title: Serum matrix metalloproteinase-9 (MMP-9) as a biomarker for monitoring disease progression in Duchenne muscular dystrophy (DMD).
abstract: To identify serum biomarkers that allow monitoring of disease progression and treatment effects in Duchenne muscular dystrophy (DMD) patients, levels of matrix metalloproteinase-9 (MMP-9), tissue inhibitors of metalloproteinase-1 (TIMP-1) and osteopontin (OPN) were determined in 63 DMD patients on corticosteroid therapy. These proteins were selected for their role in the pathogenesis of muscular dystrophy. Levels of MMP-9 and TIMP-1 were significantly higher in sera of DMD patients compared to healthy controls, whereas the OPN levels showed no significant difference. MMP-9 levels were also observed to be significantly higher in older, nonambulant patients, compared to ambulant patients. Longitudinal data from a smaller cohort of DMD patients followed up for over 4years showed that MMP-9, but not TIMP-1 increased significantly with age. Hence, MMP-9 is a potential DMD biomarker for disease progression. Future studies have to confirm whether serum MMP-9 levels can be used to monitor therapeutic response.
authors: Department of Human Genetics, Leiden University Medical Centre (LUMC), RC, Leiden, The Netherlands.
authors: V D
authors: VD
authors: Nadarajah
authors: M
authors: M
authors: van Putten
authors: A
authors: A
authors: Chaouch
authors: P
authors: P
authors: Garrood
authors: V
authors: V
authors: Straub
authors: H
authors: H
authors: Lochmüller
authors: H B
authors: HB
authors: Ginjaar
authors: A M
authors: AM
authors: Aartsma-Rus
authors: G J B
authors: GJ
authors: van Ommen
authors: J T
authors: JT
authors: den Dunnen
authors: P A C
authors: PA
authors: 't Hoen
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000818:Animals; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004195:Disease Models, Animal; D018450:Disease Progression; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D020780:Matrix Metalloproteinase 9 / Q000097:blood*; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D020388:Muscular Dystrophy, Duchenne / Q000097:blood* / Q000188:drug therapy / Q000503:physiopathology*; D053495:Osteopontin / Q000097:blood; D019715:Tissue Inhibitor of Metalloproteinase-1 / Q000097:blood; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D015415:Biomarkers; D019715:Tissue Inhibitor of Metalloproteinase-1; D053495:Osteopontin; D020780:Matrix Metalloproteinase 9