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Ultrastructural changes in the lung in Niemann-Pick type C mouse
Virchows Archiv : an International Journal of Pathology
|January 1, 1995
Summary
Niemann-Pick disease type C (NP-C) mouse lungs show unique ultrastructural changes in endothelial cells and type I pneumocytes. These cells, not type II pneumocytes, appear to be the primary site of metabolic derangement in NP-C mouse lungs.
Area of Science:
- Cell Biology
- Pathology
- Genetics
Background:
- Niemann-Pick disease type C (NP-C) is a rare genetic disorder affecting cellular lipid metabolism.
- NP-C mouse models are crucial for studying disease mechanisms, but pulmonary pathology remains under-investigated.
Purpose of the Study:
- To investigate the ultrastructural pulmonary pathology in Niemann-Pick disease type C (NP-C) mouse models.
- To identify the specific cell types and morphological changes involved in NP-C lung pathology.
Main Methods:
- Transmission electron microscopy was used to examine lung tissue from NP-C mice.
- Ultrastructural features of macrophages, endothelial cells, type I pneumocytes, and type II pneumocytes were analyzed.
Main Results:
- Macrophages in younger NP-C mice exhibited dense granules and annulolamellar structures; older mice showed increased multilamellar concentric structures.
- Endothelial cells and type I pneumocytes displayed membrane-bound bodies with dense granules, vesicular structures, and amorphous materials.
- Type II pneumocytes appeared morphologically normal throughout the study.
Conclusions:
- Endothelial cells and type I pneumocytes are the primary sites of metabolic derangement in the NP-C mouse lung, leading to significant morphological alterations.
- Multilamellar concentric structures in alveolar macrophages may result from disturbed surfactant disposal by type II pneumocytes, rather than storage within type I pneumocytes.