Article
Multidisciplinary Sciences
Mack B. Reynolds, Hanna S. Hong, Britton C. Michmerhuizen, Anna-Lisa E. Lawrence, Li Zhang, Jason S. Knight, Costas A. Lyssiotis, Basel H. Abuaita, Mary X. O'Riordan
Summary: Macrophage metabolic plasticity allows the redirection of electron transport for inflammation and host defense. Inflammatory activation leads to disassembly of Complex II and loss of succinate dehydrogenase subunit B through sequestration and selective mitophagy. The regulatory role of cardiolipin in coordinating Complex II function and inflammatory metabolic remodeling is demonstrated.
Article
Biochemistry & Molecular Biology
Sui Lay, Xavier Pearce, Oana Sanislav, Paul Robert Fisher, Sarah Jane Annesley
Summary: Mitochondrial Complex II is composed of four core subunits and mutations in any of these subunits can result in lowered Complex II activity. However, mutations in different subunits are associated with distinct patterns of clinical disease. In this study, knockdown of individual Complex II subunits in Dictyostelium discoideum revealed specific phenotypic defects and mitochondrial dysfunction associated with SdhA knockdown. These defects were mediated by the chronic activation of AMP-activated protein kinase.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Roberta Peruzzo, Samantha Corra, Roberto Costa, Michele Brischigliaro, Tatiana Varanita, Lucia Biasutto, Chiara Rampazzo, Daniele Ghezzi, Luigi Leanza, Mario Zoratti, Massimo Zeviani, Cristiano De Pitta, Carlo Viscomi, Rodolfo Costa, Ildiko Szabo
Summary: Pyocyanin, a bacterial redox cycler, has been shown to replace the redox functions of complex III in cells from patients with complex III deficiencies, as well as in genetic models of fruit flies and zebrafish. This suggests the potential use of redox cyclers for the treatment of complex III disorders in mitochondrial diseases.
NATURE COMMUNICATIONS
(2021)
Article
Cell Biology
Ritu Som, Brian D. Fink, Liping Yu, William I. Sivitz
Summary: Previous research showed that mitochondria with low membrane potential in skeletal muscle and interscapular brown adipose tissue can accumulate oxaloacetate to inhibit complex II respiration. Further studies on mice with different inner membrane potential in brown adipose tissue verify this mechanism and its physiological significance.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
(2023)
Review
Biology
Michela Rugolo, Claudia Zanna, Anna Maria Ghelli
Summary: This study provides a detailed description of the assembly characteristics of supercomplexes (SCs) of mitochondrial respiratory chain complex III, as well as the metabolic disturbances associated with defective complex III and interventions to mitigate these deficiencies.
Article
Agronomy
Christine Njiru, Corinna Saalwaechter, Konstantinos Mavridis, John Vontas, Sven Geibel, Nicky Wybouw, Thomas Van Leeuwen
Summary: A target site mutation in a spider mite pest has been found to confer resistance to certain acaricides and has significant fitness costs. These findings highlight the importance of considering multiple factors in assessing the impact of resistance mutations on pest populations.
PEST MANAGEMENT SCIENCE
(2023)
Article
Cell & Tissue Engineering
Kaito Harada, Takashi Yahata, Makoto Onizuka, Takamasa Ishii, Abd Aziz Ibrahim, Eri Kikkawa, Yoichi Gondo, Kiyoshi Ando
Summary: This study reveals that mitochondrial complex II (MCII) plays an essential role in long-term hematopoiesis and hematopoietic reconstitution. Dysfunctions in MCII result in increased reactive oxygen species (ROS) accumulation and DNA damage in hematopoietic stem cells (HSCs), leading to premature senescence.
Article
Chemistry, Medicinal
Keisuke Komatsuya, Takaya Sakura, Kazuro Shiomi, Satoshi Omura, Kenji Hikosaka, Tomoyoshi Nozaki, Kiyoshi Kita, Daniel Ken Inaoka
Summary: The mitochondrial electron transport chain of Plasmodium falciparum has been identified as a promising target for chemotherapy, with two enzymes and complex II considered as candidate drug targets. Siccanin has been discovered as a potent inhibitor of complex II and III in P. falciparum, showing no inhibition of corresponding complexes in mammalian mitochondria. This compound has a dual mechanism of action and is a potential candidate for the development of new antimalarial drugs.
Review
Biochemistry & Molecular Biology
Shuting Guan, Li Zhao, Ruiyun Peng
Summary: Mitochondrial supercomplexes, consisting of enzyme complexes and electron carriers, play a crucial role in improving the efficiency of mitochondrial respiratory chain and maintaining cellular metabolism homeostasis.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Biochemistry & Molecular Biology
Eric Goetzman, Zhenwei Gong, Bob Zhang, Radhika Muzumdar
Summary: Aging is linked to diminished mitochondrial function, which contributes to age-related diseases. Mitochondrial Complex II has emerged as a crucial factor in the aging process, as its dysfunction disrupts energy production and promotes oxidative stress. Interestingly, the accumulation of succinate, the substrate for Complex II, during mitochondrial dysfunction, has been shown to have signaling effects. This review explores recent advancements in understanding the function of Complex II, succinate signaling, and their roles in aging and age-related diseases.
Article
Multidisciplinary Sciences
Agnes Moe, Justin Di Trani, John L. Rubinstein, Peter Brzezinski
Summary: Energy conversion in aerobic organisms involves electron transfer from low-potential donors to dioxygen through the membrane-bound respiratory chain. The formation of supercomplex CIII2CIV1/2 facilitates electron transfer by two-dimensional diffusion of cyt. c, increasing QH(2):O-2 oxidoreductase activity and suggesting a regulatory role for supercomplex formation in the respiratory chain.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Multidisciplinary Sciences
Thomas D. Jackson, Jordan J. Crameri, Linden Muellner-Wong, Ann E. Frazier, Catherine S. Palmer, Luke E. Formosa, Daniella H. Hock, Kenji M. Fujihara, Tegan Stait, Alice J. Sharpe, David R. Thorburn, Michael T. Ryan, David A. Stroud, Diana Stojanovski
Summary: Sideroflexins (SFXNs) are a family of proteins that have different functions in mitochondrial biology. Loss of SFXN4 leads to complex I assembly defect and it interacts with the core components of the mitochondrial complex I intermediate assembly complex.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Agronomy
Steven Kildea, Pierre Hellin, Thies M. Heick, Fiona Hutton
Summary: The study found a wide range of sensitivity to fenpicoxamid in European Z. tritici populations, with limited or non-existing target-site resistance mechanisms and limited impact of non-target site resistance mechanisms in the field.
PEST MANAGEMENT SCIENCE
(2022)
Article
Biochemistry & Molecular Biology
Gergely Svab, Marton Kokas, Ildiko Sipos, Attila Ambrus, Laszlo Tretter
Summary: Methylene blue shows promising effects in neurodegenerative disease models, mainly by modulating mitochondrial function. Research indicates that it can improve bioenergetic parameters of mitochondria, such as respiration and membrane potential.
Article
Biochemistry & Molecular Biology
Adam J. Rauckhorst, Liping Yu, Brian D. Fink, Ritu Som, Eric B. Taylor, William I. Sivitz
Summary: Under low inner mitochondrial membrane potential, oxaloacetate accumulates in organelles and inhibits succinate dehydrogenase, leading to decreased complex II-energized respiration. This process is more active in muscle than in interscapular brown adipose tissue (IBAT) mitochondria. In IBAT mitochondria, oxaloacetate is decarboxylated to pyruvate.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
(2023)
Article
Genetics & Heredity
Valentina Barone, Chiara La Morgia, Leonardo Caporali, Claudio Fiorini, Michele Carbonelli, Laura Ludovica Gramegna, Fiorina Bartiromo, Caterina Tonon, Luca Morandi, Rocco Liguori, Aurelia Petrini, Rachele Brugnano, Rachele Del Sordo, Carla Covarelli, Manrico Morroni, Raffaele Lodi, Valerio Carelli
Summary: This study reports a unique combination of LHON, nephropathy, sensorineural deafness, and subcortical and cerebellar atrophy associated with the m.13513G>A variant.
FRONTIERS IN GENETICS
(2022)
Article
Genetics & Heredity
Arianna Manini, Leonardo Caporali, Megi Meneri, Simona Zanotti, Daniela Piga, Ignazio Giuseppe Arena, Stefania Corti, Antonio Toscano, Giacomo Pietro Comi, Olimpia Musumeci, Valerio Carelli, Dario Ronchi
Summary: This article describes two cases of mitochondrial DNA maintenance disorders characterized by progressive external ophthalmoplegia, ptosis, and muscle weakness. Sequencing analysis revealed different mutations in the RNASEH1 gene in these patients.
FRONTIERS IN GENETICS
(2022)
Article
Biochemistry & Molecular Biology
Elisa Boschetti, Leonardo Caporali, Roberto D'Angelo, Carolina Malagelada, Anna Accarino, Maria Teresa Dotti, Roberta Costa, Giovanna Cenacchi, Loris Pironi, Rita Rinaldi, Vincenzo Stanghellini, Stefano Ratti, Lucia Manzoli, Valerio Carelli, Roberto De Giorgio
Summary: MNGIE is a rare genetic disorder characterized by a deficiency in the TP enzyme, leading to impaired mitochondrial DNA replication and energy production in metabolically active tissues. TP replacement through transplantation is the only effective life-saving approach, but gut tissue changes may persist even after transplant.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Mariantonietta Capristo, Valentina Del Dotto, Concetta Valentina Tropeano, Claudio Fiorini, Leonardo Caporali, Chiara La Morgia, Maria Lucia Valentino, Monica Montopoli, Valerio Carelli, Alessandra Maresca
Summary: The study suggests that rapamycin-based therapy may be a promising treatment option for MERRF. The first therapeutic strategy was ineffective in high-mutation load cell lines, while the second strategy showed significant improvement in fibroblasts with intermediate-mutation load, rescuing the bioenergetics defect completely.
MOLECULAR MEDICINE
(2022)
Article
Clinical Neurology
Marco Percetti, Giulia Franco, Edoardo Monfrini, Leonardo Caporali, Raffaella Minardi, Chiara La Morgia, Maria Lucia Valentino, Rocco Liguori, Ilaria Palmieri, Donatella Ottaviani, Maria Vizziello, Dario Ronchi, Federica Di Berardino, Antoniangela Cocco, Bertil Macao, Maria Falkenberg, Giacomo Pietro Comi, Alberto Albanese, Bruno Giometto, Enza Maria Valente, Valerio Carelli, Alessio Di Fonzo
Summary: This study identified potentially pathogenic variants in the TWNK gene in a cohort of Italian PD patients and found that some TWNK-adPEO patients also exhibited parkinsonism. Further studies are needed to confirm these findings.
MOVEMENT DISORDERS
(2022)
Letter
Clinical Neurology
Giulia Amore, Veria Vacchiano, Chiara La Morgia, Maria L. Valentino, Leonardo Caporali, Claudio Fiorini, Danara Ormanbekova, Fabrizio Salvi, Anna Bartoletti-Stella, Sabina Capellari, Rocco Liguori, Valerio Carelli
JOURNAL OF NEUROLOGY
(2023)
Article
Ophthalmology
Piero Barboni, Chiara La Morgia, Maria Lucia Cascavilla, Eun Hee Hong, Marco Battista, Anna Majander, Leonardo Caporali, Vincenzo Starace, Giulia Amore, Antonio Di Renzo, Michele Carbonelli, Paolo Nucci, Neringa Jurkute, Benson S. Chen, Roberta Panebianco, Anna Maria De Negri, Federico Sadun, Vincenzo Parisi, Francesco Bandello, Alfredo A. Sadun, Valerio Carelli, Patrick Yu-wai-man
Summary: The purpose of this study was to investigate the clinical and molecular genetic features of childhood-onset Leber hereditary optic neuropathy (LHON) and understand the factors influencing the visual outcome. The study found that children who developed LHON before the age of 9 had a better visual prognosis than those who developed it later. This study is of importance for understanding LHON.
AMERICAN JOURNAL OF OPHTHALMOLOGY
(2023)
Letter
Clinical Neurology
Claudio Fiorini, Danara Ormanbekova, Flavia Palombo, Michele Carbonelli, Giulia Amore, Martina Romagnoli, Pietro d'Agati, Maria Lucia Valentino, Piero Barboni, Maria Lucia Cascavilla, Annamaria De Negri, Federico Sadun, Arturo Carta, Francesco Testa, Vittoria Petruzzella, Silvana Guerriero, Stefania Bianchi Marzoli, Valerio Carelli, Chiara La Morgia, Leonardo Caporali
Article
Ophthalmology
Marco Battista, Maria Lucia Cascavilla, Enrico Borrelli, Costanza Barresi, Giorgio Lari, Leonardo Caporali, Chiara Vigano, Alessandro Berni, Valerio Carelli, Francesco Bandello, Piero Barboni
Summary: The study aimed to compare the choroidal vascularity index (CVI) in patients with Leber hereditary optic neuropathy (LHON), dominant optic atrophy (DOA), and healthy subjects. The results showed that CVI remained stable in both DOA and LHON patients, suggesting that the choroidal structure is not irreversibly compromised even in the chronic phase of the neuropathy.
Letter
Clinical Neurology
Marco Percetti, Edoardo Monfrini, Leonardo Caporali, Raffaella Minardi, Valerio Carelli, Enza Maria Valente, Alessio Di Fonzo
MOVEMENT DISORDERS
(2023)
Article
Oncology
Daniele Boso, Martina Tognon, Matteo Curtarello, Sonia Minuzzo, Ilaria Piga, Valentina Brillo, Elisabetta Lazzarini, Jessica Carlet, Ludovica Marra, Chiara Trento, Andrea Rasola, Ionica Masgras, Leonardo Caporali, Fabio Del Ben, Giulia Brisotto, Matteo Turetta, Roberta Pastorelli, Laura Brunelli, Filippo Navaglia, Giovanni Esposito, Angela Grassi, Stefano Indraccolo
Summary: This study identified clonal populations in ovarian cancer cells with different sensitivity to glucose starvation and investigated the effects of anti-VEGF therapy on this phenotype through multi-omics analysis. Glucose deprivation sensitive (GDS) clones died while glucose deprivation resistant (GDR) clones adapted and survived through enhanced mitochondrial oxidative phosphorylation and nutrient uptake. Furthermore, GDR clones showed increased tumorigenic properties and sensitivity to the mitochondrial respiratory chain inhibitor metformin.
JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH
(2023)
Article
Genetics & Heredity
Claudio Fiorini, Andrea Degiorgi, Maria Lucia Cascavilla, Concetta Valentina Tropeano, Chiara La Morgia, Marco Battista, Danara Ormanbekova, Flavia Palombo, Michele Carbonelli, Francesco Bandello, Valerio Carelli, Alessandra Maresca, Piero Barboni, Enrico Baruffini, Leonardo Caporali
Summary: We report the first family with a homozygous MECR variant causing an LHON-like optic neuropathy, which pairs the recent MCAT findings, reinforcing the impairment of mtFAS as a novel pathogenic mechanism in LHON.
JOURNAL OF MEDICAL GENETICS
(2023)
Article
Biochemistry & Molecular Biology
Stefano Miglietta, Giulia Girolimetti, Lorena Marchio, Manuela Sollazzo, Noemi Laprovitera, Sara Coluccelli, Dario De Biase, Antonio De Leo, Donatella Santini, Ivana Kurelac, Luisa Iommarini, Anna Ghelli, Davide Campana, Manuela Ferracin, Anna Myriam Perrone, Giuseppe Gasparre, Anna Maria Porcelli
Summary: This article analyzes a case of SCCOPT and provides in-depth characterization of its features and characteristics. SCCOPT is a rare and aggressive tumor with poor prognosis, and its biology and molecular features have not been thoroughly investigated yet. The analysis of miRNA expression profile and metabolic markers can provide useful information for future diagnoses.
Article
Ophthalmology
Michele Carbonelli, Chiara La Morgia, Martina Romagnoli, Giulia Amore, Pietro D'Agati, Maria Lucia Valentino, Leonardo Caporali, Maria Lucia Cascavilla, Marco Battista, Enrico Borrelli, Antonio Di Renzo, Vincenzo Parisi, Nicole Balducci, Valerio Carelli, Piero Barboni
Summary: In the study of LHON, thinning of sector 8 is identified as the structural hallmark of the conversion from the presymptomatic to the affected state, which shows dynamic thickening in the presymptomatic stage and gradual thinning following the conversion.
AMERICAN JOURNAL OF OPHTHALMOLOGY
(2022)
Meeting Abstract
Biochemistry & Molecular Biology
Flavia Palombo, Camille Peron, Leonardo Caporali, Angelo Iannielli, Alessandra Maresca, Ivano Di Meo, Claudio Fiorini, Alice Segnali, Tiina Manninen, Francesca L. Sciacca, Ambra Rizzo, Sonia Levi, Anu Suomalainen, Alessandro Prigione, Vania Broccoli, Valerio Carelli, Valeria Tiranti
EUROPEAN JOURNAL OF HUMAN GENETICS
(2022)
Article
Biochemistry & Molecular Biology
Brandon P. Russell, David J. Vinyard
Summary: The Mn4CaO5 oxygen-evolving complex in Photosystem II is crucial for water oxidation. D1 residue R334 participates in proton release and interacts with PsbO. A D1-R334G mutant destabilizes the OEC but stabilizes the S2 intermediate.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
(2024)
Article
Biochemistry & Molecular Biology
Alexander A. Bulychev, Tatiana S. Strelets
Summary: Excitable cells of higher plants and characean algae respond to stressful stimuli by generating action potentials that influence chlorophyll fluorescence and photosynthesis for an extended period of time. While plant leaves exhibit a reversible depression in the efficiency of photosystem II reaction after an individual action potential, characean algae show long-lasting oscillations of photosystem II reaction efficiency after firing an action potential. This study investigates the possible mechanisms behind these oscillations and suggests that they are a result of metabolic rearrangements in chloroplasts and the cyclosis cessation-recovery cycle induced by calcium influx during action potentials. The findings also indicate that fluidic communications between different cell regions play a role in these oscillations, and the inhibition of oscillations occurs when these communications are restricted or eliminated.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
(2024)
Article
Biochemistry & Molecular Biology
Dmitry Zlenko, Elena A. Protasova, Georgy Tsoraev, Nikolai N. Sluchanko, Dmitry A. Cherepanov, Thomas Friedrich, Baosheng Ge, Song Qin, Eugene G. Maksimov, Andrew B. Rubin
Summary: The conformation of chromophores in isolated phycobiliproteins is heterogeneous, but not in the entire phycobilisome (PBS). Under low-energy excitation, there is no significant uphill energy transfer from the core to the peripheral rods of the PBS, while transfer from the terminal emitters to bulk allophycocyanin chromophores is highly probable.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
(2024)
Article
Biochemistry & Molecular Biology
Makio Yokono, Chiyo Noda, Jun Minagawa
Summary: This paper investigates the energy transfer between Photosystem II and Photosystem I in Arabidopsis thaliana, and finds that the fast spillover is reversibly regulated depending on pH.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
(2024)