Article
Biochemistry & Molecular Biology
Audrey-Ann Dumont, Lauralyne Dumont, Delong Zhou, Hugo Giguere, Chantal Pileggi, Mary-Ellen Harper, Denis P. Blondin, Michelle S. Scott, Mannix Auger-Messier
Summary: The study demonstrates the importance of Srsf3 in maintaining mitochondrial integrity during cardiac development, with Srsf3 deletion leading to severe heart remodeling and cardiomyocyte enlargement.
Review
Biochemistry & Molecular Biology
Margherita Protasoni, Massimo Zeviani
Summary: Mitochondria are intracellular organelles responsible for energy production in eukaryotic cells, with growing interest due to their association with various pathologies. Dysfunction of mitochondria can lead to a wide range of clinical phenotypes, particularly affecting tissues with high-energy demand. Mitochondrial diseases are genetically heterogeneous conditions, making it difficult to identify common causes and potential therapeutic targets.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Erika Fernandez-Vizarra, Massimo Zeviani
Summary: Mitochondrial disorders are common inborn errors of metabolism, primarily due to dysfunction of the oxidative phosphorylation system (OXPHOS). Around half of diagnosed cases have a known genetic cause, often involving pathogenic variants in genes encoding structural subunits or factors directly involved in ETC assembly.
Review
Cell Biology
Janne Purhonen, Juha Klefstrom, Jukka Kallijarvi
Summary: This article explores the expression of MYC in mitochondrial diseases and its regulatory role in metabolic pathways. The upregulation of MYC in mitochondrial diseases may be triggered by OXPHOS deficiency and has important implications for the pathogenesis of these diseases.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2023)
Article
Cell Biology
David R. L. Robinson, Daniella H. Hock, Linden Muellner-Wong, Roopasingam Kugapreethan, Boris Reljic, Elliot E. Surgenor, Carlos H. M. Rodrigues, Nikeisha J. Caruana, David A. Stroud
Summary: Mitochondria are complex organelles with diverse proteins that play different roles in the cell. Mutations causing mitochondrial disease can disrupt the assembly of protein complexes and destabilize the interactors, leading to functional defects.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Hatice Kumru, Loreto Garcia-Alen, Aina Ros-Alsina, Sergiu Albu, Margarita Valles, Joan Vidal
Summary: This study aimed to investigate whether transcutaneous spinal cord stimulation (tSCS) could improve respiratory function in individuals with cervical spinal cord injury (cSCI). The results showed that subjects who received tSCS combined with inspiratory muscle training (IMT) experienced significant improvements in breathlessness/dyspnea, hypophonia, maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), and forced vital capacity. Therefore, tSCS may serve as an effective adjunctive therapy for respiratory impairments following SCI.
Review
Cell Biology
Kunwar Somesh Vikramdeo, Sarabjeet Kour Sudan, Ajay P. Singh, Seema Singh, Santanu Dasgupta
Summary: Mitochondria play a crucial role in cellular energy production and signaling pathways, and alterations in respiratory complexes can lead to mitochondrial disorders and cancer development.
JOURNAL OF CELLULAR PHYSIOLOGY
(2022)
Article
Genetics & Heredity
Michele Brischigliaro, Elena Frigo, Samantha Corra, Cristiano De Pitta, Ildiko Szabo, Massimo Zeviani, Rodolfo Costa
Summary: Mutations in BCS1L are a common cause of human mitochondrial disease related to complex III deficiency, with diverse clinical symptoms and multisystem involvement. Studying genetic manipulation of Bcs1 in fruit flies reveals its fundamental role in complex III biogenesis and offers novel models for BCS1L-related human mitochondrial diseases.
JOURNAL OF MOLECULAR MEDICINE-JMM
(2021)
Review
Biochemistry & Molecular Biology
Paula G. Slater, Miguel E. Dominguez-Romero, Maximiliano Villarreal, Veronica Eisner, Juan Larrain
Summary: Spinal cord injury (SCI) causes paralysis in many people worldwide, with no current full functional recovery therapies available. Mitochondria play a crucial role in cellular responses after SCI, such as cell death, immune response, axon regeneration, and neural stem cell self-renewal and differentiation. Further investigation into mitochondrial function and regulation is needed to identify potential targets for SCI therapeutic intervention.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2022)
Article
Food Science & Technology
Jun Wang, Xue Tang, Yipin Lu, Yingying Zheng, Fanhang Zeng, Wentao Shi, Peng Zhou
Summary: The study demonstrates that lycopene can alleviate myocardial dysfunction and energy metabolism issues induced by Dityrosine, by improving mitochondrial performance and reducing oxidative stress to enhance heart health.
MOLECULAR NUTRITION & FOOD RESEARCH
(2022)
Article
Clinical Neurology
Carola Hedberg-Oldfors, Ulrika Lindgren, Kittichate Visuttijai, Daniel Loof, Sara Roos, Christer Thomsen, Anders Oldfors
Summary: Patients with dermatomyositis (DM) have reduced aerobic metabolism and impaired muscle function due to mitochondrial respiratory chain dysfunction. This study found that the deficiency of complexes I and IV is a major cause of the disease, while complex II is unaffected. Depletion of mitochondrial RNA and a decrease in mtDNA copy number in affected muscle regions contribute to the muscle pathology and disturbed aerobic metabolism.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Maria Patron, Daryna Tarasenko, Hendrik Nolte, Lara Kroczek, Mausumi Ghosh, Yohsuke Ohba, Yvonne Lasarzewski, Zeinab Alsadat Ahmadi, Alfredo Cabrera-Orefice, Akinori Eyiama, Tim Kellermann, Elena Rugarli, Ulrich Brandt, Michael Meinecke, Thomas Langer
Summary: Mitochondrial proteases, specifically the m-AAA protease AFG3L2, are regulated by the mitochondrial proton gradient and play a crucial role in reshaping the mitochondrial proteome in response to different energetic demands. TMBIM5, a Ca2+/H+ exchanger in the mitochondrial inner membrane, inhibits the activity of the m-AAA protease and ensures cell survival and respiration. However, persistent hyperpolarization triggers the degradation of TMBIM5 and activation of the m-AAA protease, which facilitates the proteolytic breakdown of respiratory complex I to limit ROS production in hyperpolarized mitochondria.
Article
Cell Biology
Pedro Latorre-Muro, Katherine E. O'Malley, Christopher F. Bennett, Elizabeth A. Perry, Eduardo Balsa, Clint D. J. Tavares, Mark Jedrychowski, Steven P. Gygi, Pere Puigserver
Summary: The study reveals a PERK-OGT-TOM70 axis that regulates mitochondrial protein import and cristae formation, ultimately increasing cell respiration. This mechanism also affects mitochondrial structure and plays a crucial role in cellular adaptations to stress conditions.
Article
Nanoscience & Nanotechnology
Yi Li, Liangliang Yang, Fei Hu, Ji Xu, Junsong Ye, Shuhua Liu, Lifeng Wang, Ming Zhuo, Bing Ran, Hongyu Zhang, Junming Ye, Jian Xiao
Summary: This study presents a promising bioactive system for the treatment of spinal cord injury (SCI) by immobilizing umbilical cord mesenchymal stem cells (UCMSC) and basic fibroblast growth factor (bFGF) in extracellular matrix (ECM) and heparin-poloxamer (HP). The results show that this system has therapeutic effects in reducing apoptosis and improving mitochondrial function, and it activates PAK1 and SIRT4 to protect against SCI.
ACS APPLIED MATERIALS & INTERFACES
(2022)
Review
Biochemistry & Molecular Biology
Andreas Kohler, Antoni Barrientos, Flavia Fontanesi, Martin Ott
Summary: The mitochondrial respiratory chain is an important system for energy conversion in eukaryotic cells. The organization of respiratory chain complexes into supercomplexes provides advantages for cellular energy conversion, although its universality is still debated. Further research is needed to fully understand the functional significance of these supercomplexes.