Autoantibodies against the chromosomal passenger protein INCENP found in a patient with Graham Little-Piccardi-Lassueur syndrome
| Autor: | Antonio Astola, Carmen Rodríguez, Sandrine Ruchaud, Beatriz Rodríguez-Bayona, Mario Alfredo Linares, William C. Earnshaw, Manuel M. Valdivia, Manuela Ortiz |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2007 |
| Předmět: |
0303 health sciences
Chromosomal passenger complex Pathology medicine.medical_specialty Follicular papules INCENP business.industry Research Autoantibody Scarring alopecia medicine.disease 3. Good health 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine medicine Immunology and Allergy Androgen insensitivity syndrome Graham Little Piccardi Lassueur syndrome business 030304 developmental biology |
| Zdroj: | Journal of Autoimmune Diseases |
| ISSN: | 1740-2557 |
| Popis: | Background Graham Little – Piccardi – Lassueur (GLPL) syndrome is a rare dermatosis characterized by scarring alopecia, loss of pubic and axillary hair, and progressive development of variously located follicular papules. We report a first case ever of an autoimmune response in a patient suffering from GLPL syndrome. Methods Immunofluorescence and immunoblot analysis were used in a variety of cell cultures including human, monkey, hamster, mouse and bovine cells to analyze the presence of autoantibodies in a GLPL patient. Results The autoimmune serum showed a pattern of centromere and spindle microtubule staining resembling that of the chromosomal passenger protein complex. By using a complex of proteins expressed in baculovirus, immunoblot analysis demonstrated that the INCENP protein is a major autoantigen in this patient with GLPL syndrome. Conclusion An autoimmune response in GLPL syndrome is reported against the INCENP centromere protein. The occasional development of autoimmunity in GLPL patients could serve as a test in continuing efforts to detect this disease and for a more directed therapy based on the autoantigen response. |
| Databáze: | OpenAIRE |
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