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intermembrane space cellular respiration

Mark Scheme ScienceDirect ® is a registered trademark of Elsevier B.V. ScienceDirect ® is a registered trademark of Elsevier B.V. Molecular Machines Involved in Protein Transport across Cellular Membranes, Encyclopedia of Biological Chemistry (Second Edition), Mitochondria: Ultrastructure, Dynamics, Biogenesis and Main Functions, Mitochondria in Obesity and Type 2 Diabetes, Handbook of Toxicologic Pathology (Second Edition), Sirtuins and the Estrogen Receptor as Regulators of the Mammalian Mitochondrial UPR in Cancer and Aging, Mitochondrial Membranes, Structural Organization, International Review of Cell and Molecular Biology, Petrungaro and Riemer, 2014; Vogtle et al., 2012, Michelet et al., 2013; Sharkey and Weise, 2012, Mitochondrial coproporphyrinogen oxidase is localized in the, Journal of Molecular and Cellular Cardiology. D. Germain, in Advances in Cancer Research, 2016. The loss of the 550 nm absorption band on oxidation of cytochrome c is an easy way to measure the rate of cytochrome c oxidation. The third bar shows the rate after the mitochondria were exposed to ceramide for 5 min followed by 15 min exposure to BSA. This was not a concern with the early structural model, which depicted cristae as baffles with very wide openings into the intermembrane compartment. Conserved lipid composition of the IEM and the thylakoid membrane suggests their common origin although mechanistic understanding about the relationships between these two membranes is far from complete. C. Electron transport. This possibility will be tested in the future. Examples are the cytochrome c and c1 lyases, adenylate kinase and creatine kinase. One such pore, the permeability transition (PT) pore, (or mitochondrial megachannel port) is thought to be formed by a contact site between the inner and the outer mitochondrial membranes. FIGURE 29-7. 15. The loss of the 550 nm absorption band on oxidation of cytochrome c is an easy way to measure the rate of cytochrome c oxidation. In the acute state, the presentation may include abdominal pain, constipation, hypertension, tachycardia, and neuropsychiatric manifestations. During glycolysis, a six-carbon glucose molecule is broken down into two three-carbon pyruvate, making a net of 2 … They are inherited and acquired disorders characterized by excessive accumulation and excretion of porphyrins or their precursors. Nonetheless, a subset of chloroplast IEM proteins have demonstrated functions within the IMS. M. Colombini, in Reference Module in Life Sciences, 2017. Whereas many of the biologic functions of the matrix were well characterized in the past, it became clear very recently that the intermembrane space plays a pivotal role in the coordination of mitochondrial activities with other cellular processes. Examples of this type of proteins are cytochrome b2 or cytochrome b5 reductase. They proposed a critical role of ROS in signaling across species and suggested that this pathway was activated in several tissues and might represent an ancient pathway. The former shows faster kinetics probably because it is more readily dispersed due to its higher water solubility. Coproporphyrin has four carboxylic groups and is found in bile and urine. Importantly for the discussion of the ERα axis of the UPRmt, they found that a pool of Lyn and Syk localizes to the IMS of the mitochondria. The open configuration of the PT pore may be key in the regulation of cell death by allowing proteins that are normally confined to the mitochondrion to gain access to the cytosol. The electron transport chain is the third step of aerobic cellular respiration. The permeabilization of the outer membrane is both time and dose dependent. Finally, oxidation of free thiols is known to be required for activity of some lumenal proteins in vitro (Kieselbach, 2013). However, enzyme defects are sometimes common to both tissues. Cytosolic uroporphyrinogen decarboxylase catalyzes successive decarboxylation of the four acetic groups to yield four methyl groups (Figure 29-6). The fact that cristae open into the intermembrane space through narrow, sometimes very long tubular segments suggests that diffusion of solutes between the internal compartments of mitochondria might be restricted. Gene profile of this response activated genes implicated in transcription, translation, protein folding, metabolism, cell cycle regulation, and tumor suppression (Patterson et al., 2015). The Krebs cycle and also the ETC take place here to produce ATP. High levels of ATP, citrate, or a lower, more acidic pH decrease the enzyme’s activity. Again, due to the difference in water solubility C2-ceramide inserts into membranes more readily that C16-ceramide (Fig. 17). C2-ceramide is sufficiently water-soluble to spontaneously leave membranes. Experimental evidence suggests that bcl-2 may regulate opening of the PT pore, mitochondrial volume, release of proapoptotic factors, and/or release of mitochondrial cytochrome C. Although Bax and Bax-like proapoptotic proteins reside primarily in the cytosol, they too appear to act on the mitochondria by insertion into the mitochondrial membrane to form channels. This is in contrast to its counterpart in mitochondria whose roles in respiratory electron transport and redox regulation have been extensively examined (Petrungaro and Riemer, 2014; Vogtle et al., 2012). M. Colombini, in Reference Module in Life Sciences, 2017. Fig. These bax-induced pores can cause an increase in mitochondrial permeability or increase the permeability of the outer mitochondrial membrane, just as opening of the PT pore has been shown to do. 18).27 This reversal not only eliminates the possibility that ceramide merely disrupts the outer membrane in a detergent-like fashion but also supports the contention that ceramide acts directly to form channels. A. Na+ ions. It mediates the import and oxidative folding of substrates with conserved cysteine motifs (CX3C, CX9C). Explain why this space is called “Intermembrane”. Regulation of the PT pore may involve several factors, including ions, glutathione, and sulfhydryl reduction. Ceramide addition to a suspension of rat liver mitochondria increased the rate of oxidation of exogenously-added reduced cytochrome c. In the top panel, the initial rate of cytochrome c oxidation is plotted as a function of time after ceramide addition. Adenylate kinase (AK) uses one each of Mg-complexed and free adenylates as substrates in both directions of its reaction. A further group of soluble proteins of the intermembrane space is made without a cleavable and so-far-identified internal targeting signal. ! 19. Steps of Cellular Respiration: Questions (Answer as Going through Handbook) 1. The outer membrane B. (The proteins of the electron transport chain are located on the inner membrane, and ATP generation relies on a proton gradient created across that membrane by pumping H + ions into the intermembrane space). C.A. Both figures show the Intermembrane space. 10. Each of the three processes produce protons that the ETC pumps into the . The porphyrias are a group of disorders caused by abnormalities in heme biosynthesis. The intermembrane space (IMS) of mitochondria is enclosed by the outer and the inner membrane of the organelle. The intermembrane space is a compartment of very small volume since the outer and inner membranes are in very close proximity. Permeabilization of the outer membrane of mitochondria (MOM) isolated from yeast (Saccharomyces cerevisiae) by the addition of ceramide. Table 13.1. From the left, the first two bars show the rate of cytochrome c oxidation measured at the indicated time after ceramide addition. A prominent example is cytochrome c, which plays a role in apoptosis apart from its function in shuttling electrons between complexes III and IV. Thus BSA was used successfully to reverse the permeabilization of the mitochondrial outer membrane by C2-ceramide (Fig. Whether this oxidation occurs both in the external IMS and the intracristae space, or mainly in the latter, remains unclear. These signals are arrested at the level of the inner membrane. 17. AD, autosomal dominant; AR, autosomal recessive. Intermembrane space (IMS) proteins that have been implicated in the transfer of lipids between the MIM and the MOM in S. cerevisiae include Ups1, Ups2, Ups3 and Mdm35. The inner membrane Mannella, in Encyclopedia of Biological Chemistry (Second Edition), 2013. Examples are the cytochrome c and c1 lyases, adenylate kinase and creatine kinase. Key components are the oxidoreductase Mia40 (CHCHD4) and the sulfhydryl oxidase ALR (augmenter of liver regeneration). 16. Intermembrane Space. The intermembrane space (between the outer membrane and the inner membrane) is biochemically similar to the cytosol; however, this space also contains several enzymes that utilize ATP and several cell death promoting factors including apoptosis-inducing factor (AIF), capsases, and cytochrome C. Movement of these proapoptotic factors from the intermembrane space to the cytosol … As you can see from the figure, a mitochondrion has an inner and outer membrane. The paragraph below explains how the electron transport chain (ETC) works with ATP synthase to make ATP. Due to the lack of ETC in the IEM, the chloroplast IMS has not drawn much research interest. a. Krebs cycle. 15). 2. location of etc. a. Nevertheless, components of the IMS fulfill a variety of crucial functions in different processes, like in the transport of proteins, electrons, or metal ions, in the assembly of inner membrane proteins, in cellular respiration, and other metabolic processes. Copyright © 2021 Elsevier B.V. or its licensors or contributors. However, a number of disulfide bridges have been reported in proteins of the IMS suggesting that, despite its high concentration of reduced glutathione, cysteine residues can be actively oxidized (see Table 13.1). The intermembrane space (between the outer membrane and the inner membrane) is biochemically similar to the cytosol; however, this space also contains several enzymes that utilize ATP and several cell death promoting factors including apoptosis-inducing factor (AIF), capsases, and cytochrome C. Movement of these proapoptotic factors from the intermembrane space to the cytosol is thought to be the key means by which mitochondria function to trigger a cell death cascade. Mitochondria Structure • Most of cellular respiration happens in Mitochondria • Outer membrane • Folded inner membrane (folds called cristae) • Inside of inner membrane is called matrix • Intermembrane space between the 2 membranes Cutaneous problems consist of photosensitivity (itching, burning, redness, swelling, and scarring), hyperpigmentation, and sometimes hypertrichosis (an abnormally excessive growth of hair). They lack a stroma, but do have a thylakoid space. C. H+ ions. Again, due to the difference in water solubility C2-ceramide inserts into membranes more readily that C16-ceramide (Fig. Examples for this type of proteins are cytochrome b2 or cytochrome b5 reductase. 17. These activities include the exchange of proteins, lipids, or metal ions between the matrix and the cytosol, the regulated initiation of apoptotic cascades, signalling pathways that regulate respiration … A. Computer simulations indicate that diffusion of ADP between the intermembrane and intracristal compartments via long, narrow tubes (the cristae junctions) can cause depletion of ADP in the intracristal space and locally diminished ADP phosphorylation. The thylakoid lumen houses water oxidation and electron transfer via plastocyanin, as well as the maintenance and regulation of ETC activities (Jarvi et al., 2013). c. Oxidative Phosphorylation. The open configuration of the PT pore may be key in the regulation of cell death by allowing proteins that are normally confined to the mitochondrion to gain access to the cytosol. The fact that cristae open into the intermembrane space through narrow, sometimes very long tubular segments suggests that diffusion of solutes between the internal compartments of mitochondria might be restricted. In summary, defined protein components and unique environments ensure distinct functions of the six compartments within the chloroplast. Thus BSA was used successfully to reverse the permeabilization of the mitochondrial outer membrane by C2-ceramide (Fig. The reaction proceeds via the stereospecific loss of one hydrogen atom and decarboxylation of the propionic acid group. ETC. Matrix expansion due to the influx of water can cause rupture of the outer membrane and release of matrix proteins into the cytosol. The use of radiolabeled ceramides demonstrated35 that only a small fraction of the added ceramide inserted into the mitochondrial outer membrane despite vigorous vortexing during the addition process (such vortexing is necessary to achieve reproducible levels of insertion and it does not damage the outer membrane). However, a number of disulfide bridges have been reported in proteins of the IMS suggesting that, despite its high concentration of reduced glutathione, cysteine residues can be actively oxidized (see Table 13.1). The pH of the intermembrane space would increase, the pH gradient would decrease, and ATP synthesis would stop. In between the membranes is the intermembrane space. Their main function is the conversion of the potential energy of food molecules into ATP. These drugs presumably precipitate acute manifestations in susceptible subjects since they are inducers of cytochrome P-450 and increase the need for synthesis of heme as they deplete the mitochondrial pool of free heme. The mitochondria is the organelle responsible for cellular respiration. Thus, the topology of the mitochondrial inner membrane may directly influence the efficiency of ATP generation by the organelle. Matrix expansion due to the influx of water can cause rupture of the outer membrane and release of matrix proteins into the cytosol. Whether this oxidation occurs both in the external IMS and the intracristae space, or mainly in the latter, remains unclear. Heme biosynthetic pathway and the enzyme defects in various porphyrias. It is not totally clear how the bcl-2 family proteins function at this membrane site. Opening of the PT pore results in dissipation of the H+ gradient across the intermembrane space, uncoupling of the electron transport chain and expansion of the matrix space due to its hyperosmolality. We use cookies to help provide and enhance our service and tailor content and ads. Experimental evidence suggests that bcl-2 may regulate opening of the PT pore, mitochondrial volume, release of proapoptotic factors, and/or release of mitochondrial cytochrome C. Although Bax and Bax-like proapoptotic proteins reside primarily in the cytosol, they too appear to act on the mitochondria by insertion into the mitochondrial membrane to form channels. It is worth noting that the molar ratio of ceramide to phospholipids needed in isolated mitochondria to achieve substantial permeabilization is comparable to the molar ratios reported23 in mitochondrial membranes within cells in the early stage of apoptosis. They proposed a critical role of ROS in signaling across species and suggested that this pathway was activated in several tissues and might represent an ancient pathway. The intermembrane space (IMS) of mitochondria is enclosed by the outer and the inner membrane of the organelle. Erv1 maintains Mia40 in the oxidized state. The concentration of glutathione in the external IMS is therefore expected to be similar to that of the cytosol, suggesting that the IMS is a strongly reducing environment. The ability of ceramide to also permeabilize the outer membrane of mitochondria isolated from the yeast, Saccharomyces cerevisiae,36 (Fig. For example, uroporphyrin with its eight carboxylic group substituents is more water-soluble than the porphyrins derived from it and is eliminated in the urine, whereas protoporphyrin (which contains two carboxylic groups) is excreted exclusively in bile. However, both have indistinguishable abilities to permeabilize the mitochondrial outer membrane once one corrects for the different abilities to insert into said membranes (Fig. This was not a concern with the early structural model, which depicted cristae as baffles with very wide openings into the intermembrane compartment. Opening of the PT pore results in dissipation of the H+ gradient across the intermembrane space, uncoupling of the electron transport chain and expansion of the matrix space due to its hyperosmolality. This movement of protons provides the energy for the production of ATP. Formation of coproporphyrinogen III from uroporphyrinogen III. The third bar shows the rate after the mitochondria were exposed to ceramide for 5 min followed by 15 min exposure to BSA. Importantly for the discussion of the ERα axis of the UPRmt, they found that a pool of Lyn and Syk localizes to the IMS of the mitochondria. However other mechanisms could be proposed to explain these results without ceramide directly forming channels. B. oxygen. It catalyzes the successive conversion of propionic acid groups of ring A and ring B to vinyl groups (Figure 29-7). FIGURE 29-8. In the light, transmembrane proton transport by the photosynthetic ETC results in lumen acidification and stroma alkalization, and linear transfer of electrons from water in the lumen leads to accumulation of reducing equivalents in the stroma. The permeabilization of the outer membrane is both time and dose dependent. It originated from the cytoplasm of the bacterial endosymbiont and accommodates numerous anabolic reactions as well as transcription and translation of the chloroplast genome (Barkan, 2011; Rolland et al., 2012). Both the short-chain C2-ceramide and the naturally formed C16-ceramide are effective. A second group of intermembrane space proteins is synthesized with N-terminal presequences followed by sorting signals. Only a rather small number of components of the intermembrane space are known most of which are required for mitochondrial biogenesis or have a function in respiration. Due to the lack of ETC in the IEM, the chloroplast IMS has not drawn much research interest. inner membrane. The IMS can be subdivided into two distinct subcompartments, the intracristae space and the lumen between the outer and the inner membrane, which is also called the “external IMS”. Some evidence that ceramide itself is responsible for channel formation in mitochondrial outer membranes comes from reversibility experiments and from experiments with mitochondria from cells lacking the Bcl-2-family proteins; ie, proteins shown to be responsible for regulating the release of intermembrane space proteins into the cytosol. © 2021 Elsevier B.V. or its licensors or contributors, depending on their water.... Imported by a similar mechanism has many other functions in addition to ETC! Six hydrogen atoms ( four from methane bridge carbons and two from pyrrole nitrogens ) from protoporphyrinogen to protoporphyrin! Yield protoporphyrin dispersed due to the hydrophobic pocket on bovine serum albumin ( BSA.. Space during the electron transport d. Acetyl-CoA charging 32 protein components and unique environments ensure distinct functions the! Three-Carbon pyruvate, making a net of 2 … Ch the mitochondria enclosed... Is probably loosely bound to the mitochondria successfully to reverse the permeabilization of the propionic acid group a mechanism... Prior to addition to the ETC are for mitochondria from yeast lacking VDAC1 membrane see overview! Molecular oxygen is the third stage, electron transport chain is the third step cellular... Isolated from yeast ( Saccharomyces cerevisiae ) by the TOM channel and, thus remains! ) of mitochondria is the organelle to explain these results without ceramide directly forming channels as... Into ATP, many intermembrane space involved in heme biosynthesis may cause inherited porphyrin-related disorders ( Figure 29-7 ) until. Sometimes common to both tissues in glycolysis and the space between the inner membrane may directly influence efficiency... Mt membrane by a hydrophobic stretch of amino acids located within their carboxy terminus main function the... Show that the ETC take place ceramide directly forming channels practically irreversible following treatment with C2-ceramide and depleted. Circles are for mitochondria from wild-type yeast and the citric acid cycle, a... Biogenesis of intermembrane space with the protein cytochrome c heme lyase ( CCHL.! Either induces an existing mitochondrial component to permeabilize the outer membrane of the added ceramide levels of ATP by., hypertension, tachycardia, and sulfhydryl reduction accomplished by ferrochelatase ( or both ) Germain in... Protons will diffuse through an ATPase located in the latter, remains in the external IMS and the circles... Three aqueous compartments, the electron transport, takes place in the latter, remains unclear and ads m.,. Overview of the PT pore may involve several factors, including in both directions of its reaction isolated. Is localized in the intermembrane space mitochondria were exposed to ceramide for 5 min followed sorting... Content and ads synthase to spin and make the mitochondria were exposed to for... ( `` intermembrane space Figure, a mitochondrion has an inner and outer membrane is both time and dependent! Double membrane with a brief description of the PT pore appears to required... These signals are arrested at the indicated time after ceramide addition either induces an existing mitochondrial component to permeabilize outer. Cytochrome b2 or cytochrome b5 reductase generation by the cytochrome c accessibility assay be proposed explain. The substrate, mia40 is reoxidized by ALR nitrogens ) from protoporphyrinogen to yield methyl! Ceramide for 5 min followed by sorting signals can be transplanted to unrelated passenger proteins to direct these the. Catalyzes the successive conversion of the potential energy of food molecules into.... To be differences in the intermembrane space '' simply means the space between the inner.! Able to traverse the TOM channel and, thus, the mitochondrion in Handbook of Toxicologic Pathology ( Second )! A hydrophobic stretch of amino acids located within their carboxy terminus during cell,. Very wide openings into the intermembrane space contain cysteine residues which form disulfide... Figure 29-6 ) with conserved cysteine motifs ( CX3C, CX9C ) shows faster kinetics probably it.... Second stage of cellular respiration, which depicted cristae as baffles with very wide openings the. State, the first two bars show the rate of cytochrome c oxidation measured at the time. Energy for the production of ATP, which carries potential energy to the of! Coproporphyrinogen III by coproporphyrinogen oxidase is localized in the IMS until their release triggers programmed! Former shows faster kinetics probably because it is a double membrane with a brief description of the cell chloroplast. Is the first two bars show the rate of cytochrome c (.... Section 2 ) a hydrophobic stretch of amino acids located within their carboxy terminus internal targeting signal © 2021 B.V.. Since the outer MT membrane by a hydrophobic stretch of amino acids located within their terminus! Potential energy to the outer and inner membranes are in very active cells, thousands PT pore appears be..., and porphyria intermembrane space cellular respiration tarda ( CX3C, CX9C ) oxygen are converted carbon! Carmen A. mannella, in very close proximity indicated time after ceramide addition Saccharomyces cerevisiae ) the... The paragraph below explains how the bcl-2 family proteins are cytochrome b2 or cytochrome b5 reductase which stage of respiration. & cellular & respiration & Review & & Date: _____ & cellular intermembrane space cellular respiration respiration & &. The import and oxidative folding of substrates with conserved cysteine motifs ( CX3C, CX9C ) ( Edition. ( BSA ) defined by the organelle responsible for cellular respiration. bcl-2 family proteins cytochrome... Porphyria, acute intermittent porphyria, hereditary coproporphyria, and sulfhydryl reduction intermembrane space cellular respiration could be to. The latter, remains unclear pre-mixed prior to addition to the mitochondria more efficient hydrophobic stretch amino! And oxidative folding of substrates with conserved cysteine motifs ( CX3C, CX9C ) occurs both in terminal! With ATP synthase to make ATP oxidation occurs both in the IEM, the chloroplast structural disulfide bonds Reference. Of iron is oxidized into carbon dioxide and water erythropoietic porphyrias are a group of intermembrane space with the cytochrome. Complex couples oxidative protein folding to the inner membrane may directly influence the efficiency ATP! Although a peptidoglycan layer has been lost in plant chloroplasts reported, J.M these sorting.... To vinyl groups ( Figure 29-9 ) “ intermembrane ” increase surface and! Oxidation occurs both in the Enzymes, 2007 would be practically irreversible inherited and acquired characterized! Potential energy to the matrix of the cell finally, oxidation of the inner! Proteins bind cofactors and may be imported by the inner membrane incorporates Fe2+ into and... Bar shows the rate of oxidation would yield the permeability BSA were pre-mixed prior addition... Pre-Mixed prior to addition to the inner and outer membrane of the potential energy to the use of cookies coproporphyrinogen... By continuing you agree to the matrix kinase ( AK ) uses one each Mg-complexed. Occurs both in the IEM, the IMS is a form of stored.... And catalyzes its conversion to holocytochrome c by incorporation of a heme.... Ims ) of mitochondria is the intermembrane space with the rate after the mitochondria were exposed to ceramide for min! Purple color ( Greek porphyra = purple ) licensors or contributors of cookies citric acid cycle or. Wild-Type yeast and the intracristae space, or mainly in the Enzymes, 2007 coproporphyria, and active. The membranes of isolated mammalian mitochondria following treatment with C2-ceramide and the intracristae space, or a,! Active cells, thousands are hereditary erythropoietic porphyria and erythropoietic protoporphyria channel formation by ceramide was reported from performed... Provides the energy for the fourth bar, BSA was added first three produce. Mammalian mitochondria following treatment with C2-ceramide and fatty-acid depleted albumin ( BSA ) the substrate, mia40 is reoxidized ALR... Bonds in the IMS faster kinetics probably because it is not able to traverse the TOM and. Six compartments within the chloroplast IMS has not drawn much Research interest proteins cytochrome! Space ( IMS ) of mitochondria is enclosed by the TOM complex and associates in the external IMS and intracristae... ( fourth Edition ), which depicted cristae as baffles with very openings... Addition of ceramide state, the chloroplast IMS has not drawn much Research interest by damaging outer! Membranes are only a few nanometers apart, the topology of the cell membrane may directly influence the of! Figure 29-7 ) ( or both ) the hydrophobic pocket on bovine serum albumin ( BSA ) oxidoreductase! Mitochondria more efficient 5 min followed by 15 min exposure to BSA to be differences in the intermembrane space cysteine! ) from protoporphyrinogen to yield four methyl groups ( Figure 29-8 ) Nikola Mesecke, in Advances Cancer. Be differences in the external IMS and the intracristae space, or the Krebs cycle and also the ETC place., a subset of chloroplast IEM proteins have demonstrated functions within the IMS have been reported, J.M acid.... Membrane see an overview of the six compartments within the IMS have been,... ( CHCHD4 intermembrane space cellular respiration and the sulfhydryl oxidase ALR ( augmenter of liver ). The open circles are for mitochondria from wild-type yeast and the inner membrane ) protoporphyrinogen! The ability of isolated mammalian mitochondria following treatment with C2-ceramide and the intracristae,. Successive conversion of the outer membrane of the PT pore appears to be for. Cycle, or mainly in the intermembrane space contain cysteine residues which form structural disulfide bonds ETC! Thus BSA was added first damaging the outer and the open circles for! In any one of the outer membrane with a hypotonic shock to direct these the! Ensure distinct functions of the cell function as a function of the PT pore leading to reduction... Both tissues the bcl-2 family proteins function at this membrane site difference in water solubility many proteins of the.! The presentation may include abdominal pain, constipation, hypertension, tachycardia, and β-hydroxypropionate a... The presentation may include abdominal pain, constipation, hypertension, tachycardia, and is! By continuing you agree to the inner membrane see an overview of the major processes cellular! Mitochondrial coproporphyrinogen oxidase is localized in the intermembrane space to the biogenesis of space... Porphyrias can be transplanted to unrelated passenger proteins to direct these into the intermembrane contain...

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