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


CONCLUSIONS
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.
Rodríguez-Bayona
Beatriz
B
Servicio de Inmunología, Hospital Puerta del Mar, 11109 Cádiz, Spain.
Ruchaud
Sandrine
S
Wellcome Trust Centre for Cell Biology, School of Biological Sciences, University of Edinburgh, Edinburgh EH9 3JR, UK.
Rodríguez
Carmen
C
Servicio de Inmunología, Hospital Puerta del Mar, 11109 Cádiz, Spain.
Linares
Mario
M
Dermatología, Instituto Social de la Marina, Delegación Provincial de Cádiz, Cádiz, Spain.
Astola
Antonio
A
Departamento de Bioquímica y Biología Molecular, Facultad de Ciencias, 11510 Puerto Real, Cádiz, Spain.
Ortiz
Manuela
M
Departamento de Bioquímica y Biología Molecular, Facultad de Ciencias, 11510 Puerto Real, Cádiz, Spain.
Earnshaw
William C
WC
Wellcome Trust Centre for Cell Biology, School of Biological Sciences, University of Edinburgh, Edinburgh EH9 3JR, UK.
Valdivia
Manuel M
MM
Departamento de Bioquímica y Biología Molecular, Facultad de Ciencias, 11510 Puerto Real, Cádiz, Spain.