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Encyclopedia > Kell antigen system
[[Image:|250px|Kell antigen system chemical structure]]
Kell protein
Identifiers
Symbol(s) KEL
Entrez 3792
OMIM 110900
RefSeq NM_000420
UniProt P23276
PDB [1]
Other data
EC number [2]
Locus Chr. 7 q33

The Kell antigen system (also known as Kell-Cellano system) is a group of antigens on the human red blood cell surface which are important determinants of blood type and are targets for autoimmune or alloimmune diseases which destroy red blood cells. The Kell antigens are peptides found within the kell protein, a 93 kilodalton transmembrane zinc-dependent endopeptidase which is responsible for cleaving endothelin-3.[1][2] Hugo is a masculine name. ... The Entrez Global Query Cross-Database Search System allows access to databases at the National Center for Biotechnology Information (NCBI) website. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The National Center for Biotechnology Information (NCBI) is part of the US National Library of Medicine (NLM), which is a branch of the US National Institutes of Health. ... Swiss-Prot is a curated biological database of protein sequences created in 1986 by Amos Bairoch during his PhD and developed by the Swiss Institute of Bioinformatics and the European Bioinformatics Institute. ... The Protein Data Bank (PDB) is a repository for 3-D structural data of proteins and nucleic acids. ... The Enzyme Commission number (EC number) is a numerical classification scheme for enzymes, based on the chemical reactions they catalyze. ... In biology and evolutionary computation, a locus is the position of a gene (or other significant sequence) on a chromosome. ... Chromosome 7 is one of the 23 pairs of chromosomes in humans. ... An antigen is a substance that stimulates an immune response, especially the production of antibodies. ... A total of 29 human blood group systems are recognized by the International Society of Blood Transfusion (ISBT). ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Alloimmunity is a condition in which the body gains immunity against cells from another individual of the same species. ... Peptides are the family of molecules formed from the linking, in a defined order, of various amino acids. ... The atomic mass unit (amu), unified atomic mass unit (u), or dalton (Da), is a small unit of mass used to express atomic masses and molecular masses. ... General Name, Symbol, Number zinc, Zn, 30 Chemical series transition metals Group, Period, Block 12, 4, d Appearance bluish pale gray Atomic mass 65. ...


There are several alleles of the gene which creates Kell protein. Two such alleles, K1 (Kell) and K2 (Cellano), are the most common. The kell protein is tightly bound to a second protein, XK, by a disulfide bond. Absence of the XK protein (such as through gene deletion), leads to marked reduction of the Kell antigens on the red blood cell surface. Absence of the Kell protein (K0), however, does not affect the XK protein.[3] An allele is any one of a number of viable DNA codings of the same gene (sometimes the term refers to a non-gene sequence) occupying a given locus (position) on a chromosome. ... This stylistic schematic diagram shows a gene in relation to the double helix structure of DNA and to a chromosome (right). ... A disulfide bond (SS-bond), also called a disulfide bridge, is a strong covalent bond between two sulfhydryl (-SH) groups. ... A gene deletion, or deletion mutation is a genetic mutation in which a part of a chromosome or a sequence of DNA is missing. ...

Contents


Disease association

Kell antigens are important in transfusion medicine, autoimmune hemolytic anemia, and hemolytic disease of the newborn. Individuals lacking a specific Kell antigen may develop antibodies against Kell antigens when transfused with blood containing that antigen. Subsequent blood transfusions may be marked by destruction of the new cells by these antibodies, a process known as hemolysis. People without Kell antibodies (K0), must be transfused with blood from donors who are also K0 to prevent hemolysis. Transfusion medicine (or transfusiology) is the branch of medicine that is concerned with the transfusion of blood and blood components. ... Autoimmune hemolytic anemia (AIHA) is one type of hemolytic anemias caused by excessive hemolyisis and it is identified by auto-antibodies that react with RBCs. ... Hemolytic disease of the newborn, (also known as HDN) is an alloimmune condition that develops in a fetus when IgG antibodies produced by the mother pass through the placenta and attack the fetuss red blood cells in the fetal circulation. ... Schematic of antibody binding to an antigen An antibody is a protein used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ... Hemolysis (alternative spelling haemolysis) literally means the excessive breakdown of red blood cells. ...


Autoimmune hemolytic anemia (AIHA) occurs when the body produces an antibody against a blood group antigen on its own red blood cells. The antibodies lead to destruction of the red blood cells with resulting anemia. Similarly, a pregnant woman may develop antibodies against fetal red blood cells, resulting in destruction, anemia, and hydrops fetalis in a process known as hemolytic disease of the newborn (HDN). Both AIHA and HDN may be severe when caused by anti-Kell antibodies,[4] as they are the most immunogenic antigens after those of the ABO and Rhesus blood group systems. Autoimmune hemolytic anemia (AIHA) is one type of hemolytic anemias caused by excessive hemolyisis and it is identified by auto-antibodies that react with RBCs. ... This article discusses the medical condition. ... Hydrops fetalis is a blood condition in the fetus characterized by an edema in the fetal subcutaneous tissue, sometimes leading to spontaneous abortion. ... Hemolytic disease of the newborn (anti-Kell 1) ranges from mild disease to a very severe disease. ... The ABO system is the most important blood type system in human blood transfusion. ...


McLeod phenotype

Main article: McLeod syndrome

McLeod phenotype (or McLeod syndrome) is an X-linked anomaly of the Kell blood group system in which Kell antigens are poorly detected by laboratory tests. The McLeod gene encodes the XK protein, a protein with structural characteristics of a membrane transport protein but an unknown function. The XK appears to be required for proper synthesis or presentation of the Kell antigens on the red blood cell surface. Sex-linked genes are those carried on the mammalian X chromosome but not the Y chromosome. ...


History

The Kell group was named after the first patient described with antibodies to K1, a pregnant woman named Mrs. Kellacher in 1945.[5] Mrs. Cellano was likewise a pregnant woman with the first described antibodies to K2. The K0 phenotype was first described in 1957 and the McLeod phenotype was found in Hugh McLeod, a Harvard dental student, in 1961. [6][7] The phenotype of an individual organism is either its total physical appearance and constitution or a specific manifestation of a trait, such as size, eye color, or behavior that varies between individuals. ... Harvard University is a private university in Cambridge, Massachusetts, USA, and a member of the Ivy League. ...


Other associations

Evidence supports a genetic link between the Kell blood group (on chromosome 7 q33) and the ability to taste phenylthiocarbamide, or PTC, a bitter-tasting thiourea compound. [8] [9] Bitter taste receptor proteins in the taste buds of the tongue that recognise PTC are encoded on nearby chromosome locus 7 q35-6. Chromosome 7 is one of the 23 pairs of chromosomes in humans. ... Phenylthiocarbamide, also known as PTC, or phenylthiourea, is a synthetic organic molecule. ... Thiourea is related to urea, where an oxygen atom is replaced by a sulfur atom. ... In biology and evolutionary computation, a locus is the position of a gene (or other significant sequence) on a chromosome. ...


References

  • OMIM 110900 - OMIM entry for Kell protein
  • OMIM 314850 - OMIM entry for XK protein
  1. ^ Lee S, Wu X, Reid M, Zelinski T, Redman C. Molecular basis of the Kell (K1) phenotype. Blood. 1995 Feb 15;85(4):912-6. PMID 7849312
  2. ^ Lee S, Lin M, Mele A, Cao Y, Farmar J, Russo D, Redman C. Proteolytic processing of big endothelin-3 by the kell blood group protein. Blood. 1999 Aug 15;94(4):1440-50. PMID 10438732
  3. ^ Yu LC, Twu YC, Chang CY, Lin M. Molecular basis of the Kell-null phenotype: a mutation at the splice site of human KEL gene abolishes the expression of Kell blood group antigens. J Biol Chem. 2001 Mar 30;276(13):10247-52. Epub 2000 Dec 27. PMID 11134029
  4. ^ Wiener CP, and Widness JA. Decreased fetal erythropoiesis and hemolysis in Kell hemolytic anemia. American Journal of Obstetrics and Gynecology. 1996 174: 547-55 PMID 8623782
  5. ^ Coombs RRA, Mourant AE, Race RR. A new test for the detection of weak and incomplete Rh agglutinins. Br J Exp Pathol 1945;26:255
  6. ^ Chown B, Lewis M, Kaita K. A new Kell blood-group phenotype. Nature. 1957 Oct 5;180(4588):711. PMID 13860532
  7. ^ Allen FH Jr, Krabbe SM, Corcoran PA. A new phenotype (McLeod) in the Kell blood-group system. Vox Sang. 1961 Sep;6:555-60. PMID 13477267
  8. ^ Crandall BF, Spence MA. Linkage relations of the phenylthiocarbamide locus (PTC). Hum Hered. 1974;24(3):247-52.PMID 4435792
  9. ^ Conneally PM, Dumont-Driscoll M, Huntzinger RS, Nance WE, Jackson CE. Linkage relations of the loci for Kell and phenylthiocarbamide taste sensitivity. Hum Hered. 1976;26(4):267-71. PMID 976995
Transfusion medicine - edit
Blood transfusion | Cross-matching | Coombs test | Intraoperative blood salvage | Transfusion reactions | International Society of Blood Transfusion | ISBT 128 | Platelet transfusion |
Human blood group systems - Blood type
ABO | Rhesus | Duffy | Hh | Kell | Kidd | Kx | Colton | Yt
Blood products
Blood donation | Blood | Plasma | Platelets | Cryoprecipitate | Plasmapheresis | Red blood cells 

 
 

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