Neuromuscular

NEMALINE RODS

General description
Ultrastructure
Infant
Child
Adult

Nemaline Rod Pathology

  • Light microscopy
    • Rods best visualized with Gomori trichrome stain
    • Dark blue structures
    • Most in muscle fiber cytoplasm
  • Contain
    • Z-line like material
    • α-actinin, actin & tropomyosin ± desmin at the periphery
  • Located in sarcoplasm: Often in regions with disrupted sarcomere structure
  • Occur in
  • Similar appearance & staining to: Cytoplasmic bodies
  • Also see: Rods or Cytoplasmic bodies in target fibers

Nemaline rod myopathy: Infantile

 
Gomori trichrome

Nemaline rods in gastrocnemius muscle from 4 month old child



No nemaline rods in quadriceps muscle from same child biopsied at 2 months of age

Nemaline rod myopathy, Infantile: Toluidine blue stains

Nemaline rod myopathy, Infantile: Other stains


H& E stain

Variable muscle fiber size
Refractile rods can be seen
ATPase stain, pH 9.4

Type I muscle fiber predominance

Phalloidin staining for actin
Focal (bright green), large and small aggregates of actin are present in some muscle fibers

Phalloidin staining for actin


Nemaline rod myopathy, Childhood


GT stain

Rods in smaller muscle fibers
ATPase stain, pH 9.4

Type I muscle fibers: Smaller than type II


Rods in adult nemaline myopathy

 
Gomori trichrome

Nemaline Rod Ultrastructure
Infantile-onset Rod myopathy

From: R Schmidt
 

From E Hardeman
Electron microscopy of rods from Z-lines in Met9Arg αTMslow mouse

Return to Rod myopathies

5/30/2012