Figure 3.

α-DG Glycosylation Is Reduced in Muscle Biopsies of Affected Individuals with GMPPB Mutations

An examination of skeletal-muscle cryosections from the control (A, E, I, and M), P1 (B, F, J, and N), P2 (C, G, K, and O), and P7 (D, H, L, and P) revealed that glycosylated α-DG immunolabeling (assessed with IIH6) was moderately reduced in individuals (P1, P2, and P7) with GMPPB mutations and that β-DG and core α-DG were well preserved. Stains are as follows: hematoxylin and eosin (A–D), β-DG (E–H), α-DG IIH6 (I–L), and α-DG core (M–P).