NCIt definition : Human NOVA1 wild-type allele is located within 14q and is approximately 152 kb in
length. This allele, which encodes RNA-binding protein Nova-1, is involved in the
regulation of RNA splicing or metabolism in a specific population of developing neurons.;
NCIt note : Human NOVA1 Gene (NOVA Family) at 14q encodes NOVA-1, a neuron-specific nuclear RNA-binding
protein containing three KH domains. Alternative transcript variants differ by a seventy-two
base pair exon H, as well as by a nine base pair motif. NOVA-1 may regulate RNA splicing
or metabolism in specific developing neurons. Murine Nova1 is expressed in ventral
spinal cord, brain stem nuclei, deep cerebellar nuclei, and hypothalamus. It binds
RNA in a sequence-specific manner to regulate neuron-specific splicing of inhibitory
receptor pre-mRNAs in postnatal motor neurons. Nova1 also specifically binds to the
GLRA2 intronic sequence (UCAUY)3. Antibodies against NOVA-1 occur in paraneoplastic
opsoclonus-ataxia (POA).;