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Revolutionizing cellular energy: The convergence of mitochondrial dynamics and delivery technologies Mitochondrion (IF 4.4) Pub Date : 2024-03-17 Dilpreet Singh
The intersection of mitochondrial dynamics and delivery technologies heralds a paradigm shift in cellular biology and therapeutic intervention. Mitochondrial dynamics, encompassing fusion, fission, transport, and mitophagy, are critical for cellular energy production, signaling, and homeostasis. Dysregulation of these processes is implicated in a myriad of diseases, including neurodegenerative disorders
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Muscle-UCP3 in the regulation of energy metabolism Mitochondrion (IF 4.4) Pub Date : 2024-03-17 Lucio Della Guardia, Livio Luzi, Roberto Codella
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The deletion of ppr2 interferes iron sensing and leads to oxidative stress response in Schizosaccharomyces pombe Mitochondrion (IF 4.4) Pub Date : 2024-03-16 Zecheng Liu, Ting Jin, Bingxin Qin, Rongrong Li, Jinjie Shang, Ying Huang
Pentatricopeptide repeat proteins are involved in mitochondrial both transcriptional and posttranscriptional regulation. Ppr2 is a general mitochondrial translation factor that plays a critical role in the synthesis of all mitochondrial DNA-encoded oxidative phosphorylation subunits, which are essential for mitochondrial respiration. Our previous analysis showed that deletion resulted in increased
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Genetic heterogeneity and respiratory chain enzyme analysis in pediatric Indian patients with mitochondrial disorder: Report of novel variants in POLG1 gene and their functional implication using molecular dynamic simulation Mitochondrion (IF 4.4) Pub Date : 2024-03-11 Debolina Saha, Sonam Kothari, Shilpa Duttaprasanna Kulkarni, Menaka Thambiraja, Ragothaman M Yennamalli, Dhanjit K Das
Mitochondrial disorders are a heterogeneous group of disorders caused by mutations in the mitochondrial DNA or in nuclear genes encoding the mitochondrial proteins and subunits. Polymerase Gamma (POLG) is a nuclear gene and mutation in the POLG gene are one of the major causes of inherited mitochondrial disorders. In this study, 15 pediatric patients, with a wide spectrum of clinical phenotypes were
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Effects of chronic hydrogen peroxide exposure on mitochondrial oxidative stress genes, ROS production and lipid peroxidation in HL60 cells Mitochondrion (IF 4.4) Pub Date : 2024-03-11 M. Montserrat-Mesquida, M.D. Ferrer, A. Pons, A. Sureda, X. Capó
Hydrogen peroxide (HO) is a reactive species that is also involved in the redox regulation of cells because of it is relative stability. In numerous pathological situations, a chronic increase in the production of reactive species is observed, which is related to oxidative stress and cellular damage. This study aimed to evaluate the effects of long-term exposure to different HO concentrations on oxidative
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Involvement of mitochondria in Alzheimer’s disease pathogenesis and their potential as targets for phytotherapeutics Mitochondrion (IF 4.4) Pub Date : 2024-03-09 Swathi Maruthiyodan, Kamalesh Dattaram Mumbrekar, Kanive Parashiva Guruprasad
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Ancient mitogenomes suggest complex maternal history of one of the oldest settlements of western India Mitochondrion (IF 4.4) Pub Date : 2024-03-09 Bhavna Ahlawat, Lomous Kumar, Abhijit Ambekar, Jagmahender Singh Sehrawat, Yadubir Singh Rawat, Niraj Rai
The ancient township of Vadnagar tells a story of a long chain of cultural diversity and exchange. Vadnagar has been continuously habituated and shows a presence of rich cultural amalgamation and continuous momentary sequences between the 2th century BCE and present-day. Seven cultural periods developed a complex and enriched settlement at Vadnagar in spatio-temporality. Although archaeological studies
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Identification of a novel MT-ND3 variant and restoring mitochondrial function by allotopic expression of MT-ND3 gene Mitochondrion (IF 4.4) Pub Date : 2024-03-02 Nurun Nahar Borna, Yoshihito Kishita, Masaru Shimura, Kei Murayama, Akira Ohtake, Yasushi Okazaki
Mitochondrial diseases are caused by nuclear, or mitochondrial DNA (mtDNA) variants and related co-factors. Here, we report a novel m.10197G > C variant in in a patient, and two other patients with m.10191 T > C. variants are known to cause Leigh syndrome or mitochondrial complex I deficiency. We performed the functional analyses of the novel m.10197G > C variant that significantly lowered MT-ND3 protein
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Mitochondria in biology and medicine – 2023 Mitochondrion (IF 4.4) Pub Date : 2024-02-28 B. Disha, Rohan Peter Mathew, Ashwin B. Dalal, Ajay K. Mahato, Kapaettu Satyamoorthy, Keshav K. Singh, Kumarasamy Thangaraj, Periyasamy Govindaraj
Mitochondria are an indispensable part of the cell that plays a crucial role in regulating various signaling pathways, energy metabolism, cell differentiation, proliferation, and cell death. Since mitochondria have their own genetic material, they differ from their nuclear counterparts, and dysregulation is responsible for a broad spectrum of diseases. Mitochondrial dysfunction is associated with several
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Miro1 improves the exogenous engraftment efficiency and therapeutic potential of mitochondria transfer using Wharton's jelly mesenchymal stem cells Mitochondrion (IF 4.4) Pub Date : 2024-02-24 Yu-Han Lin, Kai-Lieh Lin, Xiao-Wen Wang, Jong-Jer Lee, Feng-Sheng Wang, Pei-Wen Wang, Min-Yu Lan, Chia-Wei Liou, Tsu-Kung Lin
Mitochondria are important for maintaining cellular energy metabolism and regulating cellular senescence. Mitochondrial DNA (mtDNA) encodes subunits of the OXPHOS complexes which are essential for cellular respiration and energy production. Meanwhile, mtDNA variants have been associated with the pathogenesis of neurodegenerative diseases, including MELAS, for which no effective treatment has been developed
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Mitochondrial functioning in Rheumatoid arthritis modulated by estrogen: Evidence-based insight into the sex-based influence on mitochondria and disease Mitochondrion (IF 4.4) Pub Date : 2024-02-24 Swati Malik, Debolina Chakraborty, Prachi Agnihotri, Alankrita Sharma, Sagarika Biswas
Alteration of immune response and synovium microvasculature in Rheumatoid arthritis (RA) progression has been suggested to be associated with mitochondrial functioning. Mitochondria, with maternally inherited DNA, exhibit differential response to the female hormone estrogen. Various epidemiological evidence has also shown the prominence of RA in the female population, depicting the role of estrogen
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Mitochondrial stress in the spaceflight environment Mitochondrion (IF 4.4) Pub Date : 2024-02-23 Agata M. Rudolf, Wendy R. Hood
Space is a challenging environment that deregulates individual homeostasis. The main external hazards associated with spaceflight include ionizing space radiation, microgravity, isolation and confinement, distance from Earth, and hostile environment. Characterizing the biological responses to spaceflight environment is essential to validate the health risks, and to develop effective protection strategies
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Host and microbial regulation of mitochondrial reactive oxygen species during mycobacterial infections Mitochondrion (IF 4.4) Pub Date : 2024-02-13 Jin Kyung Kim, Eun-Kyeong Jo
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Altered mitochondrial respiration in peripheral blood mononuclear cells of post-acute sequelae of SARS-CoV-2 infection Mitochondrion (IF 4.4) Pub Date : 2024-02-09 Sahera Dirajlal-Fargo, David P Maison, Jared C Durieux, Anastasia Andrukhiv, Nicholas Funderburg, Kate Ailstock, Mariana Gerschenson, Grace A Mccomsey
Peripheral blood mononuclear cells (PBMC) mitochondrial respiration was measured from participants without a history of COVID (n = 19), with a history of COVID and full recovery (n = 20), and with PASC (n = 20). Mean mitochondrial basal respiration, ATP-linked respiration, maximal respiration, spare respiration capacity, ATP-linked respiration, and non-mitochondrial respiration were highest in COVID + PASC+
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Interplay of mitochondria and diabetes: Unveiling novel therapeutic strategies Mitochondrion (IF 4.4) Pub Date : 2024-02-07 K, r, i, s, t, i, n, a, , X, i, a, o, , L, i, a, n, g
The interplay between mitochondrial function and diabetes has gained significant attention due to its crucial role in the pathogenesis and progression of the disease. Mitochondria, known as the cellular powerhouses, are essential for glucose metabolism. Dysfunction of these organelles has been implicated in the development of insulin resistance and beta-cell failure, both prominent features of diabetes
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RTN3 deficiency exacerbates cisplatin-induced acute kidney injury through the disruption of mitochondrial stability Mitochondrion (IF 4.4) Pub Date : 2024-02-07 Ran Du, Ji-Shi Liu, Hao Huang, Yu-Xing Liu, Jie-Yuan Jin, Chen-Yu Wang, Yi Dong, Liang-Liang Fan, Rong Xiang
Reticulum 3 (RTN3) is an endoplasmic reticulum (ER) protein that has been reported to act in neurodegenerative diseases and lipid metabolism. However, the role of RTN3 in acute kidney injury (AKI) has not been explored. Here, we employed public datasets, patient data, and animal models to explore the role of RTN3 in AKI. The underlying mechanisms were studied in primary renal tubular epithelial cells
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Decoding the nature and complexity of extracellular mtDNA: Types and implications for health and disease Mitochondrion (IF 4.4) Pub Date : 2024-01-20 Andrés Caicedo, Abigail Benavides-Almeida, Alissen Haro-Vinueza, José Peña-Cisneros, Álvaro A. Pérez-Meza, Jeremy Michelson, Sebastian Peñaherrera, Martin Picard
The mitochondrial DNA (mtDNA) is replicated and canonically functions within intracellular mitochondria, but recent discoveries reveal that the mtDNA has another exciting extracellular life. mtDNA fragments and mitochondria-containing vesicular structures are detected at high concentrations in cell-free forms, in different biofluids. Commonly referred to as cell-free mtDNA (cf-mtDNA), the field is
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Mitochondrial dynamics and mitochondrial autophagy: Molecular structure, orchestrating mechanism and related disorders Mitochondrion (IF 4.4) Pub Date : 2024-01-19 Haoran Wang, Wenjun Luo, Haoyu Chen, Zhiduan Cai, Guibin Xu
Mitochondrial dynamics and autophagy play essential roles in normal cellular physiological activities, while abnormal mitochondrial dynamics and mitochondrial autophagy can cause cancer and related disorders. Abnormal mitochondrial dynamics usually occur in parallel with mitochondrial autophagy. Both have been reported to have a synergistic effect and can therefore complement or inhibit each other
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DDQ anti-aging properties expressed with improved mitophagy in mutant tau HT22 neuronal cells Mitochondrion (IF 4.4) Pub Date : 2024-01-19 Jangampalli Adi Pradeepkiran, Priyanka Rawat, Arubala P. Reddy, Erika Orlov, P. Hemachandra Reddy
The purpose of our study is to develop age-related phosphorylated tau (p-tau) inhibitors, for Alzheimer’s disease (AD). There are wide-ranging therapeutic molecules available in the market and tested for age-related p-tau inhibition to enhance phosphatase activity and microtubule stability in AD neurons. Until now there are no such small molecules claimed to show promising results to delay the disease
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Research progress on mitochondrial damage and repairing in oocytes: A review Mitochondrion (IF 4.4) Pub Date : 2024-01-17 Zheqing Yang, Sitong Liu, Xiaoyan Pan
Oocytes are the female germ cells, which are susceptible to stress stimuli. The development of oocytes in the ovary is affected by many environmental and metabolic factors, food toxins, aging, and pathological factors. Mitochondria are the main target organelles of these factors, and the damage to mitochondrial structure and function can affect the production of ATP, the regulation of redox reactions
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Application research of novel peptide mitochondrial-targeted antioxidant SS-31 in mitigating mitochondrial dysfunction Mitochondrion (IF 4.4) Pub Date : 2024-01-17 Xinrong Du, Qin Zeng, Yunchang Luo, Libing He, Yuhong Zhao, Ninjing Li, Changli Han, Guohui Zhang, Weixin Liu
Due to the pivotal role of mitochondria in the generation of adenosine triphosphate (ATP) and the regulation of cellular homeostasis, mitochondrial dysfunction may exert a profound impact on various physiological systems, potentially precipitating a spectrum of distinct diseases. Consequently, research pertaining to mitochondrial therapeutics has assumed increasing significance, warranting heightened
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Whole genome sequencing followed by functional analysis of genomic deletion encompassing ERCC8 and NDUFAF2 genes in a non-consanguineous Indian family reveals dysfunctional mitochondrial bioenergetics leading to infant mortality Mitochondrion (IF 4.4) Pub Date : 2024-01-17 Ankit Sabharwal, Vishu Gupta, Shamsudheen KV, Ranjith Kumar Manokaran, Ankit Verma, Anushree Mishra, Rahul C. Bhoyar, Abhinav Jain, Ambily Sivadas, Sonali Rawat, Bani Jolly, Sujata Mohanty, Sheffali Gulati, Neerja Gupta, Madhulika Kabra, Vinod Scaria, Sridhar Sivasubbu
Genomic investigations on an infant who presented with a putative mitochondrial disorder led to identification of compound heterozygous deletion with an overlapping region of ∼142 kb encompassing two nuclear encoded genes namely and . Investigations on fetal-derived fibroblast culture demonstrated impaired bioenergetics and mitochondrial dysfunction, which explains the phenotype and observed infant
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Exploiting nuclear-mitochondrial cross-talk in theranostics: Enhancing drug delivery and diagnostic precision Mitochondrion (IF 4.4) Pub Date : 2023-12-28 D, i, l, p, r, e, e, t, , S, i, n, g, h
The dynamic interplay between nuclear and mitochondrial processes plays a pivotal role in cellular homeostasis and disease progression. Exploiting this nuclear-mitochondrial cross-talk has emerged as a promising avenue in the field of theranostics, offering enhanced drug delivery and diagnostic precision for a wide range of medical conditions, particularly cancer. This abstract provides a brief overview
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Mitochondrial density in skeletal and cardiac muscle Mitochondrion (IF 4.4) Pub Date : 2023-12-27 D, ., , P, e, s, t, a
Kubat et al. provide a review on the role Mitochondrial density in skeletal and cardiac muscle of mitochondrial dysfunction in muscle atrophy. They stress mitochondria's pivotal function, citing a 52 % density in skeletal muscle. However, the reference to Park et al.’s work misinterprets their findings. Park et al. report citrate synthase (CS) activity, indicating mitochondrial density as 222 ± 13 μmol
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Mitochondrial Mechanisms in Temozolomide Resistance: Unraveling the Complex Interplay and Therapeutic Strategies in Glioblastoma Mitochondrion (IF 4.4) Pub Date : 2023-12-27 Hao-Yi Li, Yin-Hsun Feng, Chien-Liang Lin, Tsung-I Hsu
Glioblastoma (GBM) is a highly aggressive and lethal brain tumor, with temozolomide (TMZ) being the standard chemotherapeutic agent for its treatment. However, TMZ resistance often develops, limiting its therapeutic efficacy and contributing to poor patient outcomes. Recent evidence highlights the crucial role of mitochondria in the development of TMZ resistance through various mechanisms, including
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The biology of mitochondrial carrier homolog 2 Mitochondrion (IF 4.4) Pub Date : 2023-12-27 Xiaohe Zheng, Binxiang Chu
The mitochondrial carrier system is in charge of small molecule transport between the mitochondria and the cytoplasm as well as being an integral portion of the core mitochondrial function. One member of the mitochondrial carrier family of proteins, mitochondrial carrier homolog 2 (MTCH2), is characterized as a critical mitochondrial outer membrane protein insertase participating in mitochondrial homeostasis
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Mitochondrial DNA variability and Covid-19 in the Slovak population Mitochondrion (IF 4.4) Pub Date : 2023-12-20 Gabriela Bľandová, Nikola Janoštiaková, Dominik Kodada, Michal Pastorek, Róbert Lipták, Július Hodosy, Katarína Šebeková, Peter Celec, Gabriela Krasňanská, Vladimír Eliaš, Lenka Wachsmannová, Michal Konečný, Vanda Repiská, Marian Baldovič
Recent studies have shown that mitochondria are involved in the pathogenesis of Covid-19. Mitochondria play a role in production of reactive oxygen species and induction of an innate immune response, both important during infections. Common variability of mitochondrial DNA (mtDNA) can affect oxidative phosphorylation and the risk or lethality of cardiovascular, neurodegenerative diseases and sepsis
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The maternal genetic origin and diversity of the extant populations of the Ladakh region in India Mitochondrion (IF 4.4) Pub Date : 2023-12-20 Lomous Kumar, Richa Rajpal, Bhavna Ahlawat, Jagmahender Singh Sehrawat, Sonam Spalzin, Ramnath Singh Fonia, Kumarasamy Thangaraj, Niraj Rai
Ladakh lies at a strategic location between the Indus River valley and the Hindu Khush Mountains, which makes the “Land of high passes” one of the major routes of movement. Through the years the region has faced multi-layered cultural movements, genetic assimilation and demographic changes. The initial settlement in the years goes back to the early Neolithic age and still continues despite its harsh
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CMT2A-linked MFN2 mutation, T206I promotes mitochondrial hyperfusion and predisposes cells towards mitophagy Mitochondrion (IF 4.4) Pub Date : 2023-12-12 Rajdeep Das, Sebabrata Maity, Palamou Das, Izaz Monir Kamal, Saikat Chakrabarti, Oishee Chakrabarti
Mutations in Mitofusin2 (MFN2) associated with the pathology of the debilitating neuropathy Charcot–Marie–Tooth type 2A (CMT2A) are known to alter mitochondrial morphology. Previously, such mutations have been shown to elicit two diametrically opposite phenotypes – while some mutations have been causally linked to enhanced mitochondrial fragmentation, others have been shown to induce hyperfusion. Our
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A sojourn on mitochondria targeted drug delivery systems for cancer: Strategies, clinical and future prospects Mitochondrion (IF 4.4) Pub Date : 2023-12-12 Dilpreet Singh
Mitochondria, often referred to as the powerhouses of the cell, have emerged as promising targets for cancer therapy due to their pivotal roles in cell survival, apoptosis, and energy metabolism. This sojourn emphasizes the significance of mitochondria-targeted drug delivery systems in cancer therapeutics. The unique characteristics of cancer cell mitochondria, such as altered membrane potential and
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Identifying the mitochondrial metabolism network by integration of machine learning and explainable artificial intelligence in skeletal muscle in type 2 diabetes Mitochondrion (IF 4.4) Pub Date : 2023-11-29 Kritika Sharma, Neeru Saini, Yasha Hasija
Imbalance in glucose metabolism and insulin resistance are two primary features of type 2 diabetes/diabetes mellitus. Its etiology is linked to mitochondrial dysfunction in skeletal muscle tissue. The mitochondria are vital organelles involved in ATP synthesis and metabolism. The underlying biological pathways leading to mitochondrial dysfunction in type 2 diabetes can help us understand the pathophysiology
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Understanding differential aspects of microdiffusion (channeling) in the Coenzyme Q and Cytochrome c regions of the mitochondrial respiratory system Mitochondrion (IF 4.4) Pub Date : 2023-11-30 Giorgio Lenaz, Salvatore Nesci, Maria Luisa Genova
Over the past decades, models of the organization of mitochondrial respiratory system have been controversial. The goal of this perspective is to assess this “conflict of models” by focusing on specific kinetic evidence in the two distinct segments of Coenzyme Q- and Cytochrome c-mediated electron transfer. Respiratory supercomplexes provide kinetic advantage by allowing a restricted diffusion of Coenzyme
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Role of F-actin-mediated endocytosis and exercise in mitochondrial transplantation in an experimental Parkinson's disease mouse model Mitochondrion (IF 4.4) Pub Date : 2023-11-29 Rachit Jain, Nusrat Begum, Shruti Rajan, Kamatham Pushpa Tryphena, Dharmendra Kumar Khatri
Dopaminergic neurons gradually deteriorate in Parkinson’s Disease (PD), which is characterized by the intracellular accumulation of Lewy bodies that are enriched with α-synuclein protein. Mitochondrial dysfunction is one of the primary contributors to this and is considered as the central player in the pathogenesis of PD. Recently, improving mitochondrial function has been extensively explored as a
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Assessment of mitochondrial DNA copy number variation relative to nuclear DNA quantity between different tissues Mitochondrion (IF 4.4) Pub Date : 2023-11-29 Jana Naue, Catarina Xavier, Steffen Hörer, Walther Parson, Sabine Lutz-Bonengel
Mitochondrial DNA is a widely tested genetic marker in various fields of research and diagnostics. Nonetheless, there is still little understanding on its abundance and quality within different tissues. Aiming to obtain deeper knowledge about the content and quality of mtDNA, we investigated nine tissues including blood, bone, brain, hair (root and shaft), cardiac muscle, liver, lung, skeletal muscle
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Effects of Humanin G (HNG) on angiogenesis and neurodegeneration markers in Age-related Macular Degeneration (AMD) Mitochondrion (IF 4.4) Pub Date : 2023-11-27 Sonali Nashine, M. Cristina Kenney
Advanced stages of Age-related Macular Degeneration (AMD) are characterized by retinal neurodegeneration and aberrant angiogenesis, and mitochondrial dysfunction contributes to the pathogenesis of AMD. In this study, we tested the hypothesis that Humanin G (HNG), a cytoprotective mitochondrial-derived peptide, positively regulates cell proliferation, cell death, and the protein levels of angiogenesis
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Mitochondrial DNA mutation pathogenicity score and neurocognitive performance in persons with HIV Mitochondrion (IF 4.4) Pub Date : 2023-11-19 Karen E. Volpe, David C. Samuels, Joanna L. Elson, Jannetta S. Steyn, Tebeb Gebretsadik, Ronald J. Ellis, Robert K. Heaton, Asha R. Kallianpur, Scott Letendre, Todd Hulgan
Background Mitochondrial DNA (mtDNA) genetic variation is associated with neurocognitive (NC) impairment (NCI) in people with HIV (PWH). Other approaches use sequence conservation and protein structure to predict the impact of mtDNA variants on protein function. We examined predicted mtDNA variant pathogenicity in the CHARTER study using MutPred scores, hypothesizing that persons with higher scores
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Oxidative stress is an essential factor for the induction of anhydrobiosis in the desiccation-tolerant midge, Polypedilum vanderplanki (Diptera, Chironomidae) Mitochondrion (IF 4.4) Pub Date : 2023-11-11 Richard Cornette, Hiroko P. Indo, Ken-ichi Iwata, Yuka Hagiwara-Komoda, Yuichi Nakahara, Oleg Gusev, Takahiro Kikawada, Takashi Okuda, Hideyuki J. Majima
The sleeping chironomid (Polypedilum vanderplanki) is the only insect capable of surviving complete desiccation in an ametabolic state called anhydrobiosis. Here, we focused on the role of oxidative stress and we observed the production of reactive oxygen species (ROS) in desiccating larvae and in those exposed to salinity stress. Oxidative stress occurs to some extent in desiccating larvae, inducing
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Mitochondrial bioenergetics and cytometric characterization of a synaptosomal preparation from mouse brain cortex Mitochondrion (IF 4.4) Pub Date : 2023-11-07 Paulina Lombardi, Analía G. Karadayian, Juan I. Guerra, Juanita Bustamante, Georgina Rodríguez de Lores Arnaiz, Silvia Lores-Arnaiz
Mitochondrial function at synapses can be assessed in isolated nerve terminals. Synaptosomes are structures obtained in vitro by detaching the nerve endings from neuronal bodies under controlled homogenization conditions. Several protocols have been described for the preparation of intact synaptosomal fractions. Herein a fast and economical method to obtain synaptosomes with optimal intrasynaptic mitochondria
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Reanalysis of mtDNA mutations of human primordial germ cells (PGCs) reveals NUMT contamination and suggests that selection in PGCs may be positive Mitochondrion (IF 4.4) Pub Date : 2023-10-30 Zoë Fleischmann, Auden Cote-L'Heureux, Melissa Franco, Sergey Oreshkov, Sofia Annis, Mark Khrapko, Dylan Aidlen, Konstantin Popadin, Dori C. Woods, Jonathan L. Tilly, Konstantin Khrapko
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Role of mitochondrial dysfunction in the pathogenesis of rheumatoid arthritis: Looking closely at fibroblast- like synoviocytes Mitochondrion (IF 4.4) Pub Date : 2023-10-30 Lakra Promila, Anubha Joshi, Shazia Khan, Amita Aggarwal, Amit Lahiri
Rheumatoid arthritis (RA) is a chronic, autoimmune, and inflammatory disease that primarily targets the joints, leading to cartilage and bone destruction. Fibroblast-like synoviocytes (FLS) are specialized cells of the synovial lining in the joint that plays a fundamental role in the development of RA. Particularly, FLS of RA patients (RA-FLS) in the joint exhibit specific characteristics like higher
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Mitochondrial dysfunction and inflammasome activation in neurodegenerative diseases: Mechanisms and therapeutic implications Mitochondrion (IF 4.4) Pub Date : 2023-10-30 Olia Hamzeh, Fatemeh Rabiei, Mahdi Shakeri, Hadi Parsian, Payam Saadat, Sahar Rostami-Mansoor
Impaired mitochondrial function is crucial to the pathogenesis of several neurodegenerative diseases. It causes the release of mitochondrial DNA (mtDNA), mitochondrial reactive oxygen species (mtROS), ATP, and cardiolipin, which activate the nucleotide-binding oligomerization domain (NOD)-like receptor protein 3 (NLRP3) inflammasome. NLRP3 inflammasome is an important innate immune system element contributing
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Complete mitochondrial DNA profile in stroke: A geographical matched case-control study in Spanish population Mitochondrion (IF 4.4) Pub Date : 2023-10-02 Ana Onieva, Joan Martin, Daniel R. Cuesta-Aguirre, Violeta Planells, Marta Coronado-Zamora, Katrin Beyer, Tomás Vega, José Eugenio Lozano, Cristina Santos, Maria Pilar Aluja
Introduction Stroke, the second leading cause of death worldwide, is a complex disease influenced by many risk factors among which we can find reactive oxygen species (ROS). Since mitochondria are the main producers of cellular ROS, nowadays studies are trying to elucidate the role of these organelles and its DNA (mtDNA) variation in stroke risk. The aim of the present study was to perform a comprehensive
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The constraints of allotopic expression Mitochondrion (IF 4.4) Pub Date : 2023-09-20 Felipe Nieto-Panqueva, Diana Rubalcava-Gracia, Patrice P. Hamel, Diego González-Halphen
Allotopic expression is the functional transfer of an organellar gene to the nucleus, followed by synthesis of the gene product in the cytosol and import into the appropriate organellar sub compartment. Here, we focus on mitochondrial genes encoding OXPHOS subunits that were naturally transferred to the nucleus, and critically review experimental evidence that claim their allotopic expression. We emphasize
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The Parkinson’s disease-associated mutation LRRK2 G2385R alters mitochondrial biogenesis via the PGC-1α-TFAM pathway Mitochondrion (IF 4.4) Pub Date : 2023-09-13 Jian Xue, Jinbao Zhang, Jinru Zhang, Junyi Liu, Fen Wang, Kai Li, Chunfeng Liu
Mutations in the Leucine-rich repeat protein kinase 2 (LRRK2) gene are the most frequent cause of familial Parkinson’s disease (PD). Although LRRK2 has been extensively studied, the pathogenic mechanism of the LRRK2 G2385R mutation, which is most common in Asian populations, especially in the Chinese Han population, remains unclear. In this study, we demonstrated that the LRRK2 G2385R mutation in HEK293T
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Understanding the neuronal synapse and challenges associated with the mitochondrial dysfunction in mild cognitive impairment and Alzheimer’s disease Mitochondrion (IF 4.4) Pub Date : 2023-09-13 Harkomal Verma, Prabhakar Gangwar, Anuradha Yadav, Bharti Yadav, Rashmi Rao, Sharanjot Kaur, Puneet Kumar, Monisha Dhiman, Giulio Taglialatela, Anil Kumar Mantha
Synaptic mitochondria are crucial for maintaining synaptic activity due to their high energy requirements, substantial calcium (Ca2+) fluctuation, and neurotransmitter release at the synapse. To provide a continuous energy supply, neurons use special mechanisms to transport and distribute healthy mitochondria to the synapse while eliminating the damaged mitochondria from the synapse. Along the neuron
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Hypoxia improves self-renew and migration of urine-derived stem cells by upregulating autophagy and mitochondrial function through ERK signal pathway Mitochondrion (IF 4.4) Pub Date : 2023-09-09 Chaoqun Hu, Yanting Sun, Wanxia Li, Yang Bi
Urine-derived stem cells (USCs) are autologous stem cells with self-renewal ability and multi-lineage differentiation potential. Our previous studies have shown that hypoxia preconditioning can improve self-renewal and migration abilities of USCs by up-regulating autophagy. The purpose of this study was to investigate the specific mechanism by which hypoxia treatment promotes the biological function
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Mitochondrial dysfunction characterized in human induced pluripotent stem cell disease models in MELAS syndrome: A brief summary Mitochondrion (IF 4.4) Pub Date : 2023-08-25 Kumarie Latchman, Mario Saporta, Carlos T. Moraes
Human induced pluripotent stem cells (hiPSCs) for MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, stroke-like episodes) may allow deeper understanding of how tissue-specific mitochondrial dysfunction result in multi-systemic disease. Here, we summarize how the m.3243G mtDNA mutation affects mitochondrial function in different tissues using iPSC and iPSC-differentiated cell
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Epilepsy: Mitochondrial connections to the ‘Sacred’ disease Mitochondrion (IF 4.4) Pub Date : 2023-08-13 Walter H. Moos, Douglas V. Faller, Ioannis P. Glavas, Iphigenia Kanara, Krishna Kodukula, Julie Pernokas, Mark Pernokas, Carl A. Pinkert, Whitney R. Powers, Konstantina Sampani, Kosta Steliou, Demetrios G. Vavvas
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What can we do to optimize mitochondrial transplantation therapy for myocardial ischemia–reperfusion injury? Mitochondrion (IF 4.4) Pub Date : 2023-08-06 Qian Liu, Meng Liu, Tianshu Yang, Xinting Wang, Peipei Cheng, Hua Zhou
Mitochondrial transplantation is a promising solution for the heart following ischemia–reperfusion injury due to its capacity to replace damaged mitochondria and restore cardiac function. However, many barriers (such as inadequate mitochondrial internalization, poor survival of transplanted mitochondria, few mitochondria colocalized with cardiac cells) compromise the replacement of injured mitochondria
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Molecular understanding of ER-MT communication dysfunction during neurodegeneration Mitochondrion (IF 4.4) Pub Date : 2023-07-24 Shivkumar S. Sammeta, Trupti A. Banarase, Sandip R. Rahangdale, Nitu L. Wankhede, Manish M. Aglawe, Brijesh G. Taksande, Shubhada V. Mangrulkar, Aman B. Upaganlawar, Sushruta Koppula, Spandana Rajendra Kopalli, Milind J. Umekar, Mayur B. Kale
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Type 1 diabetes mellitus: Inflammation, mitophagy, and mitochondrial function Mitochondrion (IF 4.4) Pub Date : 2023-07-13
Type 1 diabetes mellitus (T1DM) is a T-cell-mediated autoimmune disease characterized by the damage of insulin-secreting β-cells in the pancreatic islets of Langerhans. To date, its etiology is not fully understood, despite decades of active search for root causes, and that underlines the complexity of the disease pathogenesis. It was found that mitophagy plays a regulatory role in the development
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Mitochondrial respiratory complex I deficiency inhibits brown adipogenesis by limiting heme regulation of histone demethylation Mitochondrion (IF 4.4) Pub Date : 2023-07-13 Jingjing Liu, Wen Lu, Dongyue Yan, Junyuan Guo, Li Zhou, Bimin Shi, Xiong Su
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Mitochondrial dysfunction and skeletal muscle atrophy: Causes, mechanisms, and treatment strategies Mitochondrion (IF 4.4) Pub Date : 2023-07-13 Gokhan Burcin Kubat, Esmaa Bouhamida, Oner Ulger, Ibrahim Turkel, Gaia Pedriali, Daniela Ramaccini, Ozgur Ekinci, Berkay Ozerklig, Ozbeyen Atalay, Simone Patergnani, Beyza Nur Sahin, Giampaolo Morciano, Meltem Tuncer, Elena Tremoli, Paolo Pinton
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N-acetylcysteine modulates rotenone-induced mitochondrial Complex I dysfunction in THP-1 cells Mitochondrion (IF 4.4) Pub Date : 2023-07-05 Winston Tse-Hou Kwok, Haejin Angela Kwak, Ana Cristina Andreazza
Mitochondrial Complex I dysfunction and oxidative stress have been part of the pathophysiology of several diseases ranging from mitochondrial disease to chronic diseases such as diabetes, mood disorders and Parkinson’s Disease. Nonetheless, to investigate the potential of mitochondria-targeted therapeutic strategies for these conditions, there is a need further our understanding on how cells respond
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Maternal ancestry of first Parsi settlers of India using ancient mitogenome Mitochondrion (IF 4.4) Pub Date : 2023-06-26 Lomous Kumar, Bhavna Ahlawat, Sachin Kumar, Veena Mushrif-Tripathy, Niraj Rai
The rich cultural and genetic diversity of South Asia emerged from multiple migrations and cultural assimilation of multiple waves of migrants. The Parsi community of North-western India were one of those who migrated from West Eurasia in the aftermath of 7th century CE and assimilated into the local cultural framework. Earlier genetic studies further strengthened this notion with the finding that
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NUDT6 and NUDT9, two mitochondrial members of the NUDIX family, have distinct hydrolysis activities Mitochondrion (IF 4.4) Pub Date : 2023-06-19 Louis Debar, Layal Ishak, Amandine Moretton, Saber Anoosheh, Frederic Morel, Louise Jenninger, Isabelle Garreau-Balandier, Patrick Vernet, Anders Hofer, Siet van den Wildenberg, Geraldine Farge
The 22 members of the NUDIX (NUcleoside DIphosphate linked to another moiety, X) hydrolase superfamily can hydrolyze a variety of phosphorylated molecules including (d)NTPs and their oxidized forms, nucleotide sugars, capped mRNAs and dinucleotide coenzymes such as NADH and FADH. Beside this broad range of enzymatic substrates, the NUDIX proteins can also be found in different cellular compartments
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MOTS-c: A potential anti-pulmonary fibrosis factor derived by mitochondria Mitochondrion (IF 4.4) Pub Date : 2023-06-10 Zewei Zhang, Dongmei Chen, Kaili Du, Yaping Huang, Xingzhe Li, Quwen Li, Xiaoting Lv
Pulmonary fibrosis (PF) is a serious lung disease characterized by diffuse alveolitis and disruption of alveolar structure, with a poor prognosis and unclear etiopathogenesis. While ageing, oxidative stress, metabolic disorders, and mitochondrial dysfunction have been proposed as potential contributors to the development of PF, effective treatments for this condition remain elusive. However, Mitochondrial
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Corrigendum to “Prognostic value of circulating mitochondrial DNA in prostate cancer and underlying mechanism” [Mitochondrion 71 (2023) 40-49] Mitochondrion (IF 4.4) Pub Date : 2023-06-03 Supriya Borah, Rajeev Mishra, Sananda Dey, Surabhi Suchanti, Neil A Bhowmick, Biplab Giri, Subhash Haldar
Abstract not available