MITOCHONDRIAL DISEASE: ULTRASTRUCTURE
Ultrastructural features of mitochondria in mitochondrial disease
- Numbers: Increased in subsarcolemmal & intermyofibrillar spaces
- Shape: Abnormal
- Size: Enlarged or Elongated (Normal < 3-4 sarcomeres in length)
- Cristae: Irregular; Deficient; Stacking; Whorling
- Granules: Electron dense; Spherical; Multiple
- Paracrystalline structures (Inclusions)
- Have regular geometrical periodicity
- Most specific morphologic abnormality
- Commonly occur in fibers with mitochondrial proliferation
- "Parking Lot"
- May occur in muscle, liver or brain
- Also see: Ultrastructure
 Muscle mitochondria with normal structure. |
 Mitochondrial myopathy: Subsarcolemmal accumulation of mitochondria |
 Mitochondrial myopathy: Enlarged whorled mitochondrion |
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| Mitochondrial disease: Abnormal muscle mitochondria from a child | |
Mitochondrial inclusions
- Major protein in inclusions
- Creatine kinase, mitochondrial
- Similar inclusions may occur in
- Injured muscle cells
- Dermatomyositis
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| Inclusions: Present in many mitochondria |
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| Longitudinal sections of crystalloid inclusions |
 Cross sections of crystalloid inclusion |
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| Cross sections of crystalloid inclusions | |
 Cross section of dark crystalloid inclusion |
  Dense parallel cristae |
 
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PEO: Crystalloid Inclusions (Parking lot bodies)
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7/6/2023