gen receptor (BCR) in mature or activated B-cells leads to inhibition of a number of cellular responses such as cell proliferation, antibody production and activation, and instead promotes apoptosis, whereas co-aggregation of FcyRIIB with FceRIIB receptor in mast cells leads to an inhibition of antigen-induced degranulation and cytokine production . ZNF91 is highly expressed in T lymphocytes and acts to repress FcyRIIB expression in these cells. Deficiency of FcyRIIB in mice leads mainly to susceptibility to autoimmune disease.
Here, screening of the ZNF91 gene revealed no mutations. As no polymorphic variants were identified in the ZNF91 gene it is impossible to conclude whether both alleles are present in HS patients. Haploinsufficiency of ZNF91 may cause HS by lifting the repression on FcyRIIB leading to an inhibition of cell proliferation.
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