Intracellular assembly of Kell and XK blood group proteins

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Study of Kell blood group genotype in alloimmiunized thalassemia patients

Abstract Background and Objectives Alloimmunization is the most serious problem in thalassemia patients and Anti-K is the most prevalent antibody in these patients. So accurate identification of this antigen can significantly decrease the rate of alloimmunization. Serological phenotyping is usually not reliable in multi-transfused patients. Molecular genotyping can overcome limitations of hema...

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Identification of a defect in the intracellular trafficking of a Kell blood group variant.

Blood group polymorphisms have been used as tools to study the architecture of the red blood cell (RBC) membrane. Some blood group variants have reduced antigen expression at the cell surface. Understanding the underlying mechanism for this reduced expression can potentially provide structural information and help to elucidate protein trafficking pathways of membrane proteins. The Kp(a+) phenot...

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A combination of the effects of rare genotypes at the XK and KEL blood group loci results in absence of Kell system antigens from the red blood cells.

The 22 antigens of the Kell blood group system are located on a red blood cell (RBC) membrane glycoprotein that shows sequence homology with a family of metalloendopeptidases. Expression of the Kell system antigens is partially governed by XK, an X-linked gene that encodes the Kx protein; absence of Kx results in reduced Kell antigen expression. Almost total absence of Kell antigens from the RB...

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Inactivation of Kell blood group antigens by 2-aminoethylisothiouronium bromide.

Human red cells incubated with a solution containing 6% 2-aminoethylisothiouronium bromide (AET) lose activity of antigens that are part of, or related to, the Kell blood group system. However, Kx antigen is not inactivated. Studies on a wide range of other blood-group antigens show no other evidence of changes and AET appears to react specifically with red-cell membrane structures that have Ke...

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Expression of Kell blood group protein in nonerythroid tissues.

The Kell blood group protein is a zinc endopeptidase that yields endothelin-3, a potent bioactive peptide, by cleavage of big endothelin-3, a larger intermediate precursor. On red cells, Kell protein is linked by a single disulfide bond to XK, a protein that traverses the membrane 10 times and whose absence, as occurs in the McLeod phenotype, is associated with a set of clinical symptoms that i...

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ژورنال

عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Biomembranes

سال: 1999

ISSN: 0005-2736

DOI: 10.1016/s0005-2736(99)00148-0