Ovine ceroid-lipofuscinosis II: Pathologic changes interpreted in light of biochemical observations.
Am J Med Genet Suppl
; 5: 159-70, 1988.
Article
em En
| MEDLINE
| ID: mdl-3146314
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 10A 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.
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Base de dados:
MEDLINE
Assunto principal:
Pâncreas
/
Doenças dos Ovinos
/
Fígado
/
Lipofuscinoses Ceroides Neuronais
Limite:
Animals
Idioma:
En
Revista:
Am J Med Genet Suppl
Assunto da revista:
GENETICA MEDICA
Ano de publicação:
1988
Tipo de documento:
Article
País de afiliação:
Nova Zelândia