| First Author | Buckanovich RJ | Year | 1996 |
| Journal | J Neurosci | Volume | 16 |
| Issue | 3 | Pages | 1114-22 |
| PubMed ID | 8558240 | Mgi Jnum | J:306713 |
| Mgi Id | MGI:6718242 | Doi | 10.1523/JNEUROSCI.16-03-01114.1996 |
| Citation | Buckanovich RJ, et al. (1996) The onconeural antigen Nova-1 is a neuron-specific RNA-binding protein, the activity of which is inhibited by paraneoplastic antibodies. J Neurosci 16(3):1114-22 |
| abstractText | Nova-1, a protein expressed in tumors and neurons, is a target antigen in a human paraneoplastic motor disorder [paraneoplastic opsoclonus-myoclonus ataxia (POMA)]. We evaluated the relationship between the function of Nova-1 and its role as a disease antigen. We show that Nova-1 is a neuron-specific RNA-binding protein with sequence and functional similarities to FMR-1. Nova-1 mRNA is restricted to the subcortical nervous system, and the protein binds to RNA with high affinity. Nova-1 KH domains mediate this RNA binding, and point mutations within them abrogate binding. POMA disease antisera (6/6) recognize the third KH domain but not an inactive point mutant, and affinity-purified antibody blocks Nova-1 RNA binding. Thus, a cardinal feature of POMA is the production of antibodies that inhibit Nova-1-RNA interactions, suggesting such inhibition may cause the neurological disease. |
