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LGMD 2T (GMPPB mutations)

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H & E stain

Myopathy: Mild active & chronic changes   Necrosis & Regeneration: Scattered   Fiber size: Varied     Small fibers: Rounded or Polygonal   Endomysial connective tissue: Normal or Slightly increased   Internal nuclei: Some fibers

H & E stain

Myopathy   Necrosis: Scattered   Fiber size: Varied   Internal nuclei: Some fibers Gomori trichrome stain

VvG stain

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Myopathy   Small muscle fibers: Both types; Scattered   2C muscle fibers: Few ATPase pH 9.4 stain

ATPase pH 4.3 stain

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Small muscle fiber: : Regenerating, Coarse internal architecture NADH stain

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Necrotic muscle fiber: Replaced by esterase+ cells Esterase stain

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Aggregated Caveolin-3: Sub-sarcolemmal Caveolin-3 stain

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α-Dystroglycan (Green): Reduced or Absent on the surface of some muscle fibers α-Dystroglycan - Green; Caveolin-3 - Red

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11/1/2016