Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice
<p>Abstract</p> <p>Lysosomal storage diseases are a group of disorders where accumulation of catabolites is manifested in the lysosomes of different cell types. In metachromatic leukodystrophy (Arylsulfatase A [EC.3.1.6.8] deficiency) storage of the glycosphingolipid sulfatide in t...
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| Format: | Article |
| Language: | English |
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BMC
2011-02-01
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| Series: | Lipids in Health and Disease |
| Online Access: | http://www.lipidworld.com/content/10/1/28 |
| _version_ | 1811315885904756736 |
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| author | Månsson Jan-Eric Gieselmann Volkmar Blomqvist Maria |
| author_facet | Månsson Jan-Eric Gieselmann Volkmar Blomqvist Maria |
| author_sort | Månsson Jan-Eric |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Lysosomal storage diseases are a group of disorders where accumulation of catabolites is manifested in the lysosomes of different cell types. In metachromatic leukodystrophy (Arylsulfatase A [EC.3.1.6.8] deficiency) storage of the glycosphingolipid sulfatide in the brain leads to demyelination, resulting in neuromotor co-ordination deficits and regression. In a mouse model for metachromatic leukodystrophy, the ASA null mutant mouse, the accumulation of sulfatide in correlation to phenotype has been thoroughly investigated. Another lipid species reported to accumulate in patients with metachromatic leukodystrophy is the sulfatide related lipid lysosulfatide. Lysosulfatide was shown to be a cytotoxic compound in cell culture experiments and thus suggested to be involved in the pathology of metachromatic leukodystrophy. In this study, we further investigated the developmental profile of lysosulfatide in the brain of ASA null mutant mice by using high performance liquid chromatography. Lysosulfatide could be detected in the brain of normal mice (ASA +/+) from 1.8 months up to 23.1 months of age. From the age of 8.8 months the lysosulfatide levels remained constant at 1 pmol/mg wet tissue. The developmental change (< 20 months) of brain lysosulfatide showed an accumulation in ASA null mutant mice at ages above one month compared to its normal counterpart (ASA +/+). Thus, the ASA null mutant mouse might be a suitable model to further investigate the role of lysosulfatide in the pathogenesis of metachromatic leukodystrophy.</p> |
| first_indexed | 2024-04-13T11:39:28Z |
| format | Article |
| id | doaj.art-365d5e003a6a43f38ed1df3a4ff7e288 |
| institution | Directory Open Access Journal |
| issn | 1476-511X |
| language | English |
| last_indexed | 2024-04-13T11:39:28Z |
| publishDate | 2011-02-01 |
| publisher | BMC |
| record_format | Article |
| series | Lipids in Health and Disease |
| spelling | doaj.art-365d5e003a6a43f38ed1df3a4ff7e2882022-12-22T02:48:21ZengBMCLipids in Health and Disease1476-511X2011-02-011012810.1186/1476-511X-10-28Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient miceMånsson Jan-EricGieselmann VolkmarBlomqvist Maria<p>Abstract</p> <p>Lysosomal storage diseases are a group of disorders where accumulation of catabolites is manifested in the lysosomes of different cell types. In metachromatic leukodystrophy (Arylsulfatase A [EC.3.1.6.8] deficiency) storage of the glycosphingolipid sulfatide in the brain leads to demyelination, resulting in neuromotor co-ordination deficits and regression. In a mouse model for metachromatic leukodystrophy, the ASA null mutant mouse, the accumulation of sulfatide in correlation to phenotype has been thoroughly investigated. Another lipid species reported to accumulate in patients with metachromatic leukodystrophy is the sulfatide related lipid lysosulfatide. Lysosulfatide was shown to be a cytotoxic compound in cell culture experiments and thus suggested to be involved in the pathology of metachromatic leukodystrophy. In this study, we further investigated the developmental profile of lysosulfatide in the brain of ASA null mutant mice by using high performance liquid chromatography. Lysosulfatide could be detected in the brain of normal mice (ASA +/+) from 1.8 months up to 23.1 months of age. From the age of 8.8 months the lysosulfatide levels remained constant at 1 pmol/mg wet tissue. The developmental change (< 20 months) of brain lysosulfatide showed an accumulation in ASA null mutant mice at ages above one month compared to its normal counterpart (ASA +/+). Thus, the ASA null mutant mouse might be a suitable model to further investigate the role of lysosulfatide in the pathogenesis of metachromatic leukodystrophy.</p>http://www.lipidworld.com/content/10/1/28 |
| spellingShingle | Månsson Jan-Eric Gieselmann Volkmar Blomqvist Maria Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice Lipids in Health and Disease |
| title | Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice |
| title_full | Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice |
| title_fullStr | Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice |
| title_full_unstemmed | Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice |
| title_short | Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice |
| title_sort | accumulation of lysosulfatide in the brain of arylsulfatase a deficient mice |
| url | http://www.lipidworld.com/content/10/1/28 |
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