry, Genetics and Molecular BiologyMolecular BiologyOpen Access
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Evidence of myofibrillar protein oxidation induced by postischemic reperfusion in isolated rat hearts

Verified

Marcella Canton, Irina Neverova, Roberta Menabò, Jennifer E. Van Eyk et al.

Journal: American Journal of Physiology-Heart and Circulatory PhysiologyYear: 2004Citations: 149

Although the contribution of reactive oxygen species to myocardial ischemia is well recognized, the possible intracellular targets, especially at the level of myofibrillar proteins (MP), are not yet fully characterized. To assess the maximal extent of oxidative degradation of proteins, isolated rat ...

Health SciencesMedicinePathology and Forensic Medicine
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FOXO1-mediated upregulation of pyruvate dehydrogenase kinase-4 (PDK4) decreases glucose oxidation and impairs right ventricular function in pulmonary hypertension: therapeutic benefits of dichloroacetate

Verified

Lin Piao, Vaninder K. Sidhu, Yong-Hu Fang, John Ryan et al.

Journal: Journal of Molecular MedicineYear: 2012Citations: 147

Pyruvate dehydrogenase kinase (PDK) is activated in right ventricular hypertrophy (RVH), causing an increase in glycolysis relative to glucose oxidation that impairs right ventricular function. The stimulus for PDK upregulation, its isoform specificity, and the long-term effects of PDK inhibition ar...

Life SciencesBiochemistry, Genetics and Molecular BiologyMolecular BiologyOpen Access
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Glutaric acidemia type 1

Verified

Gary L. Hedlund, Nicola Longo, Marzia Pasquali

Journal: American Journal of Medical Genetics Part C Seminars in Medical GeneticsYear: 2006Citations: 129

Glutaric acidemias comprise different disorders resulting in an increased urinary excretion of glutaric acid. Glutaric acidemia type 1 (GA-1) is an autosomal recessive disorder of lysine, hydroxylysine, and tryptophan metabolism caused by deficiency of glutaryl-CoA dehydrogenase. It results in the a...

Life SciencesBiochemistry, Genetics and Molecular BiologyClinical BiochemistryOpen Access
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Genetic alterations in Krebs cycle and its impact on cancer pathogenesis

Verified

Karishma Sajnani, Farhadul Islam, Robert A. Smith, Vinod Gopalan et al.

Journal: BiochimieYear: 2017Citations: 125

Cancer cells exhibit alterations in many cellular processes, including oxygen sensing and energy metabolism. Glycolysis in non-oxygen condition is the main energy production process in cancer rather than mitochondrial respiration as in benign cells. Genetic and epigenetic alterations of Krebs cycle ...

Life SciencesBiochemistry, Genetics and Molecular BiologyCancer Research
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A nanoscale, multi-parametric flow cytometry-based platform to study mitochondrial heterogeneity and mitochondrial DNA dynamics

Verified

J. Macdonald, Alisha M. Bothun, Sofia Annis, Hannah C. Sheehan et al.

Journal: Communications BiologyYear: 2019Citations: 114

Mitochondria are well-characterized regarding their function in both energy production and regulation of cell death; however, the heterogeneity that exists within mitochondrial populations is poorly understood. Typically analyzed as pooled samples comprised of millions of individual mitochondria, th...

Life SciencesBiochemistry, Genetics and Molecular BiologyMolecular BiologyOpen Access
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Axle-Less F <sub>1</sub> -ATPase Rotates in the Correct Direction

Verified

Shou Furuike, Mohammad Delawar Hossain, Yasushi Maki, Kengo Adachi et al.

Journal: ScienceYear: 2008Citations: 114

F1-adenosine triphosphatase (ATPase) is an ATP-driven rotary molecular motor in which the central gamma subunit rotates inside a cylinder made of three alpha and three beta subunits alternately arranged. The rotor shaft, an antiparallel alpha-helical coiled coil of the amino and carboxyl termini of ...

Life SciencesBiochemistry, Genetics and Molecular BiologyMolecular Biology
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Silver nanoparticles modulate mitochondrial dynamics and biogenesis in HepG2 cells

Verified

Jiangyan Li, Bangyong Zhang, Xiaoru Chang, Junying Gan et al.

Journal: Environmental PollutionYear: 2019Citations: 110

Silver nanoparticles (AgNPs) are inevitably released into the environment owing to their widespread applications in industry and medicine. The potential of their toxicity has aroused a great concern. Previous studies have shown that AgNPs exposure in HepG2 cells is primarily related to the damage of...

Life SciencesBiochemistry, Genetics and Molecular BiologyMolecular Biology
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Mitophagy-related regulated cell death: molecular mechanisms and disease implications

Verified

Molin Yang, Xiang Wei, Xin Yi, Ding‐Sheng Jiang

Journal: Cell Death and DiseaseYear: 2024Citations: 96

During oxidative phosphorylation, mitochondria continuously produce reactive oxygen species (ROS), and untimely ROS clearance can subject mitochondria to oxidative stress, ultimately resulting in mitochondrial damage. Mitophagy is essential for maintaining cellular mitochondrial quality control and ...

Health SciencesMedicineEpidemiologyOpen Access
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Lathyrism: a neurotoxic disease.

Verified

Peter S. Spencer, Herbert H. Schaumburg

Journal: PubMedYear: 1984Citations: 93

Lathyrism, one of the oldest neurotoxic diseases known to Man, results from excessive consumption of the chickling pea, Lathyrus sativus, and certain related species. Once prevalent throughout Europe, N. Africa, Middle East and parts of the Far East, the disease is presently restricted to India, Ban...

Life SciencesNeuroscienceNeurology
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Role of mitochondria-endoplasmic reticulum coupling in lycopene preventing DEHP-induced hepatotoxicity

Verified

Yi Zhao, Jia-Gen Cui, Hao Zhang, Xue‐Nan Li et al.

Journal: Food & FunctionYear: 2021Citations: 71

), but LYC alleviated these alterations. Therefore, LYC prevented DEHP-induced hepatic mitochondrial dynamics and MAM disorder, leading to ER stress. The present study provides novel evidence of mitochondria-ER coupling as a target for LYC that prevents DEHP-induced hepatotoxicity.

Health SciencesMedicineBiochemistry
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mRNA localization, reaction centre biogenesis and thylakoid membrane targeting in cyanobacteria

Verified

Moontaha Mahbub, Luisa Hemm, Yuxiao Yang, Ramanpreet Kaur et al.

Journal: Nature PlantsYear: 2020Citations: 70

The thylakoid membranes of cyanobacteria form a complex intracellular membrane system with a distinctive proteome. The sites of biogenesis of thylakoid proteins remain uncertain, as do the signals that direct thylakoid membrane-integral proteins to the thylakoids rather than to the plasma membrane. ...

Life SciencesBiochemistry, Genetics and Molecular BiologyMolecular Biology
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Multifarious roles of <scp>mTOR</scp> signaling in cognitive aging and cerebrovascular dysfunction of Alzheimer's disease

Verified

Md. Sahab Uddin, Md. Ataur Rahman, Md. Tanvir Kabir, Tapan Behl et al.

Journal: IUBMB LifeYear: 2020Citations: 68

Age-related cognitive failure is a main devastating incident affecting even healthy people. Alzheimer's disease (AD) is the utmost common form of dementia among the geriatric community. In the pathogenesis of AD, cerebrovascular dysfunction is revealed before the beginning of the cognitive decline. ...

Life SciencesBiochemistry, Genetics and Molecular BiologyBiochemistryOpen Access
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