Tubular aggregates

TUBULAR AGGREGATES

Tubular aggregates

Differential Diagnosis

Pathology

Location: Cytoplasm
Origin: Sarcoplasmic reticulum
Histochemistry: Similar to Cylindrical spirals
- H & E
  - Irregular, internal regions, slits or lakes
  - May be clear, or contain pink material
- Gomori trichrome
  - Contain red material
  - Location: Central or sub-sarcolemmal
- VvG stain: Gray
- NADH: Stain strongly
- ATPase: Often in type II > I muscle fibers
- Sudan: Tubular aggregates contain lipid
- AMPDA: Stain strongly
- No reaction
  - Mitochondrial stains: COX & SDH
  - Acid & Alkaline phosphatase
Protein contents ¹
- DHPR: T-tubules
- RyR1: Terminal cisternae; Sarcoplasmic reticulum
- SERCA1: Longitudial sarcoplasmic reticulum
- Other: In some patients
  - SERCA2: Longitudial sarcoplasmic reticuilum
  - SAR1 : Endoplasmic exit sites
  - Dysferlin
  - CASQ1
Ultrastructure (below): 3 types

H & E stain

Muscle fibers
Pink cytoplasmic areas: May contain nuclei
Irregular-shaped clear regions

H & E stain

Congo red stain

Gomori trichrome stain Tubular aggregates Stain red on Gomori trichrome

Gomori trichrome stain

Gomori trichrome stain

Tubular aggregates: May have elongated or rounded shapes

Gomori trichrome stain

Gomori trichrome stain

VvG stain

Tubular aggregates
Stain gray-black on VvG

NADH-TR reductase (NADH) stain

Tubular aggregates:
Stain dark on NADH

NADH stain

AMPDA stain

Tubular aggregates: Contain AMPDA

AMPDA stain

ATPase pH 9.4 stain

Tubular aggregates (Pale regions): Are often more common in type 2 muscle fibers

ATPase pH 4.3 stain

Sudan black stain

Tubular aggregates: Stain for lipid on Sudan black

Sudan black stain

Tubular aggregates
May have mild or patchy staining for Acid Phosphatase

Acid Phosphatase stain

Tubular aggregates: Ultrastructure; 3 types

I: 50 to 70 nm diameter tubules with 40 nm inner central tubule
II: 70 to 400 nm tubules with moderately dense material in center
III: 130 to 400 nm diameter tubules containing several 25 to 40 nm tubules

From: Tahseen Mozaffar

Tubular Aggregates: Ultrastructure

From: Oliver Ni

Tubular Aggregates: Mouse Muscle

From: Andrew Findlay

From: Andrew Findlay

Return to Myopathies with tubular aggregates

References
1. J Neuropathol Exp Neurol 2016;75:1171-1178

10/9/2024