Review
Cell Biology
Jerry Edward Chipuk, Jarvier N. Mohammed, Jesse D. Gelles, Yiyang Chen
Summary: Mitochondria play crucial roles not only in maintaining organismal homeostasis and contributing to human diseases, but also in regulating regulated cell death processes. They serve as essential compartments for the production and presentation of key molecules, as well as platforms for enabling, initiating, and executing regulated cell death.
DEVELOPMENTAL CELL
(2021)
Article
Clinical Neurology
Amel Karaa, Enrico Bertini, Valerio Carelli, Bruce H. Cohen, Gregory M. Enns, Marni J. Falk, Amy Goldstein, Grainne Siobhan Gorman, Richard Haas, Michio Hirano, Thomas Klopstock, Mary Kay Koenig, Cornelia Kornblum, Costanza Lamperti, Anna Lehman, Nicola Longo, Maria Judit Molnar, Sumit Parikh, Han Phan, Robert D. S. Pitceathly, Russell Saneto, Fernando Scaglia, Serenella Servidei, Mark Tarnopolsky, Antonio Toscano, Johan L. K. Van Hove, John Vissing, Jerry Vockley, Jeffrey S. Finman, David A. Brown, James A. Shiffer, Michelango Mancuso
Summary: Primary mitochondrial myopathies (PMMs) impair mitochondrial oxidative phosphorylation and affect physical function, exercise capacity, and quality of life. A phase-3 clinical trial showed that elamipretide treatment did not improve outcomes in patients with PMM. This study provides evidence that elamipretide does not improve the 6MWT or fatigue compared with placebo in PMM patients.
Article
Biochemistry & Molecular Biology
Valeria Manganelli, Antonella Capozzi, Serena Recalchi, Gloria Riitano, Vincenzo Mattei, Agostina Longo, Roberta Misasi, Tina Garofalo, Maurizio Sorice
Summary: This study investigated the presence of cardiolipin (CL) within mitochondria-associated membranes (MAMs) following autophagic stimulus, and the potential role of CL-enriched raft-like microdomains as a signaling platform in autophagosome formation. The findings indicate that CL accumulates in MAM fractions after autophagic stimulation, where it interacts with proteins such as MFN2 and CANX, contributing to the multimolecular complex involved in autophagosome formation. This suggests that CL may have structural and functional implications in the pathophysiology of neurodegenerative diseases.
Article
Multidisciplinary Sciences
Mukesh Mahajan, Nikhil Bharambe, Yutong Shang, Bin Lu, Abhishek Mandal, Pooja Madan Mohan, Rihua Wang, Jennifer C. Boatz, Juan Manuel Martinez Galvez, Anna Shnyrova, Xin Qi, Matthias Buck, Patrick C. A. van der Wel, Rajesh Ramachandran
Summary: The study elucidated the interaction mechanism between Drp1 and mitochondrial outer membrane cardiolipin, which triggers mitochondrial hyperfragmentation under stress conditions. Mutations in the CBM weakened this interaction, impairing Drp1-dependent fission under stress and inducing the formation of donut-shaped mitochondria.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Biochemistry & Molecular Biology
Yaiza R. Varela, Emilio J. Gonzalez-Ramirez, Marina N. Iriondo, Uxue Ballesteros, Asier Etxaniz, Lidia Ruth Montes, Felix M. Goni, Alicia Alonso
Summary: Cardiolipin (CL) is an important lipid for damaged mitochondrial recognition by the LC3/GABARAP autophagy proteins. The role of ceramide (Cer) is uncertain, but it has been proposed that CL and Cer may coexist in mitochondria. This study examined the interaction of LC3/GABARAP proteins with model membranes containing eSM, DOPE, CL, and/or Cer. The results suggest that Cer-enriched rigid nanodomains within the fluid phase may facilitate LC3/GABARAP protein interaction by creating structural defects at the nanointerfaces.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Orthopedics
Xueying Zhang, Edward Bowen, Meng Zhang, Hazel H. Szeto, Xiang-Hua Deng, Scott A. Rodeo
Summary: This study investigated the potential of the mitochondrial protectant SS-31 to improve mitochondrial function and promote tendon healing in a murine supraspinatus tendinopathy model. The results showed that SS-31 improved mitochondrial function and promoted tendon healing, especially when combined with removal of subacromial impingement.
JOURNAL OF BONE AND JOINT SURGERY-AMERICAN VOLUME
(2022)
Article
Medicine, Research & Experimental
Jeffrey D. Tamucci, Nathan N. Alder, Eric R. May
Summary: Mitochondrial dysfunction is associated with several leading causes of death in the US. Synthetic mitochondria-targeted peptides (MTPs) show promise in restoring mitochondrial function and treating common diseases. This study used molecular dynamics simulations to investigate the interactions between MTPs and lipid bilayers, revealing that MTPs modulate membrane properties and reduce energy sinks. The findings suggest that MTPs have therapeutic potential and their interactions with lipid bilayers are crucial to their mechanism of action.
MOLECULAR PHARMACEUTICS
(2023)
Article
Biochemistry & Molecular Biology
Kristyn Gumpper-Fedus, Ki Ho Park, Hanley Ma, Xinyu Zhou, Zehua Bian, Karthikeyan Krishnamurthy, Matthew Sermersheim, Jingsong Zhou, Tao Tan, Lei Li, Jianxun Liu, Pei-Hui Lin, Hua Zhu, Jianjie Ma
Summary: MG53 interacts with mitochondrial-specific lipid CL to preserve mitochondrial integrity and prevent mitophagy, showing potential for targeted protein therapy for cardiac protection.
Article
Biochemistry & Molecular Biology
Shyamalagauri Jadhav, Olga Protchenko, Fengmin Li, Ethan Baratz, Minoo Shakoury-Elizeh, Alan Maschek, James Cox, Caroline C. Philpott
Summary: Iron is an essential nutrient that forms cofactors required for cellular protein activity, but can be toxic if not properly managed. PCBP1 is a multifunctional protein that binds iron and nucleic acids, regulating their fate. Deletion of PCBP1 in the liver results in dysregulated iron balance, leading to chronic liver disease with lipid accumulation and mitochondrial dysfunction. Supplementation with coenzyme Q is necessary to restore mitochondrial function in this context of ongoing oxidative damage.
FREE RADICAL BIOLOGY AND MEDICINE
(2021)
Article
Multidisciplinary Sciences
Jargalsaikhan Dagvadorj, Karolina Mikulska-Ruminska, Gantsetseg Tumurkhuu, Rojo A. Ratsimandresy, Jessica Carriere, Allen M. Andres, Stefanie Marek-Iannucci, Yang Song, Shuang Chen, Malcolm Lane, Andrea Dorfleutner, Roberta A. Gottlieb, Christian Stehlik, Suzanne Cassel, Fayyaz S. Sutterwala, Ivet Bahar, Timothy R. Crother, Moshe Arditi
Summary: The balance between NLRP3 inflammasome activation and mitophagy is crucial for cellular homeostasis, with IL-1 alpha playing a key role in regulating this balance. IL-1 alpha deficiency can lead to altered cellular functions and health by affecting mitochondrial damage and NLRP3 inflammasome activation. The interaction between IL-1 alpha and mitochondrial cardiolipin disrupts mitophagy and enhances NLRP3 inflammasome activation, highlighting the importance of this regulatory mechanism in cellular health.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Biochemistry & Molecular Biology
Markus Rose, Martin Kurylowicz, Mohammad Mahmood, Sheldon Winkel, Jose M. Moran-Mirabal, Cecile Fradin
Summary: This study focuses on the interaction between two core members of the Bcl-2 family, Bax and tBid, revealing their different conformations on the membrane and their increased affinity when transitioning from loosely membrane-associated to transmembrane forms. The researchers used single particle imaging and cross-correlation analysis to infer protein conformation and detect transient interactions in a mitochondria-like planar supported lipid bilayer. This leads to an updated molecular model for the activation of Bax by tBid.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Cell Biology
Haruhiko Sakiyama, Lan Li, Sachi Kuwahara-Otani, Tsutomu Nakagawa, Hironobu Eguchi, Daisaku Yoshihara, Masakazu Shinohara, Noriko Fujiwara, Keiichiro Suzuki
Summary: ChREBP is a glucose-responsive transcription factor that regulates gene expression in the liver, converting excess carbohydrates into storage fat. ChREBP knockout mice display an anti-obesity phenotype, with alterations in electron transport system proteins and mitochondrial structure in their brown adipose tissue. The study clarifies the new role of ChREBP in adipose tissue and its involvement in mitochondrial function, which could potentially improve obesity management.
MOLECULAR AND CELLULAR BIOCHEMISTRY
(2021)
Article
Biochemistry & Molecular Biology
Alaumy Joshi, Vishal M. Gohil
Summary: Barth syndrome is a debilitating disorder caused by mutations in the TAFAZZIN gene, which is involved in maintaining normal levels of cardiolipin in mitochondrial membranes. A recent study found that in addition to calcium, the levels of magnesium are significantly reduced in Barth syndrome patients. This study also identified a decrease in the abundance of the mitochondrial magnesium influx channel MRS2 in various models of Barth syndrome. The researchers attributed this reduction to the increased turnover of MRS2 in models with cardiolipin deficiency. These findings suggest that perturbation of mitochondrial magnesium homeostasis may contribute to the pathology of Barth syndrome.
HUMAN MOLECULAR GENETICS
(2023)
Review
Genetics & Heredity
Krystie Chew, Linlin Zhao
Summary: Mitochondria play crucial roles in eukaryotic cells, carrying their own genomic DNA that encodes essential components of the oxidative phosphorylation system. The mitochondrial transcription factor TFAM interacts with different forms of DNA structures, impacting mtDNA repair and packaging. Understanding these interactions is important for maintaining cellular and organismal functions.
Article
Immunology
Rui Miao, Cong Jiang, Winston Y. Chang, Haiwei Zhang, Jinsu An, Felicia Ho, Pengcheng Chen, Han Zhang, Caroline Junqueira, Dulguun Amgalan, Felix G. Liang, Junbing Zhang, Charles L. Evavold, Iva Hafner-Bratkovic, Zhibin Zhang, Pietro Fontana, Shiyu Xia, Markus Waldeck-Weiermair, Youdong Pan, Thomas Michel, Liron Bar-Peled, Hao Wu, Jonathan C. Kagan, Richard N. Kitsis, Peng Zhang, Xing Liu, Judy Lieberman
Summary: Gasdermin D (GSDMD)-activated inflammatory cell death causes mitochondrial damage, leading to reduced mitochondrial numbers, mitophagy, ROS, loss of transmembrane potential, attenuated oxidative phosphorylation (OXPHOS), and release of mitochondrial proteins and DNA. Mitochondrial damage occurs independently of the B-cell lymphoma 2 family and depends on the binding of GSDMD-NT to cardiolipin. Cardiolipin synthase and scramblase play a role in suppressing inflammasome activation and mitochondrial damage.
Article
Multidisciplinary Sciences
Mehdi Hichor, Venkat Krishnan Sundaram, Stephanie A. Eid, Ronza Abdel-Rassoul, Patrice X. Petit, Didier Borderie, Jean Bastin, Assaad A. Eid, Marin Manuel, Julien Grenier, Charbel Massaad
SCIENTIFIC REPORTS
(2018)
Article
Biology
Emilie Ma, Pauline Dupaigne, Laurent Maloisel, Raphael Guerois, Eric Le Cam, Eric Coic
Review
Cell Biology
Nazanin Sadat Aghili Moghaddam, Mohammad Nosrati Oskouie, Alexandra E. Butler, Patrice X. Petit, George E. Barreto, Amirhossein Sahebkar
JOURNAL OF CELLULAR PHYSIOLOGY
(2019)
Article
Multidisciplinary Sciences
Eliana Moreira Tavares, William Douglass Wright, Wolf-Dietrich Heyer, Eric Le Cam, Pauline Dupaigne
NATURE COMMUNICATIONS
(2019)
Article
Cell Biology
Francisco J. Sala de Oyanguren, Nathan E. Rainey, Aoula Moustapha, Ana Saric, Franck Sureau, Jose-Enrique O'Connor, Patrice X. Petit
Article
Cell Biology
Patrice X. Petit, Hector Ardilla-Osorio, Lucile Penalvia, Nathan E. Rainey
Article
Genetics & Heredity
Jonathan Ribeiro, Pauline Dupaigne, Cynthia Petrillo, Clotilde Duquenne, Xavier Veaute, Carole Saintome, Didier Busso, Raphael Guerois, Emmanuelle Martini, Gabriel Livera
Summary: During meiosis, MEIOB and SPATA22 form a protein complex that interacts with RPA, providing specific properties to ssDNA condensation in meiotic cells to meet the requirements of homologous recombination.
Article
Biochemistry & Molecular Biology
Ayeong So, Elodie Dardillac, Ali Muhammad, Catherine Chailleux, Laura Sesma-Sanz, Sandrine Ragu, Eric Le Cam, Yvan Canitrot, Jean Yves Masson, Pauline Dupaigne, Bernard S. Lopez, Josee Guirouilh-Barbat
Summary: Selection of DSB repair pathway is crucial for genetic stability. RAD51 plays a role in preventing nonconservative repair by controlling the second step of the repair process in human cells. RAD51's DNA binding ability is required for inhibiting SSA/A-EJ, not GC.
NUCLEIC ACIDS RESEARCH
(2022)
Article
Biochemistry & Molecular Biology
Yann Benureau, Caroline Pouvelle, Pauline Dupaigne, Sonia Baconnais, Eliana Moreira Tavares, Gerard Mazon, Emmanuelle Despras, Eric Le Cam, Patricia L. Kannouche
Summary: Research has found that DNA lesions in S phase can threaten genome stability, but specific damage tolerance mechanisms can overcome these obstacles and ensure the continuity of DNA synthesis. Specifically, the TLS polymerase eta plays a crucial role at the replication fork, and RAD51 also supports repriming at the replication fork, providing important clues for addressing issues caused by DNA damage.
NUCLEIC ACIDS RESEARCH
(2022)
Article
Genetics & Heredity
Oriane Trouillard, Pauline Dupaigne, Margaux Dunoyer, Mohamed Doulazmi, Morten Krogh Herlin, Solene Frismand, Audrey Riou, Veronique Legros, Guillaume Chevreux, Xavier Veaute, Didier Busso, Coralie Fouquet, Cecile Saint-Martin, Aurelie Meneret, Alain Trembleau, Isabelle Dusart, Caroline Dubacq, Emmanuel Roze
Summary: Mirror movements are involuntary movements of one hand that mirror intentional movements of the other hand. Congenital mirror movements (CMM) is a rare genetic disorder with autosomal dominant inheritance, in which mirror movements are the main neurological manifestation. RAD51 haploinsufficiency, including loss-of-function of non-truncating variants, results in CMM.
JOURNAL OF MEDICAL GENETICS
(2023)
Article
Biochemistry & Molecular Biology
Maud Hertzog, Thomas Noe Perry, Pauline Dupaigne, Sandra Serres, Violette Morales, Anne-Lise Soulet, Jason C. Bell, Emmanuel Margeat, Stephen C. Kowalczykowski, Eric Le Cam, Remi Fronzes, Patrice Polard
Summary: This study investigated the ATP-dependent assembly mechanism of the presynaptic filament of Streptococcus pneumoniae RecA (SpRecA). It was found that the pneumococcal Single-Stranded DNA Binding (SSB) protein cannot assist SpRecA filamentation, while the conserved RadA helicase promotes SpRecA nucleofilamentation. The results reveal differences in the HR activities and ATP-dependent DNA binding modes between SpRecA and Escherichia coli RecA.
NUCLEIC ACIDS RESEARCH
(2023)
Article
Biochemical Research Methods
Yann Benureau, Eliana Moreira Tavares, Ali-Akbar Muhammad, Sonia Baconnais, Eric Le Cam, Pauline Dupaigne
BIOLOGY METHODS & PROTOCOLS
(2020)
Article
Cell Biology
Nathan E. Rainey, Aoula Moustapha, Ana Saric, Gael Nicolas, Franck Sureau, Patrice X. Petit
CELL DEATH DISCOVERY
(2019)
Article
Cell Biology
Maxence de Taffin de Tilques, Jean-Paul Lasserre, Francois Godard, Elodie Sardin, Marine Bouhier, Marina Le Guedard, Roza Kucharczyk, Patrice X. Petit, Eric Testet, Jean-Paul di Rago, Deborah Tribouillard-Tanvier
Article
Biochemistry & Molecular Biology
Mehdi Hichor, Nirmal Kumar Sampathkumar, Julia Montanaro, Didier Borderie, Patrice X. Petit, Victor Gorgievski, Eleni T. Tzavara, Assaad A. Eid, Frederic Charbonnier, Julien Grenier, Charbel Massaad
ANTIOXIDANTS & REDOX SIGNALING
(2017)