The MAZ protein is an autoantigen of Hodgkin's disease and paraneoplastic cerebellar dysfunction

Authors

  • Luis Bataller MD,

    1. Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR.
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  • Deborly F. Wade BS,

    1. Department of Microbiology and Immunology, University of Arkansas for Medical Sciences, Little Rock, AR.
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  • Francesc Graus MD,

    1. Department of Neurology, Hospital Clinic, Barcelona, Spain
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  • Myrna R. Rosenfeld MD, PhD,

    1. Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR.
    2. Department of Microbiology and Immunology, University of Arkansas for Medical Sciences, Little Rock, AR.
    3. Central Arkansas Veterans Health Care System, Little Rock, AR.
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  • Josep Dalmau MD, PhD

    Corresponding author
    1. Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR.
    2. Department of Microbiology and Immunology, University of Arkansas for Medical Sciences, Little Rock, AR.
    • Department of Neurology, University of Pennsylvania, 3400 Spruce Street, 3 W. Gates, Philadelphia, PA 19104
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Abstract

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

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