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

Ann Neurol. 2003 Jan;53(1):123-7. doi: 10.1002/ana.10434.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Autoantibodies / blood
  • Autoantigens / genetics
  • Autoantigens / immunology*
  • Cell Adhesion Molecules / metabolism
  • DCC Receptor
  • DNA-Binding Proteins
  • Epitopes
  • Gene Library
  • Hodgkin Disease / immunology*
  • Humans
  • Paraneoplastic Cerebellar Degeneration / immunology*
  • Purkinje Cells / immunology
  • Receptors, Cell Surface
  • Transcription Factors / genetics
  • Transcription Factors / immunology*
  • Transcription Factors / metabolism
  • Tumor Suppressor Proteins / metabolism

Substances

  • Autoantibodies
  • Autoantigens
  • Cell Adhesion Molecules
  • DCC Receptor
  • DCC protein, human
  • DNA-Binding Proteins
  • Epitopes
  • Receptors, Cell Surface
  • Transcription Factors
  • Tumor Suppressor Proteins
  • c-MYC-associated zinc finger protein