Abnormality of vascular elastic fibers in the macular mouse and a patient with Menkes' disease: ultrastructural and immunohistochemical study.

Publisher: Springer-Verlag Tokyo Country of Publication: Japan NLM ID: 9430789 Publication Model: Print Cited Medium: Print ISSN: 0918-4287 (Print) Linking ISSN: 09184287 NLM ISO Abbreviation: Med Electron Microsc Subsets: MEDLINE

Publication: Tokyo : Springer-Verlag Tokyo
Original Publication: Moriguchi, Osaka, Japan : The Society, [1993-2004]

Blood Vessels/*abnormalities ; Blood Vessels/*ultrastructure ; Elastic Tissue/*abnormalities ; Elastic Tissue/*ultrastructure ; Menkes Kinky Hair Syndrome/*pathology; Animals ; Blood Vessels/metabolism ; Child, Preschool ; Disease Models, Animal ; Elastic Tissue/metabolism ; Elastin/metabolism ; Female ; Humans ; Immunohistochemistry ; Male ; Menkes Kinky Hair Syndrome/genetics ; Menkes Kinky Hair Syndrome/metabolism ; Mice ; Mice, Mutant Strains ; Microscopy, Electron

The macular mouse is a mutant mouse with the same gene abnormality as that of Menkes' disease, and it exhibits symptoms and abnormalities similar to those of Menkes' disease. In an electron microscopic study, we examined morphological changes in the internal elastic lamina (IEL) of the elastic arteries (EA) and the muscular arteries (MA) in a patient with Menkes' disease and in the macular mouse, an animal model of this disease. The IEL of the EA was significantly thinner in the macular mouse than that in controls, but the IEL of the MA in the macular mouse was significantly thicker than that of the controls. These contrary results for the thickness of the IEL in the MA and the EA in this animal model of Menkes' disease may reflect differences in the anatomical and pathophysiological properties of the two types of vessels.

9007-58-3 (Elastin)

Date Created: 20020712 Date Completed: 20021204 Latest Revision: 20041117

20221213

10.1007/s007950200003

12111404