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BIOCHEmISTRY  ``BIOCHEMISTRY—CEllUlAR                  BIOCHEmISTRY  ``BIOCHEMISTRY—CEllUlAR          SECTION II         49




                  Cilia structure        9 doublet + 2 singlet arrangement of     Kartagener syndrome (1° ciliary dyskinesia)—
                                          microtubules  A .                        immotile cilia due to a dynein arm defect.
                                         Basal body (base of cilium below cell     Autosomal recessive. Results in  male and
                                          membrane) consists of 9 microtubule      female fertility due to immotile sperm and
                                          triplets  B  with no central microtubules.  dysfunctional fallopian tube cilia, respectively;
                                         Axonemal dynein—ATPase that links peripheral    risk of ectopic pregnancy. Can cause
                                          9 doublets and causes bending of cilium by   bronchiectasis, recurrent sinusitis, chronic
                                          differential sliding of doublets.        ear infections, conductive hearing loss, and
                                         Gap junctions enable coordinated ciliary   situs inversus (eg, dextrocardia on CXR  C ).
                                          movement.                                  nasal nitric oxide (used as screening test).
                                                                                    (Kartagener’s restaurant: take-out only; there’s
                                                                                    no dynein “dine-in”.)
                                        A                           B                           C
                                           Dynein
                                             arm                                                  R                     L
                                        Microtubule
                                               A
                                        Microtubule
                                               B
                                            Nexin









                  Sodium-potassium       Na -K  ATPase is located in the plasma   Pumpkin = pump K  in.
                                                                                                   +
                                              +
                                           +
                  pump                    membrane with ATP site on cytosolic side.   Ouabain (a cardiac glycoside) inhibits by
                                                                                              +
                                                                   +
                                          For each ATP consumed, 3 Na  leave the cell   binding to K  site.
                                                                    +
                                          (pump phosphorylated) and 2 K  enter the   Cardiac glycosides (digoxin and digitoxin)
                                                                                                        +
                                                                                                      +
                                          cell (pump dephosphorylated).             directly inhibit the Na -K  ATPase, which
                                                                                                                  2+
                                         Plasma membrane is an asymmetric lipid     leads to indirect inhibition of Na /Ca
                                                                                                              +
                                                                                                  2+
                                          bilayer containing cholesterol, phospholipids,   exchange Ž  [Ca ] i  Ž  cardiac contractility.
                                          sphingolipids, glycolipids, and proteins.
                                                 Extracellular             3Na +             2K +
                                                 space
                                                 Plasma
                                                 membrane
                                                                            P
                                                 Cytosol                                            2K +
                                                              3Na +      ATP    ADP     P
























          FAS1_2019_01-Biochem.indd   49                                                                                11/7/19   3:16 PM
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