Page 1909 - Hematology_ Basic Principles and Practice ( PDFDrive )

Chapter 110 Human Blood Group Antigens and Antibodies 1689

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Possible adhesion Water transport Ligand for integrins Part of the complement cascade Transport; possible neuro-transmitter Structural Interacts with protein 4.1 and p55 Complement regulation; binds C3b; disassembles C3/C5 convertase Complement regulation; binds C3b and C4b; mediates phagocytosis Binds hyaluronic acid; mediates adhesion of leukocytes Possible adhesion

Function Enzymatic Glycosylation

Disease Association Absent from PNH III RBCs Monosomy 7, congenital dyserythropoietic anemia Depressed in some malignant diseases; decreased in Rh null syndrome Certain phenotypes increased susceptibility to autoimmune conditions and infections C4-deficient predisposes for SLE Decreased in some tumor cells Increased in hematopoietic stress X-linked midlife onset neuropathy, elevated CPK, muscular dystrophy, acanthocytosis; sometimes associated wit

Present in Other Tissue Lymphocytes, spleen, lymph nodes, GI, ovary, testes, heart, liver Broad tissue distribution Kidney, liver, gallbladder, eye, capillary endothelium Adsorbed from plasma Broad distribution Soluble—all fluids except CSF in secretors Fetal liver, adult skeletal muscle, brain, pancreas, heart Fetal liver, renal endothelium Vascular endothelium Epithelial GI, GU, CNS Soluble form in plasma and urine Blood cells, glomerular podocytes,

Principal Associated Blood Group Antigens (null phenotype) Sc1, Sc2, Sc3, Rd (Sc −1, −2, −3) Do a , Do b , Gy a , Hy, Jo a (Gy(a−)) Co a , Co b , Co3 (Co(a−b−)) LW a , LW b , LW ab (LW(a−b−)) Ch1, Ch2, Rg1 H (Bombay O h) Kx (McLeod) Ge2, Ge3, Ge4 (Leach phenotype) Cr a , Tc a , Tc b , Tc c , Dr a , Es a , IFC (Inab) Kn a , Kn b , McC a , Sl a , Yk a , KCAM (no nulls reported) In a , In b Ok a MER2 (Raph−)

Component Name ERMAP Do glycoprotein; ART 4 Aquaporin LW glycoprotein ICAM-4 C4A; C4B Carbohydrate XK glycoprotein GPC GPD DAF CR1 Hermes antigen Basigin CD151

Predicted Topology (Number of Amino Acids [AA]) Type I (475 AA) GPI-linked (314 AA) Multipass—6 spans (269 AA) Type I IgSF (241 AA) Not endogenous to RBC (1191 AA) Fucosyl-transferase Type II (365 AA) Multipass—10 spans (444 AA) Type I (128 AA) (107 AA) GPI-linked (347 AA) Type I (1998 AA) Type I (341 AA) Type I IgSF (248 AA) Multipass—4 spans (253 AA)

Gene Name ERMAP (SC) ART4 (DO) AQP1 (CO) ICAM4 (LW) C4A, C4B (CH/RG) FUT1(H) XK (XK) GYPC (GE) CD55 (CROM) CR1 (KN) CD44 (IN) BSG (OK) MER2

ISBT System Name (Number) Scianna (013) Dombrock (014) Colton (015) Landsteiner- Wiener (016) Chido/ Rodgers (017) H (018) Kx (019) Gerbich (020) Cromer (021) Knops (022) Indian (023) Ok (024) RAPH (025)