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Abstract
This chapter discusses the Kell blood group system. The terminology, expression, database accession numbers, and the carrier molecule related to the Kell blood group system are also reviewed. The Kell blood group was named in 1946 after the first antibody producer (Kelleher) of anti-K that caused HDN. The carrier molecule is a single-pass red blood cell (RBC) membrane glycoprotein (type II) that is highly folded via disulphide bonds. In the RBC membrane Kell glycoprotein is covalently linked at Cys72 to the Cys347 of the XK protein. The function and disease association of the Kell blood group system is also reviewed. Kell glycoprotein is an endothelin-3-converting enzyme, preferentially cleaving big endothelin-3, a 41 amino acid polypeptide, at Trp21-Ile22, creating bioactive endothelin-3, a potent vasoconstrictor. Kell, in common with all zinc endopeptidases, shares a pentameric sequence, HEXXH, which is central to zinc binding and catalytic activity.
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