Analysis of growth factor expression in affected and unaffected muscles of oculo-pharyngeal muscular dystrophy (OPMD) patients: a pilot study.
Oculo-pharyngeal muscular dystrophy (OPMD) is characterised by progressive eyelid drooping (ptosis) and difficulties with swallowing (dysphagia). In order to determine the role of growth factors, cytokines and chemokines in the physiopathology of muscle disease we have compared the level of expression of 174 factors in both the affected (cricopharyngeal) and non-affected (sternocleidomastoid) muscles of 8 OPMD patients by means of antibody arrays. Despite an important inter-individual variability the expression of sixty factors was found to be persistently different between affected and non-affected muscles. Many of the differentially expressed factors in our study are known to be involved in the formation of fibrosis in both the liver and the lung, indicating the possibility that treatments such as those used in hepatic fibrosis may have a beneficial effect in OPMD patients.
Bouazza
Belaid
B
UPMC, Université Pierre et Marie Curie Paris VI, Institut de Myologie, 105, Bd de l'Hôpital, 75634 Paris, France.
Kratassiouk
Gueorgui
G
Gjata
Bernard
B
Perie
Sophie
S
Lacau St Guily
Jean
J
Butler-Browne
Gillian S
GS
Svinartchouk
Fedor
F
D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000032:analysis / Q000378:metabolism; D018925:Chemokines / Q000032:analysis / Q000378:metabolism; D019295:Computational Biology / Q000379:methods; D016207:Cytokines / Q000032:analysis / Q000378:metabolism*; D005260:Female; D005355:Fibrosis / Q000276:immunology / Q000378:metabolism* / Q000503:physiopathology; D006801:Humans; D007150:Immunohistochemistry; D036341:Intercellular Signaling Peptides and Proteins / Q000032:analysis / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000276:immunology / Q000378:metabolism* / Q000503:physiopathology; D039141:Muscular Dystrophy, Oculopharyngeal / Q000276:immunology / Q000378:metabolism* / Q000503:physiopathology; D010865:Pilot Projects; D040081:Protein Array Analysis / Q000379:methods; D040901:Proteomics / Q000379:methods; D012984:Software
D015415:Biomarkers; D018925:Chemokines; D016207:Cytokines; D036341:Intercellular Signaling Peptides and Proteins