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Mitocôndria Morfologia e constituintes da mitocôndria Reprodução Função •Produção de energia para a célula; •Respiração celular. Envolvimento da mitocôndria nos processos de obtenção de energia •Glicólise (Glucose2piruvatos); •Oxidação do piruvato em acetil CoA (2 piruvato 2acetil CoA) •Beta-oxidação (ácido graxo n acetil CoA); •Ciclo do ácido cítrico; •Fosforilação oxidativa. A organização geral de uma mitocôndria A organização geral de uma mitocôndria Figure 14-8. The general organization of a mitochondrion. In the liver, an estimated 67% of the total mitochondrial protein is located in the matrix, 21% is located in the inner membrane, 6% in the outer membrane, and 6% in the intermembrane space. As indicated below, each of these four regions contains a special set of proteins that mediate distinct functions. (Courtesy of Daniel S. Friend.) http://www.sumanasinc.com/webcontent/ animations/content/organelles.html http://www.sumanasinc.com/webcontent/animations/content/organelles.html Origem da mitocôndria Figure 1-35. The origin of mitochondria. An ancestral eucaryotic cell is thought to have engulfed the bacterial ancestor of mitochondria, initiating a symbiotic relationship. http://www.sumanasinc.com/webcontent/animations/content/organelles.html Bactéria aeróbia Célula eucariótica anaeróbia ancestral Genoma da mitocôndria de humanos Figure 14-58. The organization of the human mitochondrial genome. The genome contains 2 rRNA genes, 22 tRNA genes, and 13 protein-coding sequences. The DNAs of many other animal mitochondrial genomes have also been completely sequenced. Most of these animal mitochondrial DNAs encode precisely the same genes as humans, with the gene order being identical for animals that range from mammals to fish. Síntese das proteínas da mitocôndria Figure 14-64 The origins of mitochondrial RNAs and proteins. The proteins encoded in the nucleus and imported from the cytosol have a major role in creating the genetic system of the mitochondrion, in addition to contributing most of the organelle's other proteins. Not indicated in this diagram are the additional nucleus-encoded proteins that regulate the expression of individual mitochondrial genes at posttranscriptional levels. The mitochondrion itself contributes only mRNAs, rRNAs, and tRNAs to its genetic system. Origem das proteínas e RNAs da mitocôndria http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5688 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5568 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5055 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5582 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5688 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5568 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5688 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5163 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5215 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5496 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5767 http://www.ncbi.nlm.nih.gov/bookshelf/?book=mboc4&part=A4754&rendertype=def-item&id=A5902 Figure 14-54. Mitochondrial fission. These processes involve both outer and inner mitochondrial membranes. (A) During fusion and fission, both matrix and intermembrane space compartments are maintained. Different membrane fusion machines are thought to operate at the outer and inner membranes. Conceptually, the fission process resembles that of bacterial cell division (discussed in Chapter 18). The pathway shown has been postulated from static views such as that shown in (B). (B) An electron micrograph of a dividing mitochondrion in a liver cell. (B, courtesy of Daniel S. Friend.) Fissão ou divisão binária da mitocôndria http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=mboc4.chapter.3331 Gordura Proteína Piruvato Piruvato Glicerol + Ácido graxo Ácido graxo Proteína aminoácidos glucose CICLO DE KREBS FOSFORILAÇÃO OXIDATIVA Acetil CoA Carboidratos Carboidratos Citoplasma Mitocôndria Membrana Plasmática Envolvimento da mitocôndria na produção de energia G L I C Ó L I S E - O X I D A Ç À O ATP ATP ATP C6H12O6 (glucose) 6 CO2 + 6 H2O + Energia na forma de calor e luz Oxidação não biológica da glucose 6 CO2 + 36 H2O + 30(32) ATP C6H12O6 + 6 O2 + 30(32) Pi + 30(32) ADP + 30 H + Oxidação Biológica aeróbia da glucose Energia na forma de ATP (glucose) Enzimas Piruvato Piruvato glucose CICLO DE KREBS FOSFORILAÇÃO OXIDATIVA Acetil CoA Carboidratos Carboidratos Citoplasma Mitocôndria Membrana Plasmática ATP G L I C Ó L I S E Degradação de carboidratos na célula para obtenção de energia ATP ATP Glicólise (Glucose 2 piruvatos) http://205.246.6.53/cooper/4e/animations0303.html http://205.246.6.53/cooper/4e/animations0303.html Figure 2-71. An outline of glycolysis. Each of the 10 steps shown is catalyzed by a different enzyme. Note that step 4 cleaves a six-carbon sugar into two three-carbon sugars, so that the number of molecules at every stage after this doubles. As indicated, step 6 begins the energy generation phase of glycolysis, which causes the net synthesis of ATP and NADH molecules (see also Panel 2-8). Resumo da Glicólise (Glucose 2 piruvatos) http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=mboc4.box.293 http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=mboc4.box.293 http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=mboc4.box.293 http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=mboc4.box.293 http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=mboc4.box.293 Nicotinamida adenina dinucleotídeo (NAD+) NADH Nicotinamida Adenina Piruvato Piruvato glucose CICLO DE KREBS FOSFORILAÇÃO OXIDATIVA Acetil CoA Carboidratos Carboidratos Citoplasma Mitocôndria Membrana Plasmática ATP G L I C Ó L I S E Degradação de carboidratos na célula para obtenção de energia ATP ATP Conversão do piruvato à acetil-CoA Glicólise/ Ciclo de Krebs/ Fosforilação oxidativa Piruvato Piruvato glucose CICLO DE KREBS FOSFORILAÇÃO OXIDATIVA Acetil CoA Carboidratos Carboidratos Citoplasma Mitocôndria Membrana Plasmática ATP G L I C Ó L I S E Degradação de carboidratos na célula para obtenção de energia ATP ATP Gordura Glicerol + Ácido graxo Ácido graxo - O X I D A Ç À O Ciclo de Krebs http://205.246.6.53/cooper/4e/animations0304.html http://www.mcgrawhill.ca/school/applets/abbio/quiz/ch05/how_the_kre bs_cycle_wor.swf http://205.246.6.53/cooper/4e/animations0304.html http://www.mcgrawhill.ca/school/applets/abbio/quiz/ch05/how_the_krebs_cycle_wor.swf Flavina adenina dinucleotídeo (FAD) FADH2 Piruvato Piruvato glucose CICLO DE KREBS FOSFORILAÇÃO OXIDATIVA Acetil CoA Carboidratos Carboidratos Citoplasma Mitocôndria Membrana Plasmática ATP G L I C Ó L I S E Degradação de carboidratos na célula para obtenção de energia ATP ATP Gordura Glicerol + Ácido graxo Ácido graxo - O X I D A Ç À O Cadeia Respiratória ou cadeia transportadora de elétrons na FOSFORILAÇÃO OXIDATIVA http://highered.mcgraw- hill.com/olcweb/cgi/pluginpop.cgi?it=swf::535::535::/sites/dl/free/0072437316/120071/bio11.swf::Electron%20Transport%20System%20and%20ATP%20Synthe sisElectron http://www.nature.com/nrg/journal/v2/n5/animation/nrg0501_342a_swf_MEDIA1.html NADH + H+ NAD+ FADH2 FADH + H+ ATP sintase http://highered.mcgraw-hill.com/olcweb/cgi/pluginpop.cgi?it=swf::535::535::/sites/dl/free/0072437316/120071/bio11.swf::Electron%20Transport%20System%20and%20ATP%20SynthesisElectronhttp://www.nature.com/nrg/journal/v2/n5/animation/nrg0501_342a_swf_MEDIA1.html http://www.nature.com/nrg/journal/v2/n5/animation/nrg0501_342a_swf_MEDIA1.html ATP sintase Figure 14-15. ATP synthase. (A) The enzyme is composed of a head portion, called the F1 ATPase, and a transmembrane H+ carrier, called F0. Both F1 and F0 are formed from multiple subunits, as indicated. A rotating stalk turns with a rotor formed by a ring of 10 to 14 c subunits in the membrane (red). The stator (green) is formed from transmembrane a subunits, tied to other subunits that create an elongated arm. This arm fixes the stator to a ring of 3α and 3β subunits that forms the head. (B) The three- dimensional structure of the F1 ATPase, determined by x-ray crystallography. This part of the ATP synthase derives its name from its ability to carry out the reverse of the ATP synthesis reaction—namely, the hydrolysis of ATP to ADP and Pi, when detached from the transmembrane portion. (B, courtesy of John Walker, from J.P. Abrahams et al., Nature 370:621–628, 1994. © Macmillan Magazines Ltd.) Metabolismo aeróbio x Metabolismo anaeróbio da glucose Degradação de carboidratos na célula para obtenção de energia Integração da produção de NADH e FADH2 e da produção de ATP Figure 2-87. Glycolysis and the citric acid cycle provide the precursors needed to synthesize many important biological molecules. The amino acids, nucleotides, lipids, sugars, and other molecules—shown here as products—in turn serve as the precursors for the many macromolecules of the cell. Each black arrow in this diagram denotes a single enzyme-catalyzed reaction; the red arrows generally represent pathways with many steps that are required to produce the indicated products. Coenzima A -oxidação (Ácido graxo acetil-CoA) http://www.brookscole.com/chemistry_d/templates/student_resources/shared_resour ces/animations/carnitine/carnitine1.html http://library.med.utah.edu/NetBiochem/FattyAcids/9_3.html http://www.brookscole.com/chemistry_d/templates/student_resources/shared_resources/animations/carnitine/carnitine1.html Produtos fornecidos a partir da oxidação de açúcares e de gorduras Gordura Proteína Piruvato Piruvato Glicerol + Ácido graxo Ácido graxo Proteína aminoácidos glucose CICLO DE KREBS FOSFORILAÇÃO OXIDATIVA Acetil CoA Carboidratos Carboidratos Citoplasma Mitocôndria Membrana Plasmática Envolvimento da mitocôndria na produção de energia G L I C Ó L I S E - O X I D A Ç À O ATP Etapas da -oxidação http://www.wiley.com/legacy/college/boyer/0470003790/animatio ns/fatty_acid_metabolism/fatty_acid_metabolism.htm8 http://www.brookscole.com/chemistry_d/templates/student_resources/shared_resour ces/animations/carnitine/carnitine1.html http://library.med.utah.edu/NetBiochem/FattyAcids/9_3.html http://www.wiley.com/legacy/college/boyer/0470003790/animations/fatty_acid_metabolism/fatty_acid_metabolism.htm8 http://www.wiley.com/legacy/college/boyer/0470003790/animations/fatty_acid_metabolism/fatty_acid_metabolism.htm8 http://www.brookscole.com/chemistry_d/templates/student_resources/shared_resources/animations/carnitine/carnitine1.html
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