Neuromuscular

Phosphoglycerate Mutase deficiency (GSD 10)


H&E stain
Muscle fibers
  Size: Normal
  Contain: Tubular aggregates


H&E stain
Tubular aggregates
  Pink-stained regions on H&E stain
    Internal or sub-sarcolemmal

H&E stain

Tubular aggregates: Red stained

Gomori trichrome stain


VvG stain
Tubular aggregates
  Dark stained
  Irregular shapes

VvG stain

VvG stain
Tubular aggregates
  Dark stained
  Irregular shapes

VvG stain


NADH stain
Tubular aggregates
  NADH stained: Composed of SR-like tubules
  Irregular shapes
  May be small or large
  May have several in one muscle fiber

NADH stain

NADH stain


AMPDA stain
Tubular aggregates
  Contain AMPDA

AMPDA stain

Phosphoglycerate Mutase deficiency
  Glycogen: May be increased in muscle fibers
  Tubular aggregates: Can stain for PAS

PAS stain


Sudan black stain


ATPase pH 4.3 stain

Glycogen: Membrane bound & Free in cytoplasm

Return to PGAM
Return to Tubular aggregates

7/2/2016