title: Ultrastructural changes in the lung in Niemann-Pick type C mouse.
abstract: The biochemical and morphological aspects of BALB/c mice with many features of the Niemann-Pick disease type C in man (NP-C mouse) have been studied extensively. However, the pulmonary pathology has not been studied extensively and we describe here some unique ultrastructural features of the lung in the NP-C mouse. Ultrastructurally, macrophages in younger mice contained osmiophilic dense granules and annulolamellar structures, but larger multilamellar concentric structures increased in the macrophages of older mice. In contrast, endothelial cells and type I pneumocytes showed membrane-bound bodies with dense granules and vesicular or vesiculogranular structures as well as amorphous materials. Type II pneumocytes were unremarkable throughout. Our study suggests that endothelial cells and type I pneumocytes are the major site of metabolic derangement resulting in pronounced morphological changes with granular and round membranous structures in the lungs of NP-C mouse. Alveolar macrophages with multilamellar concentric structures may be a result of disturbed disposal of surfactant material from type II pneumocytes rather than that from storage material of type I pneumocyte.
authors.affiliation: Department of Pathology, Kawasaki Medical School, Kurashiki, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Manabe
authors.forename: T
authors.initials: T
authors.lastname: Yamane
authors.forename: Y
authors.initials: Y
authors.lastname: Higashi
authors.forename: P G
authors.initials: PG
authors.lastname: Pentchev
authors.forename: K
authors.initials: K
authors.lastname: Suzuki
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D008168:Lung / Q000473:pathology / Q000648:ultrastructure*; D051379:Mice; D008807:Mice, Inbred BALB C; D008817:Mice, Mutant Strains; D008854:Microscopy, Electron; D009542:Niemann-Pick Diseases / Q000473:pathology*