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2332           Part XIII:  Transfusion Medicine                                                                                                                       Chapter 136: Erythrocyte Antigens and Antibodies           2333





                TABLE 136–1.  Characteristics of International Society of Blood Transfusion Defined Blood Group Systems and Antigen
                Collections (Continued)
                                                                                    Function of RBC
                              ISBT                   ISBT Gene                      Membrane
                Conventional   Symbol   Chromosome   Name (ISGN If   Associated Antigens   Component
                Name          (No.)     Location     Different)   [Null Phenotype]  (CD No.)        Disease Association
                H             H (018)   19q13.33     H (FUT1)     H [Bombay, O ]    Glycocalyx (CD   Decreased in some tumor
                                                                            h
                                                                                    173)            cells, increased in hemato-
                                                                                                    poietic stress
                Kx            XK (019)  Xp21.1       XK           Kx [McLeod]       Possible neuro-  Acanthocytosis, muscular
                                                                                    transmitter, func-  dystrophy, hemolytic ane-
                                                                                    tion in RBCs not   mia; McLeod syndrome
                                                                                    known           sometimes associated
                                                                                                    with CGD, peripheral neu-
                                                                                                    ropathy, cardiomyopathy
                                                                                                    seizures, a late-onset
                                                                                                    dementia, and behavioral
                                                                                                    changes
                Gerbich       GE (020)  2q14.3       GE (GYPC)    Ge2, Ge3, Ge4,    Membrane attach-  Hereditary elliptocyto-
                                                                  and 8 more [Leach   ment; interacts   sis, hemolytic anemia,
                                                                  phenotype]        with 4.1R and p55   decreased 4.1R and p55
                                                                                    (CD236)
                                                                            c
                                                                               a
                Cromer        CROM      1q32.2       CROM (CD55;   Cr , Tc , Tc , Tc , Dr ,   Complement   Absent from PNH III RBCs,
                                                                         b
                                                                    a
                                                                       a
                                                                                                      a
                              (021)                  DAF)         and 13 more [Inab   regulation, binds   Dr  is the receptor for uro-
                                                                  phenotype]        C3b, disassembles   pathogenic E. coli
                                                                                    C3/C5 convertase
                                                                                    (CD55)
                Knops         KN (022)  1q32.2       KN (CR1)     Kn , Kn , McC , SI , Yk ,  Complement reg-  Antigens depressed in
                                                                        b
                                                                            a
                                                                                  a
                                                                               a
                                                                    a
                                                                  and 4 more [Helge-  ulation, binds C3b   certain autoimmune and
                                                                  son phenotype]    and C4b, mediates   malignant conditions
                                                                                    phagocytosis
                                                                                    (CD35)
                Indian        IN (023)  11p13        IN (CD44)    In , In , INFI, INJA  Binds hyaluronic   Depressed in pregnancy,
                                                                      b
                                                                    a
                                                                                    acid, mediates   congenital dyserythropoi-
                                                                                    adhesion of leuko-  etic anemia
                                                                                    cytes (CD44)
                Ok            OK (024)  19p13.3      OK (BSG)     Ok , OKGV,        Possible adhesion
                                                                    a
                                                                  OKVM              (CD147)
                Raph          RAPH (025)  11p15.5    MER2 (CD151)  MER2 [RAPH ]     Adhesion molecule  Renal disease, associated
                                                                            null
                                                                                    involved in kidney   with pretibial epidermoly-
                                                                                    function (CD151)  sis bullosa and sensori-
                                                                                                    neural deafness
                John Milton   JMH (026)  15q24.1     JMH (SEMA7A)  JMH, and 5 more  Adhesion mole-  Absent from PNH III RBCs
                Hagen                                                               cule, function in
                                                                                    RBCs not known
                                                                                    (CD108)
                I             I (027)   6p24.2       GCNT2        I [I–; i adult]   Glycocalyx      Congenital cataracts in
                                                                                                    Asians
                GLOB          Globoside   3q26.1     P (β3GALNT1)  P, PX2 [P–]      Glycocalyx      Receptor E. coli and parvo-
                              (028)                                                                 virus B19
                Gil           GIL (029)  9p13.3      GIL (AQP3)   GIL [GIL–]        Glycerol/water/
                                                                                    urea transporter
                Rh-associated   RHAG (030) 6p21.3    RHAG         Duclos, and 3 more  Possibly trans-  Hemolytic anemia, heredi-
                glycoprotein                                                        ports CO , or NH    tary stomatocytosis
                                                                                                3
                                                                                           2
                                                                                    (CD241)
                FORS          FORS (031)  9q34.2     GBGT1        FORS1             Glycocalyx
                JR            JR (032)  4q22.1       JR, ABCG2    Jr  [Jr(a–)]      ATP-dependent
                                                                    a
                                                                                    transporter
                Lan           LAN (033)  2q36        LAN, ABCB6   Lan [Lan–]        ATP-dependent
                                                                                    transporter
                                                                                                                (continued )




          Kaushansky_chapter 136_p2327-2352.indd   2332                                                                 9/21/15   4:30 PM
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