Article
Multidisciplinary Sciences
Benjamin Cartes-Saavedra, Daniel Lagos, Josefa Macuada, Duxan Arancibia, Florence Burte, Marcela K. Sjoberg-Herrera, Maria Estela Andres, Rita Horvath, Patrick Yu-Wai-Man, Gyorgy Hajnoczky, Veronica Eisnera
Summary: The fusion and shape of the inner mitochondrial membrane rely on the protein OPA1. Mutations in OPA1 cause autosomal dominant optic atrophy (ADOA), a significant cause of inherited blindness. The specific roles of the GTPase and GED domains of OPA1 in mitochondrial fusion and cristae shape are still unclear.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2023)
Article
Cell Biology
Benjamin Cartes-Saavedra, Josefa Macuada, Daniel Lagos, Duxan Arancibia, Maria E. Andres, Patrick Yu-Wai-Man, Gyoergy Hajnoczky, Veronica Eisner
Summary: Autosomal Dominant Optic Atrophy (ADOA), caused by OPA1 mutations, affects Ca2+ homeostasis by modulating ER-mitochondria coupling, potentially contributing to the progression of ADOA.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Rosanna Chianese, Riccardo Pierantoni
Summary: In addition to ATP production, mitochondria play key roles in various cellular functions. Although sperm mitochondria are destroyed inside the zygote, their integrity and functionality are critical for fertilization and embryo development.
Article
Cell Biology
Xiao-Hui Zhang, Yue Xie, Quan-Gang Xu, Kai Cao, Ke Xu, Zi-Bing Jin, Yang Li, Shi-Hui Wei
Summary: The study found that approximately 46.8% of patients with Ethambutol-induced optic neuropathy (EON) had mitochondrial mutations, with OPA1 mutations and LHON-mtDNA mutations being the most common. Patients with mitochondrial mutations were younger, more likely to have optic disc hyperemia, and had a poorer visual prognosis.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Review
Cell Biology
Robert Gilkerson, Patrick De la Torre, Shaynah St. Vallier
Summary: Mammalian mitochondria play a crucial role in cellular stress response and development outcomes, responding to stimuli and stresses through structural dynamics. Fusion and fission of mitochondria, regulated by OPA1, are essential for maintaining organelle structure and function. The proteolytic mechanism involving OMA1 provides sensitive regulation of mitochondrial structure/function and is implicated in apoptotic pathways and cellular stress response.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Pharmacology & Pharmacy
Zhen Xing Gao, Ze Long Cui, Min Ran Zhou, Yue Fu, Fen Liu, Lu Zhang, Sai Ma, Chun Yan Chen
Summary: This study identified BAM15 as a novel mitochondrial uncoupling agent that effectively inhibits AML cell activity, while being less cytotoxic to normal cells. BAM15 can also enhance the anti-cancer activity of cytarabine when used in combination. This discovery provides a potential drug candidate for the treatment of AML.
BIOCHEMICAL PHARMACOLOGY
(2022)
Article
Agriculture, Multidisciplinary
Bo Zou, Qianqian Yu, Lele Shao, Yingying Sun, Xingmin Li, Ruitong Dai
Summary: Lipid molecules play a crucial role in mitochondria-mediated apoptosis. This study reveals that the levels of mitochondrial lipids increase during early postmortem, accelerating apoptosis in beef muscle. The psoas major muscle has a higher degree of apoptosis and more severe muscle oxidation and antioxidant system damage compared to the longissimus lumborum muscle.
JOURNAL OF AGRICULTURAL AND FOOD CHEMISTRY
(2022)
Article
Cell Biology
Rabia Ramzan, Amalia M. Dolga, Susanne Michels, Petra Weber, Carsten Culmsee, Ardawan J. Rastan, Sebastian Vogt
Summary: This study investigates the effects of ATP inhibition on cytochrome c oxidase (CytOx) in isolated rat heart mitochondria. The findings suggest that inhibiting CytOx activity with ATP leads to static respiration and decreased ROS production, highlighting the importance of ATP/ADP ratio for mitochondrial function.
Article
Biochemistry & Molecular Biology
Miji Kim, Garam An, Whasun Lim, Gwonhwa Song
Summary: This study investigated the toxicological effects of FPMH on porcine trophectoderm and uterine luminal epithelial cells. FPMH induced cytotoxic effects on the cells, affecting their functionality.
PESTICIDE BIOCHEMISTRY AND PHYSIOLOGY
(2022)
Article
Chemistry, Medicinal
Hui He, Chengqiang Wang, Gen Liu, Haoyue Ma, Mingdong Jiang, Pan Li, Qianwei Lu, Li Li, Hongyi Qi
Summary: The study demonstrates that Isobavachalcone induces apoptosis and differentiation of acute myeloid leukemia cells through multiple pathways, with the ROS-mediated signaling pathway playing a key role.
PHYTOTHERAPY RESEARCH
(2021)
Review
Multidisciplinary Sciences
Xueying Zhang, Claire D. Eliasberg, Scott A. Rodeo
Summary: Tendinopathy is a common musculoskeletal condition characterized by pain, swelling, and impaired function, affecting a wide range of patients. The multifactorial etiology of tendinopathy includes intrinsic and extrinsic mechanisms. Mitochondrial dysfunction, verified by increased ROS and decreased SOD activity, has been identified as a potential mechanism contributing to tendinopathy, providing new insights and potential therapeutic targets.
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES
(2021)
Article
Medicine, Research & Experimental
Mario K. Shammas, Xiaoping Huang, Beverly P. Wu, Evelyn Fessler, Insung Y. Song, Nicholas P. Randolph, Yan Li, Christopher K. E. Bleck, Danielle A. Springer, Carl Fratter, Ines A. Barbosa, Andrew F. Powers, Pedro M. Quiros, Carlos Lopez-Otin, Lucas T. Jae, Joanna Poulton, Derek P. Narendra
Summary: Mitochondrial stress triggers responses in both cellular mitochondria and nucleus. This study focuses on the impact of a mutation in the mitochondrial protein CHCHD10, which causes myopathy. The study finds that the mutated CHCHD10 aggregates in affected tissues, causing toxic stress on the inner mitochondrial membrane. The research also reveals the crucial role of the mitochondrial metalloendopeptidase OMA1 in coordinating stress responses and reshaping the mitochondrial network and proteome.
JOURNAL OF CLINICAL INVESTIGATION
(2022)
Article
Biochemistry & Molecular Biology
Yifan Ge, Sivakumar Boopathy, Tran H. Nguyen, Camila Makhlouta Lugo, Luke H. Chao
Summary: This study demonstrates the crucial role of cardiolipin in mitochondrial inner membrane fusion, especially in the case of its asymmetric distribution, which significantly impacts membrane tethering and fusion processes.
FRONTIERS IN MOLECULAR BIOSCIENCES
(2021)
Article
Microbiology
Guoxing Zhu, Shu Chen, Yuanwei Zhang, Ling Lu
Summary: Azole resistance is a major threat to global health caused by the human fungal pathogen Aspergillus fumigatus. While mutations in the cyp51A gene have been linked to azole resistance, there is an increasing recognition of azole resistance resulting from non-cyp51A mutations. In this study, a mutation in the mitochondrial protein Mba1 was found to confer multidrug resistance in azole-resistant isolates, highlighting the role of non-cyp51A proteins in driving drug resistance mechanisms.
ANTIMICROBIAL AGENTS AND CHEMOTHERAPY
(2023)
Article
Biochemistry & Molecular Biology
Cheol Park, Hee-Jae Cha, Min Yeong Kim, EunJin Bang, Sung-Kwon Moon, Seok Joong Yun, Wun-Jae Kim, Jeong Sook Noh, Gi-Young Kim, Suengmok Cho, Hyesook Lee, Yung Hyun Choi
Summary: The study found that phloroglucinol can protect human retinal pigment epithelium cells from oxidative damage, reduce apoptosis, and improve cell function by decreasing ROS production.
Article
Cell Biology
Jing Zheng, Xiaohui Bai, Yun Xiao, Yanchun Ji, Feilong Meng, Maerhaba Aishanjiang, Yinglong Gao, Haibo Wang, Yong Fu, Min-Xin Guan
Article
Biochemistry & Molecular Biology
Yanchun Ji, Juanjuan Zhang, Yuanyuan Lu, Qiuzi Yi, Mengquan Chen, Shipeng Xie, Xiaoting Mao, Yun Xiao, Feilong Meng, Minglian Zhang, Rulai Yang, Min-Xin Guan
JOURNAL OF BIOLOGICAL CHEMISTRY
(2020)
Article
Medicine, Research & Experimental
Jialing Yu, Xiaoyang Liang, Yanchun Ji, Cheng Ai, Junxia Liu, Ling Zhu, Zhipeng Nie, Xiaofen Jin, Chenghui Wang, Juanjuan Zhang, Fuxin Zhao, Shuang Mei, Xiaoxu Zhao, Xiangtian Zhou, Minglian Zhang, Meng Wang, Taosheng Huang, Pingping Jiang, Min-Xin Guan
JOURNAL OF CLINICAL INVESTIGATION
(2020)
Article
Biochemistry & Molecular Biology
Yun Xiao, Meng Wang, Qiufen He, Lei Xu, Qinghai Zhang, Feilong Meng, Zidong Jia, Fengguo Zhang, Haibo Wang, Min-Xin Guan
NUCLEIC ACIDS RESEARCH
(2020)
Article
Biology
Shu Zhao, Jia Shi, Guohua Yu, Dali Li, Meng Wang, Chonggang Yuan, Huihui Zhou, Amirabbas Parizadeh, Zhenlin Li, Min-Xin Guan, Shixin Ye
COMMUNICATIONS BIOLOGY
(2020)
Article
Biochemistry & Molecular Biology
Qiufen He, Xiao He, Yun Xiao, Qiong Zhao, Zhenzhen Ye, Limei Cui, Ye Chen, Min-Xin Guan
Summary: The study showed significant tissue-specific differences in mammalian mitochondrial tRNA expression, correlating with mtDNA contents but not with 2-thiouridylation levels of specific tRNAs. Aminoacylation levels of mt-tRNAs varied among tissues, with a negative correlation observed between levels and mitochondrial tRNA synthetases expression. Differential levels of OXPHOS subunits encoded by mtDNA or nuclear genes may reflect functional emphasis for mitochondria in each tissue.
JOURNAL OF BIOLOGICAL CHEMISTRY
(2021)
Article
Biochemistry & Molecular Biology
Shihao Yao, Boyuan Ma, Qiuzi Yi, Min-Xin Guan, Xiaohui Cang
Summary: The study focuses on the conserved m-gate network in the mitochondrial ADP/ATP carrier, revealing interactions between residues and the promoting effect of CATR on symmetry. These findings provide new insights into the highly conserved yet variable networks within the mitochondrial carrier family.
Article
Biochemistry & Molecular Biology
Wenlu Fan, Xiaoye Jin, Man Xu, Yongmei Xi, Weiguo Lu, Xiaohang Yang, Min-Xin Guan, Wanzhong Ge
Summary: Mutations in genes encoding mitochondrial aminoacyl-tRNA synthetases are associated with various diseases, but the precise mechanisms remain unclear. Using a Drosophila model, researchers found that inactivation of dFARS2 leads to developmental delay and seizure, and modeling human disease-associated FARS2 variants in Drosophila demonstrated their role in inducing neurological disorders. This study highlights the importance of mitochondrial aminoacylation system dysfunction in pathologies and the potential of Drosophila model for functional analysis of human disease-causing variants.
NUCLEIC ACIDS RESEARCH
(2021)
Article
Biochemistry & Molecular Biology
Chao Chen, Min-Xin Guan
Summary: This study reports the generation of induced pluripotent stem cells (iPSCs) from lymphoblastoid cell lines of individuals from an Arab-Israeli family with different mutations related to hearing loss. The researchers corrected a mutation using CRISPR/Cas9 in iPSCs derived from a hearing-impaired individual. Differentiation of these iPSCs into ear epithelial progenitor cells and inner ear hair cell-like cells revealed defects in morphology and function, with greater defects observed in cells carrying multiple mutations. Transcriptome analysis of patient-derived cells showed altered gene expression related to hair cell mechanotransduction. Genetic correction of specific mutations led to recovery in morphology and function. These findings provide insights into the underlying mechanisms of inherited hearing loss and offer potential for therapeutic interventions.
HUMAN MOLECULAR GENETICS
(2022)
Article
Biochemistry & Molecular Biology
Min Liang, Yanchun Ji, Liyao Zhang, Xuan Wang, Cuifang Hu, Juanjuan Zhang, Yiwei Zhu, Jun Q. Mo, Min-Xin Guan
Summary: LHON is a maternally inherited eye disease caused by mitochondrial DNA mutations. A specific mutation has been found to affect the structure and function of complex I, leading to mitochondrial dysfunction and cell apoptosis.
HUMAN MOLECULAR GENETICS
(2022)
Article
Biochemistry & Molecular Biology
Zhipeng Nie, Chenhui Wang, Jiarong Chen, Yanchun Ji, Hongxing Zhang, Fuxin Zhao, Xiangtian Zhou, Min-Xin Guan
Summary: This study provides new insights into the pathophysiology of LHON by analyzing retinal ganglion cell-like cells derived from induced pluripotent stem cells (iPSCs) from a Chinese family. The mitochondrial dysfunctions caused by specific mutations affect the development, morphology, and functions of the cells. Individuals carrying both mutations exhibit more severe defects compared to those carrying a single mutation.
HUMAN MOLECULAR GENETICS
(2023)
Article
Cell Biology
Yanchun Ji, Juanjuan Zhang, Min Liang, Feilong Meng, Minglian Zhang, Jun Q. Mo, Meng Wang, Min-Xin Guan
Summary: This study found that mitochondrial tRNA variants play an important role in the development of LHON, accounting for 8.75% of cases in the Han Chinese population. These new findings may have beneficial applications in the pathophysiology, disease management, and genetic counseling of LHON.
Article
Biochemistry & Molecular Biology
Feilong Meng, Zidong Jia, Jing Zheng, Yanchun Ji, Jing Wang, Yun Xiao, Yong Fu, Meng Wang, Feng Ling, Min-Xin Guan
Summary: In this study, we investigated the molecular mechanism of the m.5783C > T mutation associated with deafness. We found that this mutation significantly affects mitochondrial DNA replication and tRNA metabolism, leading to mitochondrial dysfunction and the occurrence of deafness.
NUCLEIC ACIDS RESEARCH
(2022)
Article
Cell Biology
Jing Wang, Yanchun Ji, Cheng Ai, Jia-Rong Chen, Dingyi Gan, Juanjuan Zhang, Jun Q. Mo, Min-Xin Guan
Summary: In this study, it was shown that allotopic expression of the nucleus-versions of ND6 can restore complex I, apoptosis, and mitophagy deficiencies caused by the m.14484T > C mutation. Restoring mitochondrial dysfunctions caused by the m.14484T > C mutation through overexpression of ND6 is a step towards therapeutic interventions for LHON and mitochondrial diseases.
JOURNAL OF BIOMEDICAL SCIENCE
(2023)
Review
Cell Biology
Chao Chen, Min-Xin Guan
Summary: Mitochondria are essential organelles for cellular metabolism and physiology, and mtDNA mutations are associated with a wide range of degenerative and neuromuscular diseases. However, the pathophysiology of mitochondrial diseases, especially threshold effect and tissue specificity, is not well understood and effective treatment is lacking. Using patients-derived iPSCs as ex vivo models has advanced our understanding of the pathophysiology of maternally inherited diseases and has potential for therapeutic interventions.
JOURNAL OF BIOMEDICAL SCIENCE
(2023)