Functions of mitochondria

Mitochondria are one of the major ancient endomembrane systems in eukaryotic cells. Owing to their ability to produce ATP through respiration, they became a driving force in evolution. As an essential step in the process of eukaryotic evolution, the size of the mitochondrial chromosome was drastically reduced, and the behaviour of mitochondria within eukaryotic cells radically changed. Recent advances have revealed how the organelle's behaviour has evolved to allow the accurate transmission of its genome and to become responsive to the needs of the cell and its own dysfunction. Open Access articles citing this article. • • Qianqian Ding • , Xianji Liu • … Suk Ying Tsang Stem Cell Research & Therapy Open Access 07 June 2023 • • Lilia Sanchez • , Jessica Epps • … Weston W. Porter Cell Death & Differentiation Open Access 25 March 2023 • • Shiao Tong • , Minqi Xia • … Daofeng Tian Journal of Translational Medicine Open Access 22 February 2023 Access options • Lane, N. & Martin, W. The energetics of genome complexity. Nature 467, 929–934 (2010). • Gabaldón, T. & Huynen, M. A. Shaping the mitochondrial proteome. Biochim. Biophys. Acta 1659, 212–220 (2004). • Baughman, J. M. et al. Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter. Nature 476, 341–345 (2011). • De Stefani, D., Raffaello, A., Teardo, E., Szabo, I. & Rizzuto, R. A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter. Nature 476, 336...

Table of Contents • • • • • • • • • • • • • • • • • • • • • • • • • • • • • Introduction to Mitochondria The mitochondria are critically vital organelles, second only to the nucleus, whose functions are required for cell viability. The major function of the mitochondria is to generate the high energy molecule ATP, through the utilization of the energy contained in the reduced electron carriers, NADH and FADH 2. This vital process is explained in detail in the Mitochondria are surrounded by a double-membrane system, consisting of inner and outer mitochondrial membranes separated by an intermembrane space. The inner membrane forms numerous folds (cristae), which extend into the interior (or matrix) of the organelle. Although depicted as linear molecules in the Figure below, the mitochondrial genome (mtDNA) is a circular double-stranded molecule of DNA. Cross-sectional view of a typical mitochondrion The outer mitochondrial membrane is in contact with membranes of the endoplasmic reticulum (ER) at sites that are termed ER-mitochondria contact sites, ERMCS. The ERMCS is crucial for exchanging biological molecules between these two organelles. A critical protein in the outer mitochondrial membrane that is involved in forming the ERMCS is mitoguardin-2 (MIGA2). Crucial functions of the ERMCS are the regulation of mitochondrial metabolism and regulation of Ca 2+ signaling. The ERMCS plays an important role the maintenance of lipid homeostasis by facilitating local lipid synthesis...

Mitochondria Mitochondria Definition “Mitochondria are membrane-bound organelles present in the cytoplasm of all eukaryotic cells, that produce adenosine triphosphate (ATP), the main energy molecule used by the cell.” Table of Contents • • • • • Read on to explore what is mitochondria, its structure, and functions. What are Mitochondria? Popularly known as the “ Powerhouse of the cell,” mitochondria (singular: mitochondrion) are a double membrane-bound organelle found in most eukaryotic organisms. They are found inside the cytoplasm and essentially function as the cell’s “digestive system.” They play a major role in breaking down nutrients and generating energy-rich molecules for the cell. Many of the biochemical reactions involved in cellular respiration take place within the mitochondria. The term ‘mitochondrion’ is derived from the Greek words “ mitos” and “ chondrion” which means “ thread” and “ granules-like”, respectively. It was first described by a German pathologist named Richard Altmann in the year 1890. Also refer: Structure of Mitochondria • The mitochondrion is a double-membraned, rod-shaped structure found in both plant and • Its size ranges from 0.5 to 1.0 micrometre in diameter. • The structure comprises an outer membrane, an inner membrane, and a gel-like material called the matrix. • The outer membrane and the inner membrane are made of proteins and phospholipid layers separated by the intermembrane space. • The outer membrane co...

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The mitochondrial oxidative phosphorylation system is central to cellular metabolism. It comprises five enzymatic complexes and two mobile electron carriers that work in a mitochondrial respiratory chain. By coupling the oxidation of reducing equivalents coming into mitochondria to the generation and subsequent dissipation of a proton gradient across the inner mitochondrial membrane, this electron transport chain drives the production of ATP, which is then used as a primary energy carrier in virtually all cellular processes. Minimal perturbations of the respiratory chain activity are linked to diseases; therefore, it is necessary to understand how these complexes are assembled and regulated and how they function. In this Review, we outline the latest assembly models for each individual complex, and we also highlight the recent discoveries indicating that the formation of larger assemblies, known as respiratory supercomplexes, originates from the association of the intermediates of individual complexes. We then discuss how recent cryo-electron microscopy structures have been key to answering open questions on the function of the electron transport chain in mitochondrial respiration and how supercomplexes and other factors, including metabolites, can regulate the activity of the single complexes. When relevant, we discuss how these mechanisms contribute to physiology and outline their deregulation in human diseases. Open Access articles citing this article. • • Letizia Amado...

Mitochondria are essential intracellular organelles whose central role in maintaining energy homeostasis places them at the heart of cell integrity, function and survival. As the reliable provision of energy is so fundamental to every aspect of cellular function, mitochondrial dysfunction inevitably has devastating implications for the cell, the tissue and the organism. This is especially critical in the nervous system, where subtle changes in signalling and function can have catastrophic global consequences. Further, as postmitotic cells are heavily dependent on oxidative phosphorylation and are morphologically enormously complex, neurons pose a unique set of challenges for the mitochondrial population that reside within them. Mitochondrial dysfunction has profound consequences for the nervous system and is implicated in a host of neurological and neurodegenerative diseases. The following chapter introduces the form and function of these fascinating organelles and introduces key concepts and vulnerabilities that may underlie their involvement in neurodegenerative conditions. This introduction lays the foundation for the following chapters, which will explore specific aspects of the roles of these organelles in a range of neurodegenerative disease. Keywords • Mitochondria • Mitochondrial function • mtDNA genetics • Neurodegeneration • Margulis L, editor. Origin of eukaryotic cells. New Haven: Yale University Press; 1970. • Martin W, Mentel M. The origin of mitochondria. Na...

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Mitochondria are essential intracellular organelles whose central role in maintaining energy homeostasis places them at the heart of cell integrity, function and survival. As the reliable provision of energy is so fundamental to every aspect of cellular function, mitochondrial dysfunction inevitably has devastating implications for the cell, the tissue and the organism. This is especially critical in the nervous system, where subtle changes in signalling and function can have catastrophic global consequences. Further, as postmitotic cells are heavily dependent on oxidative phosphorylation and are morphologically enormously complex, neurons pose a unique set of challenges for the mitochondrial population that reside within them. Mitochondrial dysfunction has profound consequences for the nervous system and is implicated in a host of neurological and neurodegenerative diseases. The following chapter introduces the form and function of these fascinating organelles and introduces key concepts and vulnerabilities that may underlie their involvement in neurodegenerative conditions. This introduction lays the foundation for the following chapters, which will explore specific aspects of the roles of these organelles in a range of neurodegenerative disease. Keywords • Mitochondria • Mitochondrial function • mtDNA genetics • Neurodegeneration • Margulis L, editor. Origin of eukaryotic cells. New Haven: Yale University Press; 1970. • Martin W, Mentel M. The origin of mitochondria. Na...

Mitochondria are one of the major ancient endomembrane systems in eukaryotic cells. Owing to their ability to produce ATP through respiration, they became a driving force in evolution. As an essential step in the process of eukaryotic evolution, the size of the mitochondrial chromosome was drastically reduced, and the behaviour of mitochondria within eukaryotic cells radically changed. Recent advances have revealed how the organelle's behaviour has evolved to allow the accurate transmission of its genome and to become responsive to the needs of the cell and its own dysfunction. Open Access articles citing this article. • • Qianqian Ding • , Xianji Liu • … Suk Ying Tsang Stem Cell Research & Therapy Open Access 07 June 2023 • • Lilia Sanchez • , Jessica Epps • … Weston W. Porter Cell Death & Differentiation Open Access 25 March 2023 • • Shiao Tong • , Minqi Xia • … Daofeng Tian Journal of Translational Medicine Open Access 22 February 2023 Access options • Lane, N. & Martin, W. The energetics of genome complexity. Nature 467, 929–934 (2010). • Gabaldón, T. & Huynen, M. A. Shaping the mitochondrial proteome. Biochim. Biophys. Acta 1659, 212–220 (2004). • Baughman, J. M. et al. Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter. Nature 476, 341–345 (2011). • De Stefani, D., Raffaello, A., Teardo, E., Szabo, I. & Rizzuto, R. A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter. Nature 476, 336...

Mitochondria Mitochondria Definition “Mitochondria are membrane-bound organelles present in the cytoplasm of all eukaryotic cells, that produce adenosine triphosphate (ATP), the main energy molecule used by the cell.” Table of Contents • • • • • Read on to explore what is mitochondria, its structure, and functions. What are Mitochondria? Popularly known as the “ Powerhouse of the cell,” mitochondria (singular: mitochondrion) are a double membrane-bound organelle found in most eukaryotic organisms. They are found inside the cytoplasm and essentially function as the cell’s “digestive system.” They play a major role in breaking down nutrients and generating energy-rich molecules for the cell. Many of the biochemical reactions involved in cellular respiration take place within the mitochondria. The term ‘mitochondrion’ is derived from the Greek words “ mitos” and “ chondrion” which means “ thread” and “ granules-like”, respectively. It was first described by a German pathologist named Richard Altmann in the year 1890. Also refer: Structure of Mitochondria • The mitochondrion is a double-membraned, rod-shaped structure found in both plant and • Its size ranges from 0.5 to 1.0 micrometre in diameter. • The structure comprises an outer membrane, an inner membrane, and a gel-like material called the matrix. • The outer membrane and the inner membrane are made of proteins and phospholipid layers separated by the intermembrane space. • The outer membrane co...