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bare lymphocyte syndrome

   Also found in: Acronyms, Wikipedia 0.03 sec.
bare lymphocyte syndrome (bâr)
n.
The absence of human leukocyte antigens on peripheral mononuclear cells, which may result in immunodeficiency.

bare lymphocyte syndrome,
an immune deficiency condition caused by defective beta-2 microglobulin, one of the major histocompatibility antigens on cell surfaces. It is inherited as an autosomal-recessive trait. The deficiency causes a severe combined immunodeficiency resulting from the lack of antigen presentation by type I and/or type II major histocompatibility complex.


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Children born with the disease, called Bare Lymphocyte Syndrome, have a defective promoter protein that does not attach to its target on a key MHC gene.
 
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