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. 2013 Jan 3;136(1):269–281. doi: 10.1093/brain/aws312

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© The Author (2013). Published by Oxford University Press on behalf of the Guarantors of Brain.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Immunohistochemistry of muscle biopsies from cases with ISPD-related muscular dystrophy. Unfixed frozen sections of quadriceps muscle biopsies from a control and three ISPD patients (Cases 4, 6, and 7) stained with haematoxylin and eosin (H & E) and immunolabelled with antibodies against laminin-α2 (LAMA2), β-dystroglycan (BDG), the glycosylated epitope of α-dystroglycan (IIH6 ADG) and the core protein of α-dystroglycan (GT20 ADG). Laminin-α2 immunostaining was reduced in all ISPD cases. Although expression of β-dystroglycan was similar to the control (see text), glycosylated α-dystroglycan immunolabelling was absent in Cases 4 and 7 and severely reduced in Case 6. Core α-DG (GT20ADG) was well preserved in all the cases.