Brain lipid composition in grey-lethal mutant mouse characterized by severe malignant osteopetrosis. (Articolo in rivista)

Type
Label
  • Brain lipid composition in grey-lethal mutant mouse characterized by severe malignant osteopetrosis. (Articolo in rivista) (literal)
Anno
  • 2008-01-01T00:00:00+01:00 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
  • 10.1007/s10719-008-9179-8 (literal)
Alternative label
  • Prinetti A; Rocchetta F; Costantino E; Frattini A; Caldana E; Rucci F; Bettiga A; Poliani PL; Chigorno V; Sonnino S. (2008)
    Brain lipid composition in grey-lethal mutant mouse characterized by severe malignant osteopetrosis.
    in Glycoconjugate journal; Springer Science, New York (Stati Uniti d'America)
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Prinetti A; Rocchetta F; Costantino E; Frattini A; Caldana E; Rucci F; Bettiga A; Poliani PL; Chigorno V; Sonnino S. (literal)
Pagina inizio
  • 623 (literal)
Pagina fine
  • 633 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#url
  • http://www.springerlink.com/ (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 26 (literal)
Rivista
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#pagineTotali
  • 10 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroFascicolo
  • 6 (literal)
Note
  • PubMed (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • Center of Excellence on Neurodegenerative Diseases, Department of Medical Chemistry, Biochemistry and Biotechnology, University of Milan, Milano, Italy Institute of Molecular Medicine, University of Brescia, Brescia, Italy Institute of Biomedical Technologies, CNR, Milano, Italy (literal)
Titolo
  • Brain lipid composition in grey-lethal mutant mouse characterized by severe malignant osteopetrosis. (literal)
Abstract
  • The grey-lethal mouse (gl/gl) mutant most closely resembles the severe human malignant autosomal recessive OSTM1-dependent form of osteopetrosis that it has been described to be associated with neurological abnormalities. For this reason, we have analyzed the brain lipid composition (gangliosides, neutral glycosphingolipids, phospholipids and cholesterol), from gl/gl mice at different ages of development and compared with wild type mice. Both cholesterol and glycerophospholipid content and pattern in the gl/gl and control mice were very similar. In contrast, significant differences were observed in the content of several sphingolipids. Higher amount of the monosialogangliosides GM2 and GM3, and lower content of sphingomyelin, sulfatide and galactosylceramide were observed in the gl/gl brain with respect to controls. The low content of sphingomyelin, sulfatide and galactosylceramide is consistent with the immunohistochemical results showing that in the grey-lethal brain significant depletion and disorganization of the myelinated fibres is present, thus supporting the hypothesis that loss of function of the OSTM1 causes neuronal impairment and myelin deficit. (literal)
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