Autoantibodies against the chromosomal passenger protein INCENP found in a patient with Graham Little-Piccardi-Lassueur syndrome

Beatriz Rodríguez-Bayona^*¹ , Sandrine Ruchaud^*³ , Carmen Rodríguez¹ , Mario Linares² , Antonio Astola⁴ , Manuela Ortiz⁴ , William C Earnshaw³ and Manuel M Valdivia⁴

¹Servicio de Inmunología, Hospital Puerta del Mar, 11109 Cádiz, Spain

²Dermatología, Instituto Social de la Marina, Delegación Provincial de Cádiz, Cádiz, Spain

³Wellcome Trust Centre for Cell Biology, School of Biological Sciences, University of Edinburgh, Edinburgh EH9 3JR, UK

⁴Departamento de Bioquímica y Biología Molecular, Facultad de Ciencias, 11510 Puerto Real, Cádiz, Spain

author email corresponding author email* Contributed equally

Journal of Autoimmune Diseases 2007, 4:1doi:10.1186/1740-2557-4-1


Published:	12 January 2007

Abstract

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.