Article
Cell Biology
Yi Li, Veronica Costiniti, Guilherme H. Souza Bomfim, Maria Neginskaya, Ga-Yeon Son, Beverly Rothermel, Evgeny Pavlov, Rodrigo S. Lacruz
Summary: This study investigates the role of RCAN1 in enamel formation by overexpressing RCAN1 in ameloblast cells. The study shows that overexpressing RCAN1 leads to increased reactive oxygen species, decreased mitochondrial bioenergetics, and decreased expression of enamel-specific genes. These findings suggest that elevated levels of RCAN1 in ameloblasts of individuals with DS may impact enamel formation by altering the redox environment, mitochondrial function, and enamel-specific gene expression.
Article
Biochemistry & Molecular Biology
Jingtan Su, Rucha Arun Bapat, Gayathri Visakan, Janet Moradian-Oldak
Summary: By analyzing Ambn sequences of 53 species, we found that a potential amphipathic helix (AH) within the sequence encoded by Exon 5 of Ambn appeared in species with prismatic enamel. Synthetic peptides derived from mammalian Ambn interacted with liposomes and caused liposome leakage, while peptides from species without prismatic enamel did not show similar effects. Our findings suggest that the AH motif is closely related to the emergence of enamel prismatic structure and is involved in Ambn's regulation of cell morphology and polarization genes.
MOLECULAR BIOLOGY AND EVOLUTION
(2022)
Article
Biochemistry & Molecular Biology
Neoma T. Boardman, Baher Migally, Chantal Pileggi, Gaganvir S. Parmar, Jian Ying Xuan, Keir Menzies, Mary-Ellen Harper
Summary: GRX2 and SIRT3 play important roles in controlling cardiac mitochondrial redox and oxidative processes. However, their combined absence does not exacerbate effects, consistent with the overall conclusion that they function together in the complex redox and antioxidant systems in the heart.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2021)
Article
Biology
Sven Dennerlein, Sabine Poerschke, Silke Oeljeklaus, Cong Wang, Ricarda Richter-Dennerlein, Johannes Sattmann, Diana Bauermeister, Elisa Hanitsch, Stefan Stoldt, Thomas Langer, Stefan Jakobs, Bettina Warscheid, Peter Rehling
Summary: TMEM223 and SMIM4 were identified as ribosome-associated proteins involved in complex IV and III biogenesis, respectively. They stimulate mRNA translation and support early assembly steps, linking translation machinery to the biogenesis of OXPHOS complexes.
Article
Multidisciplinary Sciences
Catherine A. Makarewich, Amir Z. Munir, Svetlana Bezprozvannaya, Aaron M. Gibson, Soo Young Kim, Misty S. Martin-Sandoval, Thomas P. Mathews, Luke I. Szweda, Rhonda Bassel-Duby, Eric N. Olson
Summary: New evidence suggests that certain noncoding RNA molecules actually code for microproteins. By using comparative genomics, researchers identified a microprotein called Mtlbn that is abundantly expressed in the heart. Mtlbn localizes to the inner mitochondrial membrane and interacts with subunits of the electron transport chain and respiratory supercomplexes.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Oncology
Arvinder Kapur, Pooja Mehta, Aaron D. Simmons, Spencer S. Ericksen, Geeta Mehta, Sean P. Palecek, Mildred Felder, Zach Stenerson, Amruta Nayak, Jose Maria Ayuso Dominguez, Manish Patankar, Lisa M. Barroilhet
Summary: The Warburg effect, characterized by high glucose uptake and lactate release, is a hallmark of most cancers. Atovaquone, a mitochondrial complex III inhibitor, has been found to slow ovarian cancer growth and inhibit the proliferation of cancer stem cells both in vitro and in vivo. The drug is able to inhibit oxygen consumption and ATP production, and it alters several metabolic pathways. These findings provide mechanistic insights and preclinical data to support further investigation of atovaquone as a potential therapy for gynecologic cancers.
Article
Biochemistry & Molecular Biology
Guilherme Henrique Souza Bomfim, Marta Giacomello, Rodrigo S. Lacruz
Summary: Enamel formation is a two-step process, with crystal growth during the secretory stage and significant expansion during the maturation stage. Plasma membrane Ca2+-ATPases (PMCA1-4) are likely involved in calcium extrusion in ameloblasts, especially in the maturation stage. Functional analysis showed that PMCA activity is more efficient in maturation ameloblasts.
Article
Biochemistry & Molecular Biology
Thimios A. Mitsiadis, Pierfrancesco Pagella, Terence D. Capellini, Moya Meredith Smith
Summary: The Notch pathway is involved in cell fate specification and embryonic development. The Jagged2 gene, which encodes a ligand for Notch receptors, is expressed early in tooth development. Mutations in Jagged2 result in abnormal tooth morphology and impaired enamel deposition. The Notch signaling cascade is disrupted in Jagged2 mutant teeth, leading to dental structures more reminiscent of fish enamel. We propose that the increased number of Notch homologues in metazoa enabled the formation and maintenance of distinctive cell fates within organs and tissues during evolution.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2023)
Review
Cell Biology
Katarzyna Glombik, Jan Detka, Boguslawa Budziszewska
Summary: Hormones play a crucial role in regulating neuronal and synaptic plasticity, neurogenesis, and metabolic activity in the brain, ensuring proper communication between the brain and the body. The influence of hormones on energy metabolism and mitochondrial function in the brain has gained significant attention, as excess or deficiency of hormones is associated with cell damage and loss of function in mitochondria.
Article
Psychiatry
Stavroula Giannoulis, Meghan J. Chenoweth, Paulo Saquilayan, Rachel F. Tyndale, Caryn Lerman, James L. Kennedy, Laurie Zawertailo, Vanessa Goncalves
Summary: This study suggests that mitochondrial genetic variation may contribute to variability in smoking phenotypes, but replication in larger samples is required.
PSYCHIATRY RESEARCH
(2022)
Review
Biochemistry & Molecular Biology
Elena Obrador, Rosario Salvador-Palmer, Rafael Lopez-Blanch, ALi Jihad-Jebbar, Soraya L. Valles, Jose M. Estrela
Summary: Amyotrophic lateral sclerosis (ALS) is a common neurodegenerative disease characterized by motor neuron degeneration leading to muscle weakness and paralysis. Mitochondrial dysfunction plays a significant role in ALS pathophysiology, affecting disease progression. Research indicates that targeting mitochondrial biology may offer potential therapeutic approaches to slow down ALS progression.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Environmental Sciences
Peng Xiao, Xuan Liu, He Zhang, Wenhua Li
Summary: Chronic exposure to isoflucypram is associated with reproductive and intestinal dysfunction in adult zebrafish. Isoflucypram exposure causes irregular swelling of intestinal villi and alterations in the ultrastructure of intestinal mitochondria in zebrafish.
ENVIRONMENTAL POLLUTION
(2022)
Article
Physiology
Jake Ngu, Antonius L. J. J. Bronckers, Kaitlin Katsura, Yan Zhang, Pamela K. Den Besten
Summary: Enamel mineralization requires calcium transport into the extracellular matrix, and removal of Na+ from the enamel matrix in exchange for Ca2+ and K+ through the calcium exchanger Nckx4. This study identified Kir4.2 as the most upregulated K+ transporter in the maturation stage of enamel organs. The results suggest that Kir4.2 participates in K+ uptake by maturation ameloblasts, and that the uptake of K+ and Na+ may be regulated by pH through WDR72-mediated endocytosis and membrane trafficking.
FRONTIERS IN PHYSIOLOGY
(2023)
Review
Endocrinology & Metabolism
Yang Xu, Colin K. L. Phoon, Mindong Ren, Michael Schlame
Summary: Cardiolipin helps reduce packing stress in the inner mitochondrial membrane, alleviating issues caused by protein crowding. Patients with Barth syndrome are unable to concentrate oxidative phosphorylation proteins to a high surface density in the inner mitochondrial membrane, leading to an inability to meet peak energy demands.
JOURNAL OF INHERITED METABOLIC DISEASE
(2022)
Article
Environmental Sciences
Lin Zeng, Wen-Cheng Li, Hui Zhang, Ping Cao, Chun-Xiang Ai, Bing Hu, Wei Song
Summary: This research found that pre-hypoxia exposure improved the mitochondrial energy metabolism of large yellow croaker under Cu stress, reducing ROS production and enhancing OXPHOS efficiency for adaptive responses.
ECOTOXICOLOGY AND ENVIRONMENTAL SAFETY
(2021)
Article
Biochemistry & Molecular Biology
Veronica Costiniti, Guilherme H. S. Bomfim, Maria Neginskaya, Ga-Yeon Son, Erna Mitaishvili, Marta Giacomello, Evgeny Pavlov, Rodrigo S. Lacruz
Summary: This study analyzed the functional features of mitochondria in rat ameloblasts and found an increased mitochondrial Ca2+ handling in the maturation stage, particularly in response to Ca2+ influx via store-operated Ca2+ entry (SOCE). These findings provide insights into the role of mitochondria in enamel and suggest that SOCE and mitochondrial Ca2+ uptake are complementary processes in biological mineralization.
Article
Cell Biology
Caterina Vianello, Veronica Cocetta, Daniela Catanzaro, Gerald W. I. I. I. I. Dorn, Angelo De Milito, Flavio Rizzolio, Vincenzo Canzonieri, Erika Cecchin, Rossana Roncato, Giuseppe Toffoli, Vincenzo Quagliariello, Annabella Di Mauro, Simona Losito, Nicola Maurea, Scaffa Cono, Gabriele Sales, Luca Scorrano, Marta Giacomello, Monica Montopoli
Summary: This study found that mitochondrial morphology and autophagy are altered in cisplatin resistant cancer cell lines, and the levels of the mitophagy receptor BNIP3 are elevated. Inhibiting BNIP3-driven mitophagy may be a potential therapeutic strategy to counteract cisplatin resistance.
CELL DEATH & DISEASE
(2022)
Review
Biochemistry & Molecular Biology
Francesca Grespi, Caterina Vianello, Stefano Cagnin, Marta Giacomello, Agnese De Mario
Summary: Gliomas are heterogeneous neoplasms with different malignancies and molecular hallmarks. Glioblastoma (GB) is the highest grade of glioma and its clinical outcome is poor. Recent studies show that the reshaping of the cytoskeleton and the extent and function of mitochondria-ER contact sites (MERCs) are associated with GB progression. A meta-analysis comparing different grade GB patients reveals alterations in cytoskeletal and MERCs related genes in high-grade GB. Further analysis may provide new insights into the molecular etiopathogenesis of GB and identify potential biomarkers/targets for improved therapy.
Editorial Material
Biochemistry & Molecular Biology
Tomas Knedlik, Marta Giacomello
Article
Biochemistry & Molecular Biology
Guilherme Henrique Souza Bomfim, Marta Giacomello, Rodrigo S. Lacruz
Summary: Enamel formation is a two-step process, with crystal growth during the secretory stage and significant expansion during the maturation stage. Plasma membrane Ca2+-ATPases (PMCA1-4) are likely involved in calcium extrusion in ameloblasts, especially in the maturation stage. Functional analysis showed that PMCA activity is more efficient in maturation ameloblasts.
Article
Cell Biology
Yi Li, Veronica Costiniti, Guilherme H. Souza Bomfim, Maria Neginskaya, Ga-Yeon Son, Beverly Rothermel, Evgeny Pavlov, Rodrigo S. Lacruz
Summary: This study investigates the role of RCAN1 in enamel formation by overexpressing RCAN1 in ameloblast cells. The study shows that overexpressing RCAN1 leads to increased reactive oxygen species, decreased mitochondrial bioenergetics, and decreased expression of enamel-specific genes. These findings suggest that elevated levels of RCAN1 in ameloblasts of individuals with DS may impact enamel formation by altering the redox environment, mitochondrial function, and enamel-specific gene expression.
Article
Cell Biology
Francesco Chemello, Michela Pozzobon, Lorenza Iolanda Tsansizi, Tatiana Varanita, Ruben Quintana-Cabrera, Daniele Bonesso, Martina Piccoli, Gerolamo Lanfranchi, Marta Giacomello, Luca Scorrano, Camilla Bean
Summary: Approved gene therapies for spinal muscular atrophy (SMA) improve SMA natural history but are not curative. Loss of survival motor neuron 1 (SMN1) has detrimental effects in muscle beyond motor neurons, leading to accumulation of dysfunctional mitochondria. Amniotic fluid stem cells transplantation can restore mitochondrial morphology and expression of mitochondrial genes in SMA mouse model, suggesting that targeting muscle mitochondrial dysfunction may complement current gene therapy for SMA.
CELL DEATH & DISEASE
(2023)
Article
Cell Biology
Caterina Vianello, Federica Dal Bello, Sang Hun Shin, Sara Schiavon, Camilla Bean, Ana Paula Magalhaes Rebelo, Tomas Knedlik, Emad Norouzi Esfahani, Veronica Costiniti, Rodrigo S. Lacruz, Giuseppina Covello, Fabio Munari, Tommaso Scolaro, Antonella Viola, Elena Rampazzo, Luca Persano, Sara Zumerle, Luca Scorrano, Alessio Gianelle, Marta Giacomello
Summary: Recent studies have found a connection between changes in mitochondria physiology and cellular pathophysiological mechanisms, and the function of these organelles needs to be validated. However, a method for high-throughput quantification of mitochondrial membrane potential has not been established yet. In this study, we present a methodology for unbiased quantification of mitochondrial membrane potential in vitro, suitable for various cell models. Our method successfully analyzed mitochondrial membrane potential in different cell types and allowed discrimination between cell subpopulations using automated image analysis and machine learning.
Article
Biochemistry & Molecular Biology
Stefano Cagnin, Tomas Knedlik, Caterina Vianello, Ana Paula Magalhaes Rebelo, Agnese De Mario, Marta Giacomello
Summary: Neuroblastoma is a common childhood tumor that accounts for 15% of pediatric cancer deaths, but the mechanisms and genes involved in its progression are unknown. In this study, we analyzed the gene expression differences between neuroblastoma stage 1 and stage 4 + 4S to identify biological processes associated with its progression. The results suggest that alterations in mitochondria function and mitochondria-ER contact sites may be involved in the progression of neuroblastoma.
Article
Physiology
Guilherme Henrique Souza Bomfim, Erna Mitaishvili, Paul Schnetkamp, Rodrigo Lacruz
Summary: This study analyzed the function of NCX and NCKX exchangers in rat ameloblasts and compared their efficacy in the two main stages of enamel formation. The results showed that NCKX is more important than NCX in ameloblasts and plays a crucial role in the Ca2+ homeostasis during the maturation stage.
JOURNAL OF GENERAL PHYSIOLOGY
(2023)
Correction
Endocrinology & Metabolism
Camilla Bean, Matteo Audano, Tatiana Varanita, Francesca Favaretto, Marta Medaglia, Marco Gerdol, Lena Pernas, Fabio Stasi, Marta Giacomello, Stephanie Herkenne, Maheswary Muniandy, Sini Heinonen, Emma Cazaly, Miina Ollikainen, Gabriella Milan, Alberto Pallavicini, Kirsi H. Pietilainen, Roberto Vettor, Nico Mitro, Luca Scorrano