Ovine ceroid-lipofuscinosis II: Pathologic changes interpreted in light of biochemical observations
- 1 January 1988
- journal article
- animal model
- Published by Wiley in American Journal of Medical Genetics
- Vol. 31 (S5) , 159-170
- https://doi.org/10.1002/ajmg.1320310619
Abstract
Ceroid-lipofuscinosis is studied in a breed of sheep and the histochemical and ultrastructural changes are interpreted in light of the known chemical composition of cytosomes. It is concluded that the ovine disease is a lysosomal proteinosis. It is postulated that the multilamellar arrays reflect a complex three-dimensional structure of alternating lipid and protein with the proteinaceous component being specific polypeptides. This is supported by powder X-ray diffractions at l0Å implying a repeating proteinaceous unit in the structures. The coherent size of diffracting units was remarkably similar to the periodicity of lamellae observed in electron micrographs of lipopigment cytosomes. The fluorescent and staining characteristics of lipopigment can be explained by the above conclusion and postulate.Keywords
This publication has 24 references indexed in Scilit:
- Single bilayer liposomesPublished by Elsevier ,2003
- CEROID‐LIPOFUSCINOSIS (BATTEN'S DISEASE): PATHOGENESIS OF BLINDNESS IN THE OVINE MODELNeuropathology and Applied Neurobiology, 1985
- Phospholipid fatty acids in brains of normal sheep and sheep with ceroid-lipofuscinosisBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1985
- Inhibitors of Lysosomal Enzymes: Accumulation of Lipofuscin-Like Dense Bodies in the BrainScience, 1984
- OVINE CEROID‐LIPOFUSCINOSIS: A MODEL OF BATTEN'S DISEASENeuropathology and Applied Neurobiology, 1980
- Enzyme Topology of Intracellular MembranesAnnual Review of Biochemistry, 1977
- The Fluid Mosaic Model of the Structure of Cell MembranesScience, 1972
- Mechanism of sonication of aqueous egg yolk lecithin dispersions and nature of the resultant particlesBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1972
- Low-angle X-ray diffraction study of the polymorphic forms of synthetic α:β and α:α′-kephalins and α:β-lecithinsTransactions of the Faraday Society, 1955
- A contribution to the theory of permeability of thin filmsJournal of Cellular and Comparative Physiology, 1935