Reducing Body & FHL1 Myopathy
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FHL1: Emery Dreifus 6 |
 H & E stain From: CG Bonnemann MD |
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Reducing bodies: Irregularly shaped, cytoplasmic inclusions Muscle fiber sizes: Varied |
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 H & E stain From: C Cai FHL1 myopathy (Heterozygous, de novo, c.369C>G (p.H123Q) mutation) Fiber size variation: Marked Sarcoplasmic inclusions (Black arrow) Intranuclear inclusions (Yellow arrows) |
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 Gomori trichrome stain From: CG Bonnemann MD |
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Reducing bodies: Darkly stained on Gomori trichrome |
 Gomori trichrome stain From: C Cai FHL1 myopathy Aggregates: Cytoplasmic bodies & Reducing Bodies (Arrows) |
 Gomori trichrome stain From: C Cai FHL1 myopathy Intranuclear inclusions |
 Menadione-NBT stain From: CG Bonnemann MD |
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Reducing bodies: Stain with Menadione-NBT |
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 Congo red stain From: C Cai FHL1 myopathy Inclusions: Cytoplasmic (Black arrow) & Nuclear (Yellow arrow) |
 From: CG Bonnemann MD |
 From: CG Bonnemann MD |
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Reducing bodies: Ultrastructure Structure: Granular tubulo-filamentous material (17 nm in diameter) Location: At periphery of sarcoplasm & around nucleus |
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 From: C Cai FHL1 myopathy Inclusions: Cytoplasmic body (Black arrow) & Granule, electon dense (Yellow arrow) |
 From: C Cai FHL1 myopathy Inclusion: Granule, electon dense, composed of 20-30 nm spherical particles |
 From: C Cai FHL1 myopathy Inclusions, Intranuclear (Yellow arrow): Electron dense, granular aggregates |
 From: C Cai FHL1 myopathy Inclusion, Intranuclear: Thin filaments & Electron dense, granular aggregates |
 From: C Cai FHL1 myopathy Inclusion, Intranuclear: Filamentous material |
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4/3/2020