Brain lipid composition in grey-lethal mutant mouse characterized by severe malignant osteopetrosis |
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Authors: | Alessandro Prinetti Federica Rocchetta Elvira Costantino Annalisa Frattini Elena Caldana Francesca Rucci Arianna Bettiga Pietro L Poliani Vanna Chigorno Sandro Sonnino |
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Institution: | (1) Center of Excellence on Neurodegenerative Diseases, Department of Medical Chemistry, Biochemistry and Biotechnology, University of Milan, Milano, Italy;(2) Institute of Biomedical Technologies, CNR, Milano, Italy;(3) Institute Clinico Humanitas, Rozzano, Italy;(4) Division of Immunology, Children’s Hospital, Harvard Medical School, Boston, MA, USA;(5) Institute of Molecular Medicine, University of Brescia, Brescia, Italy;(6) Via Fratelli Cervi 93, 20090 Segrate, Milan, Italy |
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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. |
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Keywords: | Sphingolipids Gangliosides Myelin Demyelination Ostepetrosis |
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