Nova, the paraneoplastic Ri antigen, is homologous to an RNA-binding protein and is specifically expressed in the developing motor system

  title={Nova, the paraneoplastic Ri antigen, is homologous to an RNA-binding protein and is specifically expressed in the developing motor system},
  author={Ronald J. Buckanovich and Jerome B. Posner and Robert B. Darnell},

The onconeural antigen Nova-1 is a neuron-specific RNA-binding protein, the activity of which is inhibited by paraneoplastic antibodies

It is shown that Nova- 1 is a neuron-specific RNA-binding protein with sequence and functional similarities to FMR-1, a target antigen in a human paraneoplastic motor disorder and suggesting such inhibition may cause the neurological disease.

The neuronal RNA-binding protein Nova-2 is implicated as the autoantigen targeted in POMA patients with dementia.

  • Y. YangG. YinR. Darnell
  • Biology
    Proceedings of the National Academy of Sciences of the United States of America
  • 1998
The results demonstrate that the immune response in POMA targets a family of highly related sequence-specific neuronal RNA-binding proteins, and the expression pattern of the Nova-2 protein is likely to underlie the development of cognitive deficits in some PomA patients.

Paraneoplastic Encephalomyelitis Antigens Bind to the AU-rich Elements of mRNA

It is reported here that the Hu antigens bind avidly to the AU-rich element resident in many mRNAs that regulate cell proliferation, which suggests that theHu antIGens promote neuronal differentiation by suppressing the neuroblast cell cycle.

The neuronal RNA binding protein Nova-1 recognizes specific RNA targets in vitro and in vivo

Nova-1 functions as a sequence-specific nuclear RNA binding protein in vivo; disruption of the specific interaction between Nova-1 and GlyR alpha2 pre-mRNA may underlie the motor dysfunction seen in POMA.

A Post-Transcriptional Regulatory Mechanism Restricts Expression of the Paraneoplastic Cerebellar Degeneration Antigen cdr2 to Immune Privileged Tissues

It is found that the cdr2 gene, which encodes a cytoplasmic leucine zipper protein of unknown function, is expressed in PCD-associated tumors, whereas other cdr genes are not; thus, cdr1 encodes the PCD tumor antigen and is compatible with the autoimmune model of PCD pathogenesis.

A brain-enriched polypyrimidine tract-binding protein antagonizes the ability of Nova to regulate neuron-specific alternative splicing.

It is suggested that brPTB is a tissue-restricted RNA binding protein that interacts with and inhibits the ability of Nova to activate exon selection in neurons.

Mouse chromosomal locations of nine genes encoding homologs of human paraneoplastic neurologic disorder antigens.

The mouse chromosomal locations of nine genes suggest that the Hua-Hud genes arose from gene duplication and dispersion, while the other genes are dispersed in the genome.

Ma1, a novel neuron- and testis-specific protein, is recognized by the serum of patients with paraneoplastic neurological disorders.

It is demonstrated that some patients with paraneoplastic neurological disorders develop antibodies against Mal, a new member of an expanding family of 'brain/testis' proteins.



Cloning of a leucine-zipper protein recognized by the sera of patients with antibody-associated paraneoplastic cerebellar degeneration.

The occurrence of leucine-zipper and zinc-finger motifs in the predicted open reading frame suggests that this protein plays a role in the regulation of gene expression in Antibody-associated paraneoplastic cerebellar degeneration.

Differential expression of two types of the neurofibromatosis type 1 (NF1) gene transcripts related to neuronal differentiation.

It is proposed that the differential expression of type I and type II NF1-GRD transcripts might be an 'on/off' switch that regulates the catalytic activity of the NF1 gene product, which plays an important role in the regulation of neuronal differentiation.

Antiserum from a patient with cerebellar degeneration identifies a novel protein in Purkinje cells, cortical neurons, and neuroectodermal tumors

Using a novel antineuronal autoantibody, anti-Nb antibody is identified and characterized antigens present in a subset of neurons in the CNS and in some neuroectodermal tumor lines, which appear to be unique in size and distribution of expression.

Expression of the trk proto-oncogene is restricted to the sensory cranial and spinal ganglia of neural crest origin in mouse development.

The data show that trk, a gene associated with malignancy in humans, is a specific marker for a set of neural crest-derived sensory neurons, and are consistent with the hypothesis that this proto-oncogene may have an important role in the development or phenotype of the neurons where it is expressed.

Cloning and characterization of a lambert‐eaton myasthenic syndrome antigen

To isolate and characterize the antigens, a human fetal brain expression library with a high‐titer serum from a patient with Lambert‐Eaton myasthenic syndrome resulted in the isolation of a complementary DNA clone encoding an antigen the authors call myastenic syndrome antigen B (MysB).

Hel-N1: an autoimmune RNA-binding protein with specificity for 3' uridylate-rich untranslated regions of growth factor mRNAs.

Investigation of the RNA binding specificity of Hel-N1, a human neuron-specific RNA-binding protein, which contains three RNA recognition motifs, revealed that it prefers to bind RNAs containing short stretches of uridylates similar to those found in the 3' untranslated regions of oncoprotein and cytokine mRNAs.

Characterization of the rat mas oncogene and its high-level expression in the hippocampus and cerebral cortex of rat brain.

The cloned rat homolog of the mas oncogene, determined its DNA sequence, and examined its expression in various rat tissues indicate that high levels of mas RNA transcripts are present in the hippocampus and cerebral cortex of the brain, but not in other neural regions or in other tissues.

A developmentally regulated, nervous system-specific gene in Xenopus encodes a putative RNA-binding protein.

In situ hybridization to tadpole brain shows that the nrp-1 gene is expressed in the ventricular zone where cell proliferation takes place and suggests that it may be involved in the tissue-specific control of RNA processing.

The cancer-associated retinopathy antigen is a recoverin-like protein.

Serum antibodies from CAR patients have been used to isolate the gene encoding the CAR antigen from a cDNA library of human retina, and nucleotide sequence analysis suggests that theCAR antigen shows approximately 90% homology to the published amino acid sequence of bovine recoverin.