Mitochondria mitochondrial outer membrane

FIGURE 21-10 Shuttle for transfer of acetyl groups from mitochondria to the cytosol. The mitochondrial outer membrane is freely permeable to all these compounds. Pyruvate derived from amino acid catabolism in the mitochondrial matrix, or from glucose by glycolysis in the cytosol, is converted to acetyl-CoA in the matrix. Acetyl groups pass out of the mitochondrion as citrate in the cytosol they are de-... 

Mitochondria release not only cytochrome c but also many pro-apoptotic factors (Table 17.1). They are normally localized in the intermembrane space of mitochondria. However, except for cytochrome c and the apoptosis inducing factor (AIF), their functions in the mitochondria have not been determined or they may have no function under normal conditions. Because they are larger than 5kD, they remain inside the mitochondrion. Once the mitochondrial outer membrane is permeabi-... 

Siskind LJ, Kolesnick RN, Colombini M. Ceramide forms channels in mitochondrial outer membranes at physiologically relevant concentrations. Mitochondrion 2006 6 118-125. 

The mitochondrial outer membrane is a physical barrier separating the metabolic processes of the cytoplasm from those of the mitochondrion. Integrity of the outer membrane of mitochondria can be determined using a cytochrome c accessibility assay (Douce et al. 1973). Since holocytochrome c cannot diffuse across intact outer membranes, the ratio of the initial rate of succinate icytochrome c oxidore-ductase activity by the mitochondria to that of the same mitochondria after deliberate lysis of their outer membranes is proportional to the extent of damage to the outer membranes incurred during mitochondrial isolation. 

Mitochondria are distinct organelles with two membranes. The outer membrane limits the organelle and the inner membrane is thrown into folds or shelves that project inward and are called cristae mitochondriales. The uptake of most mitochondrion-selective dyes is dependent on the mitochondrial membrane potential. Conventional fluorescent stains for mitochondria, such as rhodamine and tetramethylrosamine, are readily sequestered by functioning mitochondria. They are, however, subsequently washed out of the cells once the mitochondrion s membrane potential is lost. This characteristic limits their use in experiments in which cells must be treated with aldehyde-based fixatives or other agents that affect the energetic state of the mitochondria. To overcome this limitation, the research... 

What do I mean by a proton concentration gradient Simply, there is a higher concentration of protons in the space between the inner and outer membranes of the mitochondrion than in the mitochondrial interior. The gradient is formed from the energy released in the transfer of electrons down the electron transport chain. Put another way, the released energy is employed to pump protons across the inner mitochondrial membrane into the intermembrane space. 

This reaction is catalyzed by carnitine acyltransferase I on the outer membrane (fig. 18.21). A protein carrier in the inner mitochondrial membrane transfers the acyl-carnitine derivatives across the membrane. Once inside the mitochondria, the reaction is reversed by carnitine acyltransferase II to yield a fatty acyl-CoA (see fig. 18.21). Thus, at least two distinct pools of acyl-CoA occur in the cell, one in the cytosol and the other in the mitochondrion. 

These organelles are the sites of energy production of aerobic cells and contain the enzymes of the tricarboxylic acid cycle, the respiratory chain, and the fatty acid oxidation system. The mitochondrion is bounded by a pair of specialized membranes that define the separate mitochondrial compartments, the internal matrix space and an intermembrane space. Molecules of 10,000 daltons or less can penetrate the outer membrane, but most of these molecules cannot pass the selectively permeable inner membrane. By a series of infoldings, the internal membrane forms cristae in the matrix space. The components of the respiratory chain and the enzyme complex that makes ATP are embedded in the inner membrane as well as a number of transport proteins that make it selectively permeable to small molecules that are metabolized by the enzymes in the matrix space. Matrix enzymes include those of the tricarboxylic acid cycle, the fatty acid oxidation system, and others. 

The mitochondrion is bounded by two pho-spholipid membranes. The outer membrane is freely permeable to molecules, including water, with a molecular weight of up to about 5000. The inner membrane is rich in membrane-bound proteins and consists, in terms of membrane area, of 50% phospholipid and 50% protein (Lenaz, 1988). Pyruvate dehydn>genase, a mitochondrial enzyme, is water soluble. The proteins of the respiratory chain, as well as ATP synthase, are all bound to the inner mitochondrial membrane. The enzymes of the Krebs cycle are water soluble, with the exception of succinate dehydrogenase. This enzyme is bound to the mitochondrial membrane, where it directly funnels electrons, via HAD, to the respiratory chain. 

Actively respiring fungal cells possess a distinct mitochondrion, which has been described as the power-house of the cell (Fig. 4.2). The enzymes of the tricarboxylic acid cycle (Kreb s cycle) are located in the matrix of the mitochondrion, while electron transport and oxidative phosphorylation occur in the mitochondrial inner membrane. The outer membrane contains enzymes involved in lipid biosynthesis. The mitochondrion is a semiindependent organelle as it possesses its own DNA and is capable of producing its own proteins on its own ribosomes, which are referred to as mitoribosomes. 

The Import receptors subsequently transfer the precursor proteins to an Import channel in the outer membrane. This channel, composed mainly of the Tom40 protein, is known as the general import pore because all known mitochondrial precursor proteins gain access to the interior compartments of the mitochondrion through this channel. When... 

The mitochondria are aerobic cell organelles that are responsible for most of the ATP production in eukaryotic cells. They are enclosed by a double membrane. The outer membrane permits low-molecular-weight molecules to pass through. The inner mitochondrial membrane, by contrast, is almost completely impermeable to most molecules. The inner mitochondrial membrane is the site where oxidative phosphorylation occurs. The enzymes of the citric acid cycle, of amino acid catabolism, and of fatty acid oxidation are located in the matrix space of the mitochondrion. 

Mitochondria contain most of the enzymes for the pathways of fuel oxidation and oxidative phosphorylation and thus generate most of the ATP required by mammalian cells. Each mitochondrion is surrounded by two membranes, an outer membrane and an inner membrane, separating the mitochondrial matrix from the cytosol (Fig. 10.18). The inner membrane forms invaginations known as cristae containing the electron transport chain and ATP synthase. Most of the enzymes for the TCA cycle and other pathways for oxidation are located in the mitochondrial matrix, the compartment enclosed by the inner mitochondrial membrane. (The TCA cycle and electron transport chain are described in more detail in Chapters 20 and 21.)... 

A second very important eukaryotic organelle is the mitochondrion, which, like the nucleus, has a double membrane (Figure 1.13). The outer membrane has a fairly smooth surface, but the inner membrane exhibits many folds called cristae. The space within the inner membrane is called the matrix. Oxidation processes that occur in mitochondria yield energy for the cell. Most of the enzymes responsible for these important reactions are associated with the inner mitochondrial membrane. Other enzymes needed for oxidation reactions, as well as DNA that differs from that found in the nucleus, are found in the internal mitochondrial matrix. Mitochondria also contain ribosomes similar to those found in bacteria. Mitochondria are approximately the size of many bacteria, typically about 1 pm in diameter and 2 to 8 pm in length. In theory, they may have arisen from the absorption of aerobic bacteria by larger host cells. 

A quick review of some aspects of mitochondrial structure is in order here because we shall want to describe the exact location of each of the components of the citric acid cycle and the electron transport chain. Recall from Chapter 1 that a mitochondrion has an inner and an outer membrane (Figure 19.2). The region enclosed by the inner membrane is called the mitochondrial matrix, and an intermembrane space exists between the inner and outer membranes. The inner membrane is a tight barrier between the matrix and the cytosol, and very few compounds can cross this barrier without a specific transport protein (Section 8.4). The reactions of the citric acid cycle take place in the matrix, except for the one in which the intermediate electron acceptor is FAD. The enzyme that catalyzes the FAD-linked reaction is an integral part of the inner mitochondrial membrane and is linked direcdy to the electron transport chain (Chapter 20). 

The protons are released to one side of an otherwise generally proton-impermeable inner mitochondrial membrane to collect the protons in the space between the inner and outer membranes of the mitochondrion. The resulting proton concentration gradient then drives formation of ATP by the quintessential protein-based machine, ATP synthase, as the protons flow back through the inner mitochondrial membrane by means of another special path effecting proton permeability. Thus there are two fundamental questions. The first is, how does electron flow within the membrane achieve unidirectional proton flow across the membrane The second is, how does the return flow of protons result in the formation of ATP, the energy coin of biology ... 

Figure 8.5. The mitochondrion, the energy factory of the cell. (Top) Electron micrographs (A),(B),and (C) of the inner mitochondrial membrane studded with stalks and headpieces that are the extramembrane components of ATP synthase with the remainder contained within the inner membrane. (Bottom) Drawing of a mitochondrion with an outer membrane and a folded inner mitochondrial membrane enclos-...

Basically, all mitochondria consist of two membranes which surround and enclose an inner compartment containing the mitochondrial matrix. The outer membrane is usually smooth in surface contour, whereas the inner membrane is folded into a series of lamellae, the cristae. The regions of the inner membrane between the cristae are designated collectively as the inner boundary membrane. The space between the outer and inner membranes, known as the inter membrane space, is continuous with the space bounded by the membranes of the cristae. Figure 1 shows a schematic drawing of a mitochondrion. 

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