The MAZ protein is an autoantigen of Hodgkin's disease and paraneoplastic cerebellar dysfunction.
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.
Bataller
Luis
L
Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, USA.
Wade
Deborly F
DF
Graus
Francesc
F
Rosenfeld
Myrna R
MR
Dalmau
Josep
J
D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000235:genetics / Q000276:immunology*; D015815:Cell Adhesion Molecules / Q000378:metabolism; D000075389:DCC Receptor; D004268:DNA-Binding Proteins; D000939:Epitopes; D015723:Gene Library; D006689:Hodgkin Disease / Q000276:immunology*; D006801:Humans; D020362:Paraneoplastic Cerebellar Degeneration / Q000276:immunology*; D011689:Purkinje Cells / Q000276:immunology; D011956:Receptors, Cell Surface; D014157:Transcription Factors / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D025521:Tumor Suppressor Proteins / Q000378:metabolism
D001323:Autoantibodies; D001324:Autoantigens; D015815:Cell Adhesion Molecules; D000075389:DCC Receptor; C501909:DCC protein, human; D004268:DNA-Binding Proteins; D000939:Epitopes; D011956:Receptors, Cell Surface; D014157:Transcription Factors; D025521:Tumor Suppressor Proteins; C076035:c-MYC-associated zinc finger protein