Article
Cardiac & Cardiovascular Systems
Yu-Han Chen, Albert P. Ta, Yumay Chen, Hsiao-Chen Lee, Wenjun Fan, Phang-Lang Chen, Maria C. Jordan, Kenneth P. Roos, Grant R. MacGregor, Qin Yang, Robert A. Edwards, Junfeng Li, Ping H. Wang
Summary: The study aimed to investigate the effect of mitochondrial AKT signaling on cardiac structure and function. The results showed that disrupted mitochondrial AKT signaling led to cardiomyopathy with cardiac fibrosis, left ventricular hypertrophy, and dysfunction. On the other hand, activation of mitochondrial AKT1 protected against diabetic cardiomyopathy and improved overall metabolism. These findings highlight the importance of mitochondrial AKT signaling in maintaining cardiac health.
CARDIOVASCULAR DIABETOLOGY
(2023)
Article
Microbiology
Madelaine M. Usey, Diego Huet
Summary: Coiled-coil-helix-coiled-coil-helix (CHCH) domains play important roles in the biogenesis and function of mitochondria. Recent investigations found CHCH domain-containing subunits in the Toxoplasma gondii ATP synthase, but their function was unclear. This study demonstrates the essential role of two T. gondii ATP synthase subunits containing CHCH domains in parasite survival and ATP synthase stability and function. Knockdown of these subunits disrupts multiple aspects of mitochondrial morphology and mutation of key residues in the CHCH domains causes protein mis-localization.
Article
Biology
Jingruo Zhang, Manuel A. Riquelme, Rui Hua, Francisca M. Acosta, Sumin Gu, Jean X. Jiang
Summary: This study reveals a new role of mitochondrial Cx43 and hemichannels in modulating mitochondrial function and homeostasis in bone osteocytes under oxidative stress. Through mediating K+, H+, and ATP transfer across the mitochondrial inner membrane and interacting with mitochondrial ATP synthase, mtCx43 hemichannels regulate mitochondrial ATP generation and contribute to the maintenance of mitochondrial redox levels in response to oxidative stress.
Article
Biology
Pei-Heng Jiang, Chen-Yan Hou, Shu-Chun Teng
Summary: The study reveals that Ids2 is a cochaperone essential for maintaining mitochondrial DNA stability and the proteostasis of the electron transport chain. It recruits Atp3, a subunit of complex V ATP synthase, to the folding system. Deletion of IDS2 or Atp3 leads to loss of mitochondrial DNA and Yme1 plays a critical role in maintaining Atp3 protein level.
Review
Biochemistry & Molecular Biology
Cheng Huang, Kun Deng, Minghua Wu
Summary: Mitochondria are vital for energy metabolism and their structure is influenced by mitochondrial dynamics and cristae remodeling. This review focuses on key regulators of cristae structure, including the mitochondrial contact site and cristae organizing system, optic atrophy-1, mitochondrial calcium uniporter, and ATP synthase. Dysfunction or deletion of these regulators can lead to abnormal cristae morphology, impacting cellular respiration and contributing to diseases such as Parkinson's disease, Leigh syndrome, and dominant optic atrophy.
INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES
(2023)
Article
Cell Biology
Brad Ebanks, Gunjan Katyal, Chris Taylor, Adam Dowle, Chiara Papetti, Magnus Lucassen, Nicoleta Moisoi, Lisa Chakrabarti
Summary: This study investigated the interaction of mitochondrial haemoglobin with ATP synthase and its associated proteins, and found upregulated expression of mitochondrial haemoglobin in response to hypoxia. The results suggest that mitochondrial pH and ATP synthase activity are involved in the mitochondrial haemoglobin response to hypoxia.
Article
Biochemistry & Molecular Biology
Silvia Garcia-Catalan, Luis Gonzalez-Moreno, Araceli del Arco
Summary: Research has found two variants of ATP-Mg2+/Pi carriers in unicellular eukaryotes, with a common origin unrelated to ADP/ATP translocases, suggesting recurrent losses of the regulatory module in different phyla. These truncated variants of SCaMC are predominantly found in parasitic protists and green algae, indicating a potential relationship with specific lifestyles. Additionally, these variants exhibit intricate structural diversity that may be associated with their pathogenicity.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH
(2021)
Article
Biology
Maura Greiser, Mariusz Karbowski, Aaron David Kaplan, Andrew Kyle Coleman, Nicolas Verhoeven, Carmen A. Mannella, W. Jonathan Lederer, Liron Boyman
Summary: Mitochondrial ATP production in ventricular cardiomyocytes is regulated by the CO2/bicarbonate signaling cascade activated by soluble adenylyl cyclase (sAC), which is primarily located inside the mitochondria. Activation of sAC by bicarbonate generates cAMP, which activates local EPAC1 and subsequently turns on Rap1, increasing mitochondrial ATP production. This bicarbonate- and sAC-mediated signaling process works independently of, yet in conjunction with, [Ca2+](m)-dependent ATP production in ventricular cardiomyocytes.
Article
Biochemistry & Molecular Biology
Guangying Yang, Yuanyuan Ding, Xiaohui Shang, Tong Zhao, Shan Lu, Jinghan Tian, Jun Weng, Xiaomei Zeng
Summary: This study revealed that the chaperones Atp23p and Atp10p work together to regulate the assembly of mitochondrial ATP synthase in yeast. By increasing the expression level of Atp23p, the deficiency of ATP10 was partially complemented by stabilizing some subunit 6 of the synthase.
Article
Multidisciplinary Sciences
Jiyao Song, Liesa Steidle, Isabelle Steymans, Jasjot Singh, Anne Sanner, Lena Boettinger, Dominic Winter, Thomas Becker
Summary: Mitochondrial Hsp70 plays dual roles in the formation of ATP synthase. It cooperates with Atp11 and Atp12 to form the F-1 domain, and it transfers Atp5 to link the catalytic head with the peripheral stalk. Inactivation of mtHsp70 leads to integration of assembly-defective Atp5 variants into the mature complex, indicating its quality control function.
NATURE COMMUNICATIONS
(2023)
Review
Biochemistry & Molecular Biology
Somya Patro, Sujay Ratna, Hianny A. Yamamoto, Andrew T. Ebenezer, Dillon S. Ferguson, Amanpreet Kaur, Brendan C. McIntyre, Ryan Snow, Maria E. Solesio
Summary: Alzheimer's Disease is the most common neurodegenerative disorder in society, with increasing incidence expected as the population ages. Mitochondrial dysfunction has been widely described in the brains of AD patients, and may act as a trigger for early events in the disease. Modulating ATP synthase pharmacologically could be a potential strategy to prevent mitochondrial dysfunction in AD.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Till Klecker, Benedikt Westermann
Summary: Mitochondria are complex organelles with two membranes, characterized by characteristic folds of the inner membrane called cristae. Recent studies have identified four major pathways that cooperate to shape cristae membranes, including ATP synthase dimer formation, assembly of MICOS, inner membrane remodelling by GTPase, and modulation of mitochondrial lipid composition. These pathways play a crucial role in establishing mitochondrial membrane architecture.
Article
Biochemistry & Molecular Biology
Inayathulla Mohammed, Kai A. Schmitz, Niko Schenck, Dimitrios Balasopoulos, Annika Topitsch, Timm Maier, Jan Pieter Abrahams
Summary: The mitochondrial Lon protease, LonP1, plays a crucial role in maintaining mitochondrial health by removing redundant proteins from the mitochondrial matrix. Cryo-EM analysis revealed eight nucleotide-dependent conformational states of LonP1. The study also showed how sequential ATP hydrolysis controls the translocation of substrate proteins in a 6-fold binding change mechanism.
Article
Environmental Sciences
Libing Yu, Wenjing Li, Jian Chu, Chun Chen, Xijian Li, Wei Tang, Binyuan Xia, Zhonghua Xiong
Summary: Uranium as an emerging pollutant presents serious threats to ecological and human health, with the kidney identified as a major deposition site and sensitive target organ for its toxicity, which is associated with oxidative stress and mitochondrial respiration. This study found that cytochrome c oxidase and ATP synthase play key roles in uranium-induced toxicity by inhibiting their activities in the mitochondrial respiration pathway. These inhibitory effects could explain the generation of reactive oxygen species and mitochondrial alterations observed in uranium poisoning.
ENVIRONMENTAL POLLUTION
(2021)
Article
Cell Biology
Heike Rampelt, Florian Wollweber, Mariya Licheva, Rinse de Boer, Inge Perschil, Liesa Steidle, Thomas Becker, Maria Bohnert, Ida van der Klei, Claudine Kraft, Martin van der Laan, Nikolaus Pfanner
Summary: Cristae, invaginations of the mitochondrial inner membrane, are important for oxidative phosphorylation. The study reveals that Mic10, a subunit of MICOS, also interacts with the F1Fo-ATP synthase, and it plays distinct roles in cristae shaping and respiratory adaptation and growth.