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Related Experiment Videos

Peripheral nerve pathology in Niemann-Pick type C mouse

Y Higashi1, S Murayama, P G Pentchev

  • 1Department of Pathology, School of Medicine, University of North Carolina at Chapel Hill 27599-7525, USA.

Acta Neuropathologica
|January 1, 1995
PubMed
Summary

Niemann-Pick type C disease (NPC) in mice shows early sciatic nerve swelling and hypomyelination, indicating defective cholesterol metabolism impacts myelin maintenance.

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Area of Science:

  • Neuroscience
  • Cell Biology
  • Biochemistry

Background:

  • Niemann-Pick type C disease (NPC) is a lysosomal storage disorder.
  • Cholesterol metabolism is crucial for myelin sheath integrity.
  • Peripheral nerve alterations in NPC disease are not fully understood.

Purpose of the Study:

  • To investigate sciatic nerve morphology in NPC mouse models.
  • To identify early pathological changes related to cholesterol metabolism defects.

Main Methods:

  • Light and electron microscopy of sciatic nerves.
  • Teased-fiber preparations.
  • Morphometric analysis of myelinated fibers.

Main Results:

  • Focal paranodal swellings and myelin figures observed by postnatal day 20.

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  • Progressive hypomyelination of large fibers by day 70 in NPC mice.
  • No segmental demyelination or axonal degeneration detected.

    • Conclusions:

    • NPC mouse sciatic nerves exhibit early morphological changes suggesting impaired myelin maintenance.
    • Defective cholesterol utilization likely underlies these nerve alterations.
    • Paranodal changes may reflect early-stage myelin remodeling due to metabolic dysfunction.