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The MAZ protein is an autoantigen of Hodgkin's disease and paraneoplastic cerebellar dysfunction
Author(s) -
Bataller Luis,
Wade Deborly F.,
Graus Francesc,
Rosenfeld Myrna R.,
Dalmau Josep
Publication year - 2003
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.10434
Subject(s) - cerebellum , zinc finger , medicine , paraneoplastic cerebellar degeneration , epitope , antibody , disease , immune system , immunology , cancer research , biology , gene , transcription factor , autoantibody , genetics
Probing a cerebellar expression library with TrAb sera from patients with Hodgkin's disease and paraneoplastic cerebellar degeneration resulted in the isolation of MAZ ( myc ‐associated zinc‐finger protein). Eleven of 19 TrAb sera and 16 of 131 controls reacted with MAZ, indicating a significant, although not specific, association between Tr and MAZ immunities ( p < 0.001). Interestingly, 9 of 16 positive control patients also had cerebellar dysfunction. Purified MAZ antibodies reacted with Purkinje cells. In neuronal cells, MAZ interacts with DCC ( Deleted in Colorectal Cancer product), the receptor for netrin‐1, a neuronal survival factor. These findings suggest epitope spreading between the Tr antigen and the MAZ–DCC complex and offer a possible model of immune‐mediated cerebellar disease. Ann Neurol 2003;53:000–000