The photoreceptor cell-specific nuclear receptor is an autoantigen of paraneoplastic retinopathy Journal Article
| Authors: | Eichen, J. G.; Dalmau, J.; Demopoulos, A.; Wade, D.; Posner, J. B.; Rosenfeld, M. R. |
| Article Title: | The photoreceptor cell-specific nuclear receptor is an autoantigen of paraneoplastic retinopathy |
| Abstract: | Objectives: To report a novel antibody associated with paraneoplastic retinopathy and to characterize the retinal autoantigen. Methods: Immunohistochemistry of rat and human tissues was used to identify antiretinal antibodies. Serologic screening of a bovine retinal cDNA expression library was performed to clone the target antigen. Results: A 72-year-old woman presented with a 6-month history of progressive visual loss, bilateral central scotomas, light flashes, and night blindness. Visual acuity was 20/40 OD and 20/30 OS. There was generalized loss of retinal pigment and narrow arterioles; discs were normal in appearance. The electroretinogram showed no response. Chest computed tomograph scan demonstrated a right lung mass; biopsy revealed poorly differentiated carcinoma. The patients' serum contained antibodies that immunolabeled nuclei of cells of the outer-and to a lesser extent, the inner-nuclear layer of the adult rat retina. No reactivity was identified with nonretinal adult human or rat tissues. Reactivity was seen in the developing rat embryo. Serologic screening of a bovine retinal library resulted in the isolation of three overlapping clones, encoding a protein highly homologous to the human photoreceptor cell-specific nuclear receptor gene product. Conclusions: The target antigen of an antibody associated with paraneoplastic retinopathy is the photoreceptor cellspecific nuclear receptor, a member of a conserved family of nuclear receptors involved in photoreceptor cell development or maintenance. |
| Keywords: | immunohistochemistry; human tissue; aged; unclassified drug; clinical feature; case report; nonhuman; animals; animal tissue; computer assisted tomography; lung neoplasms; diagnostic imaging; immunoreactivity; transcription factors; molecular cloning; immunoenzyme techniques; symptom; blotting, western; nucleotide sequence; rat; autoantigen; autoantigens; retinopathy; lung carcinoma; cattle; rats; antibodies; sequence homology; paraneoplastic syndrome; cell nucleus receptor; lung biopsy; autoantibodies; visual impairment; complementary dna; retinal diseases; receptors, cytoplasmic and nuclear; electroretinogram; visual acuity; scotoma; paraneoplastic syndromes; night blindness; photoreceptors, vertebrate; paraneoplastic; cancer; humans; human; female; priority journal; article; central scotoma; vision disorders; photoreceptor cell-specific nuclear receptor; photoreceptor cell specific nuclear receptor; photoreceptor cell; retina pigment degeneration |
| Journal Title: | Journal of Neuro-Ophthalmology |
| Volume: | 21 |
| Issue: | 3 |
| ISSN: | 1070-8022 |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2001-09-01 |
| Start Page: | 168 |
| End Page: | 172 |
| Language: | English |
| PUBMED: | 11725181 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Export Date: 21 May 2015 -- Source: Scopus |
