Cerebellar degeneration and autoimmunity to zinc-finger proteins of the cerebellum

Neurology. 2002 Dec 24;59(12):1985-7. doi: 10.1212/01.wnl.0000038352.01415.ce.

Abstract

The serum of a patient with subacute cerebellar dysfunction was used to probe a cDNA expression library and isolate two genes: Zic1 (zinc-finger of the cerebellum) and Zic4. The patient had intrathecal synthesis of Zic antibodies, suggesting that the Zic proteins were autoantigens of the neurologic disorder. The Zic proteins are involved in cerebellar development and are reported as being preferentially expressed by medulloblastomas. It was found that the expression of Zic proteins is enriched in, but not limited to, medulloblastomas and primitive neuroectodermal tumors.

Publication types

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

MeSH terms

  • Aged
  • Autoantibodies / analysis
  • Autoantibodies / cerebrospinal fluid
  • Autoantibodies / immunology
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology*
  • Blotting, Western
  • Cerebellar Diseases / pathology*
  • Cerebellar Neoplasms / immunology
  • Cerebellar Neoplasms / pathology
  • Cerebellum / pathology*
  • DNA, Complementary / genetics
  • Humans
  • Immunohistochemistry
  • Magnetic Resonance Imaging
  • Male
  • Medulloblastoma / immunology
  • Medulloblastoma / pathology
  • Nerve Degeneration / pathology*
  • Neuroectodermal Tumors / immunology
  • Neuroectodermal Tumors / pathology
  • Zinc Fingers / immunology*

Substances

  • Autoantibodies
  • DNA, Complementary