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126            PART 2  ■  Hematopoiesis and Cellular Maturation




                                                                   Succinyl CoA + glycine                                         q                                  m


                                                                    ALA synthetase                Delat-amino-                                . T    m            m                             x
                                                                                                  levulinic acid              k        .
                                                                       Vitamin B     6
               Body cells                                                                         (ΔALA)                        T                                                                            ,


                                  ΔALA dehydrase                                                                                                        ,                   m   . T                          -

                             ΔALA                                                                                                                                                                            m
                                                                                                                             m               m                   m   . I        m                m       -

                                  Porphobilinogen                                                                               (    m            A),

                                                                                                                                    .



                                Uroporphyrinogen                                          Mitochondrium
                                                                                                                           Sum m ary of the Assem bly of a Hem oglobin
                                                                                                                           Molecule


                            Coproporphyrinogen                                Protoporphyrin + iron                        A      m              m                        m                 q                    .

                                                                                                                           T                                  :



                                  Protophyrinogen                                                                           .  T    α-        β-                                                          m

                                                                                                                                                  mRNA .
                                                                                                                            .  O           m
                                                                                Heme synthetase
                                                                                                                                                             ,                                                  -D
                                                                                                           Heme                               .

               FIGURE 6.6  S              m                .                                                                .  T    α        β

                                                                                                                                             .


                                                                                                                            .  A                        m                                m     x              -
               q                                                                          m                                                                      m              αβ    m   .

                            . T    m            m                                       k        .                          .            m         m                m                            m

               T                        x               m                                                                        m            ,      α  β         m   .

                                                                m     ,                   z     ,

                                                      .


                                                                                                                              NOTE: This is a good time to completely review the Key
               Beta Globin Locus                                                                                              Terms in the Glossary and on                                   . It is also a good

               T                                                                           q             . T                  time to complete Review Questions related to the preceding

                  q                                             ,     mm  ,         ,               . T                       content.


                                                  mm                              m     m       .
               T                                                       . E

                                  ,                                             m     m                                                                   Beta Globin Gene Clus ter

                   . T                                      x                                                                                                    Chromos ome 11





                                                                                                                                  epsilon                 gamma                             delta                beta

                                                                                                                                                        G            A





                                                                                                                            5’                                                                                                3’




                                                                                                                                                            Hb F                           Hb A2                 Hb A







                                                                                                                                                         Alpha Globin Gene Clus ter

                                                                                                                                                                 Chromos ome 16




                                                                                                                                     Zeta 2          Zeta 1                               Alpha 2            Alpha 1






                                                                                                                            5’                                                                                                3’


                                                                                                                           FIGURE 6.8  T                             . T
               FIGURE  6.7  B                                                     m                                               (β)                                 m     m       . T              mm

               H&E        . I                                                                                  -                                                                                            m             F. T

                                  m. T                                                  m                                  “        ”         , β,         m                              . T                           m    -
                   m                                       . (                 M. Biopsy Interpretation                                              (α)                                 m     m       . E

               o  the Liver, P                  , PA: L               W      m  & W  k    ,         .)                     α-                                                                        α-                      .
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