Hu antigen specificities of ANNA-I autoantibodies in paraneoplastic neurological disease

P. H. King, D. Redden, J. S. Palmgren, L. B. Nabors, V. A. Lennon

Research output: Contribution to journalArticlepeer-review

54 Scopus citations


Despite a broad clinical spectrum, paraneoplastic enecephalomyelitis/sensory neuronopathy (PEM/SSN) is characterized by the presence of a common autoantibody, referred to as anti-Hu or type I anti- neuronal nuclear antibody (ANNA-1). The target of these antibodies is a family of four Hu antigens: three (Hel-N1, HuC, HuD) are neural-specific, while the fourth (HuR) is ubiquitous. Here, we have analysed by enzyme-linked immunosorbent assay (ELISA) the immunoreactivity of all four Hu antigens in serum from 75 patients with ANNA-1 autoantibodies and looked for clinical correlations. IgG in all the patients' sera bound to each of the four antigens, and the titers correlated with those of the ANNA-I immunofluorescence assay. Median titers for the neural-specific antigens (range: 56,892-90,051) were significantly higher than for HuR (36,799). Patients with gastrointestinal dysmotility or subacute sensory neuronopathy had the highest median titers to all four antigens, while patients with sensorineural deafness had the lowest titers. The results indicate a heterogeneous immune response to individual Hu antigens in patients with PEM/SSN, and that the titers to these antigens as a group, rather than individually, correlate with clinical profile. Furthermore, these results suggest that ELISA analysis of a single neural-specific Hu antigen is sufficient for serological screening in PEM/SSN.

Original languageEnglish (US)
Pages (from-to)435-443
Number of pages9
JournalJournal of Autoimmunity
Issue number4
StatePublished - Dec 1999


  • Hel-N1
  • HuC
  • HuD
  • HuR
  • RNA-binding proteins

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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