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Energy Metabolism         141


                 A. Components of the respiratory chain


                 Pyruvate             Liponamide-H 2                     E  OI         Complex I
                 2-Oxoglutarate      HS  SH                              (V
                                                        H                    NADH dehydrogenase (ubiquinone)
                                                                             1.6.5.3
                                                                             M r 700 - 800 kDa, 25 - 30 subunits
                                                      I                 -0.3
                                                                             1 FMN, 2 Fe S , 4 - 5 Fe S
                                                                                      2 2
                                                                                               4 4
                                                              Succinate
                 3-Hydroxybutyrate                                                     Complex II
                 3-Hydroxyacyl CoA   N   A                                   Succinate dehydrogenase 1.3.5.1
                 Malate
                 Isocitrate          NADH                                    M r 125 kDa, 4 - 6 subunits
                                                      Q        II       +0.1  1 FAD, 1 Fe S , 1 Fe S , 1Fe S
                                                                                      2 2
                                                                                             4 4
                                                                                                   3 4
                                                                             2 ubiquinone, 1 heme b
                                                                                      Complex III
                                                        H
                                                              Fumarate       Ubiquinol-cytochrome C
                 Acyl CoA                             III                    reductase 1.10.2.2
                 α-Glycerophosphate   ETF                                    M r ≈ 400 kDa, 11 subunits
                 Dihydroorotat
                 Cholin                                                      2 Fe S , 2 heme b, 1 heme c 1
                                                                                2 2
                                                                                      Complex IV
                                                          Cytochrome C
                                                          M r 12 kDa         Cytochrome C oxidase 1.9.3.1
                         nH                               1 heme
                                                                        +0.3 M r ≈ 200 kDa, 8 - 13 subunits
                                                                             2 Cu, 1 Zn, 1 heme a, 1 heme a 3
                            V
                                                        H
                                                                                      Complex V
                                                                 CN
                                                      IV         CO          H   -transporting ATP synthase
                 A                A                                          3.6.1.34
                    P P  P  nH       PP P
                                                                             M r >400 kDa, >20 subunits
                                                         O 2
                                                                2 H
                                                 1/2 O 2                                Electron flow
                                                                        +0.8            Proton flow
                                                            H O
                                                             2
                 B. Organization




                                                                                                 Outer
                                                                                                 mitochon-
                                                                                                 drial
                                                                                                 membrane
                                                                                                 Inter-
                                                                                                 membrane
                                 4 H           4 H                2 H                            space
                                       II
                                                                                                 Inner
                            I                       Cyto-       ?                                mitochon-
                                     Q             chrome C            ∆p                        drial
                                                III        IV                V                   membrane

                      2 e                   4 H       2-4 H

                             4 H                         1/2 O 2  2    3 P                        Matrix
                                                  2 H           O                                 space
                 N   A                    N   A                      3  A           3  A
                                                                           P P            PP P
                                                                                        ATP
                           Tricarboxylic  NAD           H O            10 H
                 NADH+H      acid cycle                  2
                            β-Oxidation




           Koolman, Color Atlas of Biochemistry, 2nd edition © 2005 Thieme
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