• Mitochondrial carriers are proteins from solute carrier family 25 which transfer molecules across the membranes of the mitochondria. (wikipedia.org)
  • Defects of mitochondrial carriers that supply mitochondria with the substrates of oxidative phosphorylation, inorganic phosphate and ADP, are responsible for diseases characterized by defective energy production. (wikipedia.org)
  • TC# 2.A.29.1.1) that is responsible for importing ADP into the mitochondria and exporting ATP out of the mitochondria and into the cytosol following synthesis. (wikipedia.org)
  • The translocase is among the most abundant proteins in the mitochondrial internal membrane.2 Its abundance as well as the need for this transporter possess rendered it one of the better characterized members from the mitochondrial carrier (MC) family members which facilitate the motion of a variety of metabolites in and out of mitochondria.3 The translocase comes with an intimate relationship with an integral lipid in mitochondrial membranes cardiolipin (CL). (hiv-proteases.com)
  • The difficulty in understanding and treating human pathologies caused by mitochondrial dysfunction arises from the complex relationships between mitochondria and other cellular processes, as well as the genetic background of such diseases. (biomedcentral.com)
  • Mitochondria most readily produce ATP by the oxidation of NADH and FADH 2 yielded from the breakdown of sugars such as glucose. (biomedcentral.com)
  • Electron cryoEM structures of mitochondrial, chloroplast, and bacterial ATP synthases have revealed the architecture of the FO region, helping to explain the mechanisms of proton translocation, dimerization of the enzyme in mitochondria, and cristae formation. (tcdb.org)
  • compared the structural and functional properties of F-ATP synthases in plant mitochondria with those of yeast and mammals. (tcdb.org)
  • All eukaryotic F-type ATPases pump 3-4 H + out of mitochondria, or into thylakoids of chloroplasts, per ATP hydrolyzed. (tcdb.org)
  • ABSTRACT: A computational model for the mitochondrial respiratory chain that appropriately balances mass, charge, and free energy transduction is introduced and analyzed based on a previously published set of data measured on isolated cardiac mitochondria. (physiomeproject.org)
  • ATP is ultimately formed from the generation of hydrogenated nicotinamide adenine dinucleotide (NADH) and dihydrogenated flavin adenine dinucleotide (FADH 2 ) in the citric acid cycle within mitochondria. (veteriankey.com)
  • Cell-membrane permeable substrates (glucose, pyruvate) may be applied to evaluate their effect on cell physiology, and cell-membrane impermeable substrates (succinate, ADP) are titrated as a cell viability test to assess the fraction of dead cells (dce) with permeable cell membranes and functional mitochondria. (mitoglobal.org)
  • Plant uncoupling mitochondrial protein activity in mitochondria isolated from tomatoes at different stages of ripening. (shengsci.com)
  • Green tomato mitochondria produced a membrane potential (deltapsi) high enough to phosphorylate ADP, whereas in red tomato mitochondria, BSA and A. (shengsci.com)
  • A class of nucleotide translocases found abundantly in mitochondria that function as integral components of the inner mitochondrial membrane. (nih.gov)
  • They facilitate the exchange of ADP and ATP between the cytosol and the mitochondria, thereby linking the subcellular compartments of ATP production to those of ATP utilization. (nih.gov)
  • Fatty acids, which are often found in chains as CoA-esters, are first transported across the outer mitochondrial membrane by palmitoyl transferase I, resulting in translocation to the intermembrane space and transformation into an acyl-carnitine form. (biomedcentral.com)
  • The generation of ATP is a universal process in all cells that occurs through the enzymatic machinery of the mitochondrial electron transport chain (ETC). In short, the ETC utilizes free hydrogen to create an electrochemical gradient between the inner and outer mitochondrial membrane that generates a covalent bond between ADP and inorganic phosphate to make ATP as the storage form of energy. (veteriankey.com)
  • The mitochondrial adenine nucleotide translocase (ANT also called the ADP/ATP carrier AAC) facilitates export of ATP outward over the internal membrane in to the cytoplasm in trade for import of ADP back to the matrix.1 That is driven with the electrochemical gradient over the internal membrane. (hiv-proteases.com)
  • The basic components included in the model are the reactions at complexes I, III, and IV of the electron transport system, ATP synthesis at F1F0 ATPase, substrate transporters including adenine nucleotide translocase and the phosphate\u2013hydrogen co-transporter, and cation fluxes across the inner membrane including fluxes through the K+/H+ antiporter and passive H+ and K+ permeation. (physiomeproject.org)
  • Mitochondrial nucleotide transporters, defined by structural similarlities, include the adenine nucleotide translocator family (SLC25A4, SLC25A5, SLC25A6 and SLC25A31), which under conditions of aerobic metabolism, allow coupling between mitochondrial oxidative phosphorylation and cytosolic energy consumption by exchanging cytosolic ADP for mitochondrial ATP . (guidetopharmacology.org)
  • Amyloid β, α-synuclein and the c subunit of the ATP synthase: Can these peptides reveal an amyloidogenic pathway of the permeability transition pore? (nyu.edu)
  • Conformational changes within the subunit b-dimer of the E. coli ATP synthase occur upon binding to the F(1) sector. (shengsci.com)
  • ATP synthase membrane subunit c loc. (gsea-msigdb.org)
  • ATP synthase membrane subunit f [So. (gsea-msigdb.org)
  • Further members of the mitochondrial nucleotide transporter subfamily convey diverse substrates including CoA, although not all members have had substrates identified. (guidetopharmacology.org)
  • The Mitochondrial Carrier (MC) Superfamily has been expanded to include both the original Mitochondrial Carrier (MC) family (TC# 2.A.29) and the Mitochondrial Inner/Outer Membrane Fusion (MMF) family (TC# 1.N.6). (wikipedia.org)
  • Ferdinando Palmieri mainly investigates Biochemistry, Mitochondrion, Mitochondrial carrier, Transport protein and Inner mitochondrial membrane. (research.com)
  • His Biochemistry research focuses on Gene isoform, Yeast, Mitochondrial matrix, Gene and Membrane transport protein. (research.com)
  • His Mitochondrial carrier research integrates issues from Mitochondrial membrane transport protein, ATP-ADP translocase, Saccharomyces cerevisiae and Citrate synthase. (research.com)
  • His studies in Transport protein integrate themes in fields like Glycerol-3-phosphate dehydrogenase, NAD+ kinase, Membrane transport, Genome and Membrane protein. (research.com)
  • His Inner mitochondrial membrane research incorporates elements of Phosphate and Citric acid cycle. (research.com)
  • His main research concerns Biochemistry, Mitochondrion, Mitochondrial carrier, Inner mitochondrial membrane and Molecular biology. (research.com)
  • Ferdinando Palmieri spends much of his time researching Biochemistry, Mitochondrial carrier, Mitochondrion, Inner mitochondrial membrane and Cell biology. (research.com)
  • His work is connected to Membrane transport, Saccharomyces cerevisiae, Carnitine, Mitochondrial transport and Mitochondrial matrix, as a part of Biochemistry. (research.com)
  • His work deals with themes such as Amino acid, Transport protein, Nucleotide, Gene and Stereochemistry, which intersect with Inner mitochondrial membrane. (research.com)
  • His primary areas of study are Biochemistry, Mitochondrial carrier, Mitochondrion, Inner mitochondrial membrane and Cell biology. (research.com)
  • His research in Mitochondrial carrier intersects with topics in Membrane transport, ATP-ADP translocase, NAD+ kinase and Solute carrier family. (research.com)
  • Simulation of huge patches of the model mitochondrial membrane filled with multiple copies from the translocase implies that CL connections persist in the current presence of protein-protein connections and suggests CL may mediate connections between translocases. (hiv-proteases.com)
  • The lipids of the Pralatrexate energy-minimized concatenated Personal computer membrane were then exchanged for any model mitochondrial membrane as explained above.34 The resultant system sizes were 65 nm × 65 nm × 14 nm containing 25 proteins and ~11000 lipids solvated by standard MARTINI water and neutralized by ~0.15 M NaCl. (hiv-proteases.com)
  • Four protein complexes in the inner membrane make up the electron transport chain (ETC, also known as the electron transport system), which converts the redox energy stored as NADH and FADH 2 into chemical energy in the form of ATP. (biomedcentral.com)
  • Subsequently, acyl-carnitines are translocated across the inner mitochondrial membrane by cartinine acyl translocase. (biomedcentral.com)
  • Oxidative phosphorylation is the fourth and final step that occurs in the inner mitochondrial membrane of eukaryotes or the plasma membrane of prokaryotes. (stemcelldaily.com)
  • B) Schematic diagram of the substrate transport process included in the model, including the ANT and PiHt on the inner membrane, and passive permeation of ATP, ADP, AMP, and phosphate across the outer membrane. (physiomeproject.org)
  • V-ATPase is made of two subcomplexes: a cytosolic V 1 that carries out ATP hydrolysis, and a membrane bound V o that is responsible for proton translocation. (upstate.edu)
  • The proton flow responsible for decreased pH values inside mitochondrial cristae and membrane invaginations of cyanobacteria has been c. (shengsci.com)
  • As protons flow through the channel domain of ATP synthase, a motor force is generated, which is used to rotate a large, rotating catalytic domain facing the matrix, which couples adenosine diphosphate (ADP) to an inorganic phosphate moiety (P i ) to yield ATP[ 4 ]. (biomedcentral.com)
  • To obtain reasonable fits to the available data, it is necessary to incorporate inorganic-phosphate-dependent activation of the dehydrogenase activity and the electron transport system. (physiomeproject.org)
  • Neuroinflammation and oxidative stress, mitochondrial dysfunction, dysregulation of the expression of histone deacetylases, and aggregation of pathogenic forms of proteins are among the most common and significant pathological features of neurodegenerative diseases. (actanaturae.ru)
  • These results provide contribution to the literature to clarify the effects of LETM1 loss in relation to oxidative stress and melatonin on mitochondrial function. (bioblast.at)
  • Aerobic respiration can produce up to 38 ATP molecules per glucose molecule, while anaerobic respiration can produce only 2 ATP molecules per glucose molecule. (stemcelldaily.com)
  • In this article, we will explore the details of each step of aerobic respiration, how ATP is generated in this process, and some examples of aerobic respiration in different organisms. (stemcelldaily.com)
  • The product of respiration is a molecule called adenosine triphosphate (ATP), which uses the energy stored in its phosphate bonds to power cellular processes. (stemcelldaily.com)
  • In this work, we have analyzed quantitatively the mitochondrial respiration in post-operational tissue samples taken from 55 patients with colorectal cancer (CRC). (mitophysiology.org)
  • The control of mitochondrial respiration was distributed across several mitochondrial processes: FCC for complex II (CII) was found to be high for CRC and healthy colon tissue, as well as in the case of Caco-2 cell culture (where the respiratory chain is controlled also by CIV) (Fig. 1). (mitophysiology.org)
  • Cell lines are frequently used as models to study mitochondrial (dys)function but little is known about their mitochondrial respiration and metabolic properties in comparison to the primary tissue of origin. (bioblast.at)
  • Cardoso LHD, Doerrier C, Gnaiger E (2021) Magnesium Green for fluorometric measurement of ATP production does not interfere with mitochondrial respiration. (oroboros.at)
  • A common problem with several fluorescent dyes is the inhibition of mitochondrial respiration. (oroboros.at)
  • In the present study, a coupling control protocol was applied in combination with MgG to measure ATP production simultaneously with respiration for calculation of P»/O 2 ratios. (oroboros.at)
  • TC# 2.A.29.12.1) Yeast mitochondrial proteins MRS3 (TC# 2.A.29.5.1) and MRS4 (TC# 2.A.29.5.2) Yeast mitochondrial FAD carrier protein (TC# 2.A.29.10.1) As well as many others. (wikipedia.org)
  • His research investigates the connection with Mitochondrial carrier and areas like Arabidopsis thaliana which intersect with concerns in Arabidopsis. (research.com)
  • Ferdinando Palmieri has included themes like Arabidopsis thaliana, Arabidopsis, ATP-ADP translocase and Solute carrier family in his Mitochondrial carrier study. (research.com)
  • Mechanistic implications of this "bubble" formation in the tether domain of ATP synthase b ( 2 ) are discussed. (shengsci.com)
  • Mitochondrial carriers are also classified in the Transporter Classification Database. (wikipedia.org)
  • The mitochondrial transporter family (SLC25): physiological and pathological implications. (research.com)
  • The mitochondrial transporter family SLC25: identification, properties and physiopathology. (research.com)
  • In its most traditional definition, the mitochondrion is the energy-generating organelle of the cell, responsible for the final steps of metabolizing organic substances to produce energy for the cell in the form of adenosine triphosphate (ATP). (biomedcentral.com)
  • These reactions break down glucose into carbon dioxide and water, and release energy in the form of adenosine triphosphate (ATP). (stemcelldaily.com)
  • The F 1 portion of the bovine mitochondrial F-type ATPase has been solved to 2.8 Å resolution. (tcdb.org)
  • A) Schematic diagram of the major components of the electron transport system, which transfers reducing potential from NADH to oxygen, and the F1F0 ATPase, which transduces energy from proton motive force to ATP, are illustrated. (physiomeproject.org)
  • Unlike reversible disassembly, which requires ATP hydrolysis on V 1 to break protein-protein interactions, Oxr1p mediated disassembly of V-ATPase is ATP independent and therefore a novel mode of activity regulation. (upstate.edu)
  • Pyruvate oxidation is the second step that occurs in the mitochondrial matrix of eukaryotes or the cytoplasm of prokaryotes. (stemcelldaily.com)
  • ATP generation occurs through beta-oxidation of fat and glucose oxidation through glycolysis, both of which lead to acetyl CoA production. (veteriankey.com)
  • Mitochondrial cytochrome c oxidase (COX) is the primary site of cellular oxygen consumption and is essential for aerobic energy generation in the form of ATP. (bioblast.at)
  • Mutations of mitochondrial carrier genes involved in mitochondrial functions other than oxidative phosphorylation are responsible for carnitine/acylcarnitine carrier deficiency, HHH syndrome, aspartate/glutamate isoform 2 deficiency, Amish microcephaly, and neonatal myoclonic epilepsy. (wikipedia.org)
  • His Mitochondrion study combines topics from a wide range of disciplines, such as mitochondrial fusion, Molecular biology, Carnitine and Cytosol. (research.com)
  • As electrons are shuttled through the complexes I, III and IV by electron carriers, protons (H + ) are moved from the mitochondrial matrix into the intermembrane space. (biomedcentral.com)
  • Reliable protocols were established to investigate the respiratory activity of different segments of the mitochondrial electron transfer-pathway in mechanically permeabilized tissue biopsies. (bioblast.at)
  • ER stress cross-talks to the mitochondrial or intrinsic death pathway via p53-upregulated modulator of apoptosis (Puma) and JNK-dependent upregulation of the Death protein (DP5) [27]. (cgp60474.com)
  • As the assembly was carried out in the absence of ATP, this suggests that formation of the holoenzyme from the individual subcomplexes is solely driven by protein-protein interactions at the interface. (upstate.edu)
  • Therefore, this study aimed to determine the impact of voluntary exercise on rats fed a high-fat diet on mitochondrial metabolism of carbohydrate- and lipid-based substrates in cardiac tissue. (oroboros.at)
  • These data show that HFD-induced mitochondrial dysfunction results in specific impairments in CI + CII maximal capacity to oxidize pyruvate and succinate as substrates in cardiac tissue, which is prevented by the introduction of exercise. (oroboros.at)
  • No matter where substrates enter the citric acid cycle, they are eventually turned into carbon dioxide, with oxygen as the reactant in the process for generating ATP (Shulman & Petersen, 2009). (veteriankey.com)
  • In mammalian cells, most of the redox potential used for generating ATP arrives at the mitochondrion in the form of the nicotinamide adenine dinucleotide (NADH) and flavin adenine dinucleotide (FADH 2 ), reduced coenzymes generated by the acceptance of electrons derived from the breakdown of organic substances in the tricarboxylic acid (TCA) cycle. (biomedcentral.com)
  • It involves breaking down a glucose molecule into two molecules of pyruvate, two molecules of NADH, and two molecules of ATP. (stemcelldaily.com)
  • It involves oxidizing acetyl-CoA into carbon dioxide and water, and generating ATP, NADH, and FADH2. (stemcelldaily.com)
  • This breakdown of glucose results in one 6-carbon molecule, generating two 3-carbon precursors, which eventually generate 2 NADH and 4 ATP and 2 pyruvate molecules ( Figure 4.2 ). (veteriankey.com)
  • Experiments using purified enzymes from cows and yeast revealed that the number of protons required by ATP synthase to make a single ATP molecule is almost twice as high for lower eukaryotes as it is for animals[ 5 ]. (biomedcentral.com)
  • The preferred route for protons in the intermembrane space to re-enter the matrix, in an attempt to flow down the gradient and re-establish equilibrium, is to enter through the ATP synthase enzyme complex. (biomedcentral.com)
  • How do Fatty Acyl-CoA's pass the mitochondrial intermembrane space? (flashcardmachine.com)
  • The Pralatrexate current presence of CL is vital for maximal stability of the translocase (i.e. ?1per phosphate group) based on Pralatrexate experimental estimations.36 The acyl tails of PC and PE lipids were modeled as 1-palmitoyl-2-oleoyl-tool to concatenate a system containing a single ANT1 molecule inlayed inside a PC bilayer onto a 5 × 5 grid (Figure S1). (hiv-proteases.com)
  • The rotor (which consists of the c, ε and γ subunits) is believed to rotate relative to the stator in response to either ATP hydrolysis by F 1 or proton transport through F 0 . (tcdb.org)
  • ATP generation and hydrolysis becomes a central theme to create appropriate muscle contraction. (veteriankey.com)
  • Altered mitochondrial metabolism plays a pivotal role in the development and progression of various diseases, including cancer. (bioblast.at)
  • In conclusion, combining the flexibility of cell culture models and tissue samples for respirometric analysis are powerful tools for investigation of mitochondrial function and tissue specific metabolism. (bioblast.at)
  • F 1 F 0 -ATP synthase is a large enzyme complex made up of over 22 subunits. (biomedcentral.com)
  • Thus, the production of ATP by the ATP synthase enzyme has significantly increased in efficiency during the evolution of multicellular organisms from early eukaryotic ancestors. (biomedcentral.com)
  • This creates a proton gradient that drives the synthesis of ATP by an enzyme called ATP synthase. (stemcelldaily.com)
  • A model of the structure of the enzyme near the gamma phosphate moiety is proposed. (shengsci.com)
  • Of the multiple cardiac aetiologies, cardiac mitochondrial dysfunction is a critical factor in the progression of heart disease. (oroboros.at)
  • The co-chaperone Hep1 is required to prevent the aggregation of mitochondrial Hsp70 proteins. (cipsm.de)
  • This electrochemical gradient is responsible for providing the driving force needed to produce ATP[ 3 ]. (biomedcentral.com)
  • H + transport and ATP synthesis may therefore be coupled mechanically. (tcdb.org)
  • V-type ATPases may pump 2-3 H + per ATP hydrolyzed, and these enzymes cannot catalyze pmf-driven ATP synthesis. (tcdb.org)
  • ATP is produced rapidly and does not require oxygen, which is the rate-limiting step in the citric acid cycle, making glycolysis a preferred system to generate ATP quickly and anaerobically. (veteriankey.com)
  • Chinopoulos C, Kiss G, Kawamata H, Starkov AA (2014) Measurement of ADP-ATP exchange in relation to mitochondrial transmembrane potential and oxygen consumption. (oroboros.at)
  • We quantified the control exerted by different components of the respiratory chain and the ATP synthasome complex in human CRC clinical material as compared with normal tissue and compatible cell culture Caco-2. (mitophysiology.org)
  • This review attempts to provide a summary of the background knowledge and recent developments in mitochondrial processes relating to mitochondrial-associated metabolic diseases arising from defects or deficiencies in mitochondrial function, as well as insights into current and future avenues for investigation. (biomedcentral.com)
  • Gnaiger E (2020) Mitochondrial pathways and respiratory control. (oroboros.at)
  • Gnaiger E et al ― MitoEAGLE Task Group (2020) Mitochondrial physiology. (oroboros.at)
  • Diseases caused by defects of mitochondrial carriers are reviewed by Palmieri et al. (wikipedia.org)
  • Fink BD, Bai F, Yu L, Sivitz WI (2017) Regulation of ATP production: dependence on calcium concentration and respiratory state. (oroboros.at)
  • The project Mitochondrial Physiology Map (MiPMap) is initiated to provide an overview of mitochondrial properties in cell types, tissues and species. (mitophysiology.org)
  • As part of Bioblast, MiPMap may be considered as an information synthase for Comparative Mitochondrial Physiology . (mitophysiology.org)
  • A comparative database of mitochondrial physiology may provide the key for understanding the functional implications of mitochondrial diversity from mouse to man, and evaluation of altered mitochondrial respiratory control patterns in health and disease ( Gnaiger 2009 ). (mitophysiology.org)
  • New perspectives of mitochondrial physiology. (mitophysiology.org)
  • One molecule of glucose is oxidized to 6 molecules of carbon dioxide, 6 molecules of water, and 32 molecules of ATP. (stemcelldaily.com)
  • It is slower compared to the anaerobic type but has a higher yield of ATP molecules. (stemcelldaily.com)
  • Human COX is a copper-heme A hetero-multimeric complex formed by 3 catalytic core subunits encoded in the mitochondrial DNA and 11 subunits encoded in the nuclear genome. (bioblast.at)
  • ATP is a molecule that stores and transfers energy for various cellular functions. (stemcelldaily.com)