Summary of α-DG glycosylation as assessed by flow cytometry in 21 patient fibroblasts, three healthy controls, and seven pathological controls.

The specific gene, mutation, phenotype, MFI of the IIH6 positive cells ± SEM, percentage of cells positive for the IIH6 epitope ± SEM, iMFI value ± SEM, N value, P value, and muscle α-DG IIH6 description is listed for each fibroblast cell line analysed. P values are the result of an unpaired t-test comparing the MFI, percentage of cells positive for the IIH6 epitope, or iMFI values of each fibroblast cell line to the respective values of the three healthy controls (C1, C2, C3). Muscle α-DG IIH6 description is the summary from the patient report of how the skeletal muscle α-DG glycosylation appeared by immunohistochemistry. iMFI is defined as iMFI = (MFI)(P), where P is the percentage of cells positive for the IIH6 epitope [43][44]. Abbreviations are as follows: DMD, Duchenne muscular dystrophy; BMD, Becker muscular dystrophy; LGMD, Limb-girdle muscular dystrophy; MEB, Muscle-eye-brain disease; MDC1C, Muscular dystrophy type 1 C; CMD, Congenital muscular dystrophy; FCMD, Fukuyama congenital muscular dystrophy; LGMD-CRB, limb girdle muscular dystrophy with cerebellar involvement [11]; MR, mental retardation; NS, non-significant. * = Sections evaluated with the VIA4-1 antibody.