autoimmune lymphoproliferative syndrome type 4

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autoimmune lymphoproliferative syndrome type 42016-11-24T20:53:52+00:00

autoimmune lymphoproliferative syndrome type 4

HAEMATOLOGY, IMMUNOLOGY

Definition A disorder (OMIM:614470) of apoptosis, which is characterised by chronic accumulation of non-malignant lymphocytes, defective lymphocyte apoptosis, and an increased risk of haematologic malignancy.

Molecular pathology Defects of NRAS, which encodes  a membrane protein with intrinsic GTPase activity, cause autoimmune lymphoproliferative syndrome type 4. 

Synonym Autoimmune lymphoproliferative syndrome type IV

References http://www.uniprot.org/uniprot/P01111

http://www.omim.org/entry/614470

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