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LONP1: Ataxia+

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H&E stain

Muscle Fibers
  Morphology: Normal
  Mild freeze artefact

VvG stain

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Muscle Fibers
  Internal Architecture: Normal

NADH stain

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Cytochrome oxidase (COX) stain

Muscle Fibers: Mitochondrial Pathology
  COX negative muscle fibers: Scattered

Cytochrome oxidase (COX) stain

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Succinate Dehydrogenase (SDH) stain

Muscle Fibers: Mitochondrial Pathology
  SDH negative muscle fibers: Scattered

Succinate Dehydrogenase (SDH) stain

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SDH + COX stain

Muscle Fibers: Mitochondrial Pathology
  SDH & COX: Negative in same muscle fibers

SDH + COX stain

SDH + COX stain

Muscle Fibers: Mitochondrial Pathology
  SDH & COX: Negative in same muscle fibers

SDH + COX stain

SDH + COX stain

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Sudan stain

Muscle Fibers: Mitochondrial Pathology
  Small lipid droplets in some muscle fibers

Sudan stain

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ATPase pH 9.4 stain

Muscle Fibers
  No fiber type grouping

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ATPase pH 4.3 stain

Muscle Fibers
  Few intermediate-stained type 2C fibers
Endomysial capillaries: Mild staining

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C5b-9 stain

Muscle Fibers
  No necrosis or Inflammation

Acid Phosphatase stain

Endomysial capillaries: Mild staining

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7/1/2024