On the pathogenesis of collagen VI muscular dystrophies--comment on article of Hicks et al.

SIR, in a recent article published in Brain (Hicks et al., 2009), while confirming our finding that myoblasts from patients affected by Ullrich congenital muscular dystrophy (UCMD) display a latentmitochondrial dysfunction that can be unmasked by the addition of the F1FO ATPase inhibitor oligomycin (Angelin et al., 2007), Hicks et al. conclude that ‘PTP dysregulation may be a particular characteristic of the state of these cells in culture and is not specific to the collagen VI defect’. .............................