Kell antigen system

Source: Wikipedia, the free encyclopedia.
Kell protein
Identifiers
SymbolKEL
Alt. symbolsECE3, CD238
Chr. 7 q33
Search for
StructuresSwiss-model
DomainsInterPro

The Kell antigen system (also known as the Kell–Cellano system) is a

endothelin-3.[2][3]

Protein

The KEL gene encodes a type II transmembrane

Kx antigen. The encoded protein contains sequence and structural similarity to members of the neprilysin (M13) family of zinc endopeptidases.[6]

There are several

genetic deletion or through a single point mutation within the coding region of the XK gene[7]), 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.[8]

The Kell protein has also recently been designated CD238 (cluster of differentiation 238).

Disease association

Interpretation of antibody panel to detect patient antibodies towards the most relevant human blood group systems, including Kell.
Further information: Blood compatibility testing

Kell antigens are important in transfusion medicine, autoimmune hemolytic anemia and hemolytic disease of the newborn (anti-Kell). Anti-K is the next most common immune red cell antibody after those in the ABO and Rh system. Anti-K typically presents as IgG class alloantibody. Individuals lacking a specific Kell antigen may develop antibodies against Kell antigens when transfused with blood containing that antigen. This is particularly true for the "K" antigen which shows a relatively high antigenicity and moderately low frequency (~9%) in Caucasian populations. Anti-K can also occur following transplacental hemorrhage (TPH) associated with childbirth making Kell an important concern for hemolytic disease of the newborn. Following the formation of anti-K, subsequent blood transfusions may be marked by destruction of the new cells by these antibodies, a process known as hemolysis. Anti-K does not bind complement, therefore hemolysis is extravascular. Individuals without K antigens(K0) who have formed an antibody to a K antigen, must be transfused with blood from donors who are also K0 to prevent hemolysis.[citation needed]

Rhesus blood group systems.[citation needed
]

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 of unknown function. The XK appears to be required for proper synthesis or presentation of the Kell antigens on the red blood cell surface.[citation needed
]

History

The Kell group was named after the first patient described with antibodies to K1, a pregnant woman named Mrs. Kellacher in 1945.

Harvard dental student, in 1961.[11][12]
King
Henry VIII of England may have had Kell-positive blood type, explaining the deaths of seven of his ten children at, or soon after, birth, and suggesting that his mental deterioration around age 40 could be explained by McLeod Syndrome;[13] this was supported by the revelation that Henry may have inherited Kell from his maternal great-grandmother, Jacquetta of Luxembourg.[14]

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.[15][16] Bitter taste receptor proteins in the taste buds of the tongue that recognise PTC are encoded on nearby chromosome locus 7 q35-6.[citation needed]

References

  1. ISSN 1751-2816
    .
  2. PMID 7849312
    .
  3. PMID 10438732. Archived from the original
    on 2013-04-14.
  4. ^ Russo DC, Lee S, Reid M, Redman CM. Topology of Kell blood group protein and the expression of multiple antigens by transfected cells. Blood. 1994 Nov 15;84(10):3518-23.
  5. ^ Russo DC, Redman C, Lee S. Association of XK and Kell blood group proteins. J Biol Chem. 1998 May 29;273(22):13950-6.
  6. ^ "Entrez Gene: KEL".
  7. S2CID 41663851
    .
  8. PMID 11134029
    .
  9. PMID 8623782
    .
  10. ^ 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
  11. S2CID 4187507
    .
  12. S2CID 30275809
    .
  13. S2CID 159499333
    .
  14. PMID 24350322
    .
  15. PMID 4435792
    .
  16. PMID 976995
    .

External links
Retrieved from "https://en.wikipedia.org/w/index.php?title=Kell_antigen_system&oldid=1116883705"