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X-linked Myopathy with Excessive Autophagy

H & E stain

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H & E stain   From J Mandell Myopathic changes Muscle fiber size: Varied Endomysial connective tissue: Increased Internal nuclei

H & E stain   From J Mandell Vacuoles: Small & Clear

H & E stain

Myopathic changes

• Muscle fibers
  • Size: Varied
  • Split (Partially fused)
  • Internal nuclei
  • Vacuoles: Irregular shapes
• Endomysial connective tissue: Increased

H & E stain

H & E stain

H & E stain

VvG stain

NADH stain

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Esterase stain   From J Mandell Vacuoles in Muscle fiber cytoplasm   Many stain for Esterase

Esterase stain

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Dystrophin stain Vacuoles: Stain for Dystrophin & other membrane proteins

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δ-Sarcoglycan stain Vacuoles: Stain for membrane proteins, including δ-Sarcoglycan

δ-Sarcoglycan stain

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Caveolin-3 stain Vacuoles: Stain for membrane proteins, including Caveolin-3

Caveolin-3 stain

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LAMP2 stain

LAMP2 stain
  XMEA (Above)
  Control (Below)

LAMP2 stain

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ATPase pH 4.3 stain

ATPase pH 4.3 stain

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C5b-9 complement stain

XMEA: C_5b-9 complement stains the surface of, and internal membranes in, some muscle fibers

C5b-9 complement stain

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MHC Class I stain

XMEA: MHC Class I is upregulated by muscle fibers

MHC Class I stain

MHC Class I stain

MHC Class I stain

XMEA: MHC Class I is upregulated in the cytoploasm of some muscle fibers

MHC Class I stain

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Autophagic Vacuole

From: Steve Moore & Cameron Crockett

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Autophagic Vacuole
  Extrusion
  Basal lamina: Duplication

From: Steve Moore

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Return to: X-linked myopathy with excessive autophagy
Also see: Autophagic vacuoles with sarcolemmal features
Return to: Pathology index
Return to: Neuromuscular Home Page

12/8/2021