Cerebellar degeneration and autoimmunity to zinc-finger proteins of the cerebellum.
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.
Bataller
L
L
Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Wade
D F
DF
Fuller
G N
GN
Rosenfeld
M R
MR
Dalmau
J
J
D000368:Aged; D001323:Autoantibodies / Q000032:analysis / Q000134:cerebrospinal fluid / Q000276:immunology; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology*; D015153:Blotting, Western; D002526:Cerebellar Diseases / Q000473:pathology*; D002528:Cerebellar Neoplasms / Q000276:immunology / Q000473:pathology; D002531:Cerebellum / Q000473:pathology*; D018076:DNA, Complementary / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D008527:Medulloblastoma / Q000276:immunology / Q000473:pathology; D009410:Nerve Degeneration / Q000473:pathology*; D017599:Neuroectodermal Tumors / Q000276:immunology / Q000473:pathology; D016335:Zinc Fingers / Q000276:immunology*
D001323:Autoantibodies; D018076:DNA, Complementary