Mitochondrion最新文献_第2页

Research progress on paternal mitochondrial inheritance: An overview

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-02-28 DOI: 10.1016/j.mito.2025.102019

Wen Hu , Jiting Zhang , Zhaoqi Wu , Yi Wu , Yuhui Hu , Xiaohui Hu , Jinguo Cao

{"title":"Research progress on paternal mitochondrial inheritance: An overview","authors":"Wen Hu , Jiting Zhang , Zhaoqi Wu , Yi Wu , Yuhui Hu , Xiaohui Hu , Jinguo Cao","doi":"10.1016/j.mito.2025.102019","DOIUrl":"10.1016/j.mito.2025.102019","url":null,"abstract":"<div><div>Mitochondria are self-replicating organelles with their own DNA. They play a crucial role in biological, cellular and functional processes, such as energy production, metabolism, and signal transduction. Abnormal mitochondrial function can cause various diseases such as diabetes, tumour, Parkinson’s disease, hereditary optic neuropathy, and others. Although mitochondrial functions have been extensively and widely explored, studies on mitochondrial inheritance have been limited. Mitochondrial inheritance is traditionally thought to be maternal although small amounts of paternally transmitted mitochondria have been discovered on rare occasions, and the role of paternal mitochondria transmission to offspring has been largely ignored. This review highlights the present knowledge on mitochondrial inheritance, especially the controversy and the difficulties in investigating paternal mitochondrial inheritance. More significantly, we present a comprehensive description of the physiological functions of paternal mitochondria in children and discuss the animal model to explore the mechanism of paternal mitochondrial inheritance. This review may provide a theoretical and experimental basis for improving our understanding of paternal mitochondrial inheritance, and also provide new ideas for treating mitochondrial diseases.</div></div>","PeriodicalId":18606,"journal":{"name":"Mitochondrion","volume":"82 ","pages":"Article 102019"},"PeriodicalIF":3.9,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143537391","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Mitochondria in aging and age-associated diseases

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-02-27 DOI: 10.1016/j.mito.2025.102022

Sonu Pahal , Nirjal Mainali , Meenakshisundaram Balasubramaniam , Robert J. Shmookler Reis , Srinivas Ayyadevara

引用次数: 0

Transcription coupled repair occurrence in Trypanosoma cruzi mitochondria

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-02-22 DOI: 10.1016/j.mito.2025.102009

Bruno Marçal Repolês , Wesley Roger Rodrigues Ferreira , Antônio Vinicius de Assis , Isabela Cecília Mendes , Flávia Souza Morini , Camila Silva Gonçalves , Carolina Moura Costa Catta-Preta , Shana O. Kelley , Glória Regina Franco , Andrea Mara Macedo , Jeremy C. Mottram , Maria Cristina M. Motta , Stênio Perdigão Fragoso , Carlos Renato Machado

{"title":"Transcription coupled repair occurrence in Trypanosoma cruzi mitochondria","authors":"Bruno Marçal Repolês , Wesley Roger Rodrigues Ferreira , Antônio Vinicius de Assis , Isabela Cecília Mendes , Flávia Souza Morini , Camila Silva Gonçalves , Carolina Moura Costa Catta-Preta , Shana O. Kelley , Glória Regina Franco , Andrea Mara Macedo , Jeremy C. Mottram , Maria Cristina M. Motta , Stênio Perdigão Fragoso , Carlos Renato Machado","doi":"10.1016/j.mito.2025.102009","DOIUrl":"10.1016/j.mito.2025.102009","url":null,"abstract":"<div><div>Although several proteins involved in DNA repair systems have been identified in the <em>T. cruzi</em> mitochondrion, limited information is available regarding the specific DNA repair mechanisms responsible for kinetoplast DNA (kDNA) maintenance. The kDNA, contained within a single mitochondrion, exhibits a highly complex replication mechanism compared to the mitochondrial DNA of other eukaryotes. The absence of additional mitochondria makes the proper maintenance of this single mitochondrion essential for parasite viability.</div><div>Trypanosomatids possess a distinct set of proteins dedicated to kDNA organization and metabolism, known as kinetoplast-associated proteins (KAPs). Despite studies identifying the localization of these proteins, their functions remain largely unclear. Here, we demonstrate that TcKAP7 is involved in the repair of kDNA lesions induced by UV radiation and cisplatin. TcKAP7 mutant cells exhibited phenotypes similar to those observed in <em>Angomonas deanei</em> following the deletion of this gene. This monoxenic trypanosomatid colonizes the gastrointestinal tract of insects and possesses a kinetoplast with a distinct shape and kDNA topology compared to <em>T. cruzi</em>, making it a suitable comparative model in this study. Additionally, we observed that DNA damage can trigger distinct signaling pathways leading to cell death. Furthermore, we elucidated the involvement of CSB in this response, suggesting a potential interaction between TcKAP7 and CSB proteins in transcription-coupled DNA repair. The results presented here describe, for the first time, the mechanism of mitochondrial DNA repair in trypanosomatids following exposure to UV radiation and cisplatin.</div></div>","PeriodicalId":18606,"journal":{"name":"Mitochondrion","volume":"83 ","pages":"Article 102009"},"PeriodicalIF":3.9,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143492763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Large-scale screens identify a 19-Gene MitoScore for improved risk assessment in acute myeloid leukemia

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-02-19 DOI: 10.1016/j.mito.2025.102011

Liting Niu , Hanfei Guo , Yijing Zhao

{"title":"Large-scale screens identify a 19-Gene MitoScore for improved risk assessment in acute myeloid leukemia","authors":"Liting Niu , Hanfei Guo , Yijing Zhao","doi":"10.1016/j.mito.2025.102011","DOIUrl":"10.1016/j.mito.2025.102011","url":null,"abstract":"<div><h3>Background</h3><div>AML exhibits substantial molecular and genetic heterogeneity. Therefore, identifying key biological processes and related genes involved in the pathogenesis, as well as contributing to therapeutic resistance, is imperative for enhancing clinical outcomes. However, the assessment of mitochondrial function in AML has gradually been acknowledged but has not been widely emphasized. Hence, prioritizing the identification of mitochondrial-related biomarkers is crucial to enhance existing stratification methodologies and guide decisions on risk-adapted therapies.</div></div><div><h3>Methods</h3><div>We systematically integrated and analyzed data from nine online AML transcriptomics sequencing databases, screening the Human.MitoCarta3.0 mitochondrial gene database to identify AML-specific mitochondrial genes. A prognostic mitochondrial score was developed using LASSO regression analysis in the HOVON database as training cohort (n = 618) and validated in another eight publicly available independent cohorts (n = 1,697).</div></div><div><h3>Results</h3><div>A 19-mitochondrial function gene AML score was further generated and exhibited high prognostic power in 2,315 AML patients, named as MitoScore. MitoScore was an independent survival prognosis biomarker (p < 0.001). The MitoScore effectively distinguishes several genetic abnormalities and significantly improves the ELN (European Leukemia Net) classification. Patients with a high MitoScore demonstrated a notably poor response to induction chemotherapy and related refractory AML (p < 0.001). In the favorable risk gene variant and cytogenetic abnormality group, MitoScore was significantly lower compared to patients without those variants. Conversely, in the adverse group, MitoScore was significantly higher compared to patients with favorable genetic abnormalities.</div></div><div><h3>Conclusions</h3><div>Our findings underscore the utility of the MitoScore as a powerful tool for refined risk stratification and predicting chemotherapy resistance.</div></div>","PeriodicalId":18606,"journal":{"name":"Mitochondrion","volume":"82 ","pages":"Article 102011"},"PeriodicalIF":3.9,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143471230","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Endurance swimming exacerbates mitochondrial myopathy in mice with high mtDNA deletions

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-02-14 DOI: 10.1016/j.mito.2025.102010

Sho Hanada , Kaori Ishikawa , Takanaga Shirai , Tohru Takemasa , Kazuto Nakada

引用次数: 0

The unusual suspect: A novel role for intermediate filament proteins in mitochondrial morphology

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-02-03 DOI: 10.1016/j.mito.2025.102008

Irene M.G.M. Hemel , Carlijn Steen , Simon L.I.J. Denil , Gökhan Ertaylan , Martina Kutmon , Michiel Adriaens , Mike Gerards

引用次数: 0

Novel intronic variant in NDUFS7 gene results in mitochondrial complex I assembly defect with early basal ganglia and midbrain involvement with progressive neuroimaging findings

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-01-31 DOI: 10.1016/j.mito.2025.102007

Jaakko Oikarainen , Reetta Hinttala , Naemeh Nayebzadeh , Salla M. Kangas , Katariina Mankinen , Elisa Rahikkala , Hannaleena Kokkonen , Päivi Vieira , Maria Suo-Palosaari , Johanna Uusimaa

引用次数: 0

Sex-dependent adaptations in heart mitochondria from transgenic mice overexpressing cytochrome b5 reductase-3 过表达细胞色素b5还原酶-3的转基因小鼠心脏线粒体的性别依赖性适应。

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-01-09 DOI: 10.1016/j.mito.2025.102004

Luz Marina Sánchez-Mendoza , José A. González-Reyes , Sandra Rodríguez-López , Miguel Calvo-Rubio , Pilar Calero-Rodríguez , Rafael de Cabo , M. Isabel Burón , José M. Villalba

{"title":"Sex-dependent adaptations in heart mitochondria from transgenic mice overexpressing cytochrome b5 reductase-3","authors":"Luz Marina Sánchez-Mendoza , José A. González-Reyes , Sandra Rodríguez-López , Miguel Calvo-Rubio , Pilar Calero-Rodríguez , Rafael de Cabo , M. Isabel Burón , José M. Villalba","doi":"10.1016/j.mito.2025.102004","DOIUrl":"10.1016/j.mito.2025.102004","url":null,"abstract":"<div><h3>Summary</h3><div>Cytochrome <em>b</em><sub>5</sub> reductase 3 (CYB5R3) overexpression upregulates mitochondrial biogenesis, function, and abundance in skeletal muscle and kidneys, and mimics some of the salutary effects of calorie restriction, with the most striking effects being observed in females. We aimed to investigate the mitochondrial adaptations prompted by CYB5R3 overexpression in the heart, an organ surprisingly overlooked in studies focused on this long-lived transgenic model despite the critical role played by CYB5R3 in supporting cardiomyocytes mitochondrial respiration. Given that CYB5R3 effects have been found to be sex-dependent, we focused our research on both males and females. CYB5R3 was efficiently overexpressed in cardiac tissue from transgenic mice, without any difference between sexes. The abundance of electron transport chain complexes markers and cytochrome <em>c</em> was higher in males than in females. CYB5R3 overexpression downregulated the levels of complexes markers in males but not females, without decreasing oxygen consumption capacity. CYB5R3 increased the size and abundance of cardiomyocytes mitochondria, and reduced thickness and preserved the length of mitochondria-endoplasmic reticulum contact sites in heart from males but not females. Metabolic changes were also highlighted in transgenic mice, with an upregulation of fatty acid oxidation markers, particularly in males. Our results support that CYB5R3 overexpression upregulates markers consistent with enhanced mitochondrial function in the heart, producing most of these actions in males, with illustrates the complexity of the CYB5R3-overexpressing transgenic model.</div></div>","PeriodicalId":18606,"journal":{"name":"Mitochondrion","volume":"81 ","pages":"Article 102004"},"PeriodicalIF":3.9,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142965830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Yeast Dnm1G178R causes altered organelle dynamics and sheds light on the human DRP1G149R disease mechanism 酵母Dnm1G178R引起细胞器动力学改变，揭示了人类DRP1G149R疾病机制。

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-01-07 DOI: 10.1016/j.mito.2025.102006

Ankita Adhikary, Vivian Francis Joseph, Riddhi Banerjee , Shirisha Nagotu

引用次数: 0

Reactive oxygen species favors Varicellovirus bovinealpha 5 (BoAHV-5) replication in neural cells 活性氧有利于牛痘病毒5 （BoAHV-5）在神经细胞中的复制。

IF 3.9 3区生物学

Mitochondrion Pub Date : 2025-01-06 DOI: 10.1016/j.mito.2025.102005

Juan José Rosales , María Belén Brunner , Marcelo Rodríguez , Maia Marin , Eduardo Néstor Maldonado , Sandra Pérez

{"title":"Reactive oxygen species favors Varicellovirus bovinealpha 5 (BoAHV-5) replication in neural cells","authors":"Juan José Rosales , María Belén Brunner , Marcelo Rodríguez , Maia Marin , Eduardo Néstor Maldonado , Sandra Pérez","doi":"10.1016/j.mito.2025.102005","DOIUrl":"10.1016/j.mito.2025.102005","url":null,"abstract":"<div><div><em>Varicellovirus bovinealpha</em> (BoAHV) 1 and 5 are closely related neurotropic alphaherpesviruses with distinct neuropathogenic potential. BoAHV-5 causes meningoencephalitis in calves whereas encephalitis by BoAHV-1 infection is sporadic. the mechanisms underlying the differences in tropism and clinical outcomes of the infections are not yet completely understood. Here, we used neuroblastoma SH-SY5Y cells as non-differentiated in comparison with the SH-SY5Y neuronal-like cells obtained after exposing SH-SY5Y undifferentiated cells to <em>trans</em>-retinoic acid. We aimed to establish whether there was a relationship between the production of reactive oxygen species (ROS) and the kinetics of virus replication. We demonstrated that ROS production after BoAHV infection was higher in differentiated cells. Generation of ROS was also dependent on the infecting BoAHV strain. Higher ROS levels were produced during BoAHV-5 infection concomitantly with enhanced viral replication. We propose that increased ROS production mechanistically contributes to the tissue damage and neuroinflammation induced by BoAHV-5 infection. Future studies will determine specific targets of ROS that mediate the effects on viral replication.</div></div>","PeriodicalId":18606,"journal":{"name":"Mitochondrion","volume":"81 ","pages":"Article 102005"},"PeriodicalIF":3.9,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142951500","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0