| http://purl.uniprot.org/citations/26775238 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/26775238 | http://www.w3.org/2000/01/rdf-schema#comment | "CLN diseases are mostly fatal lysosomal storage diseases that lead to neurodegeneration in the CNS. We have previously shown that CD8+ T-lymphocytes contribute to axonal perturbation and neuron loss in the CNS of Ppt1(-/-) mice, a model of CLN1 disease. We now investigated the role of the inflammation-related cell adhesion molecule sialoadhesin (Sn) in Ppt1(-/-) and Cln3(-/-) mice, a model of the most frequent form, CLN3 disease. Microglia/macrophages in the CNS of both models showed an upregulation of Sn and markers for proinflammatory M1 polarization and antigen presentation. Sn+ microglia/macrophages associated with SMI32+ axonal spheroids and CD8+ T-lymphocytes. To analyze their pathogenic impact, we crossbred both models with Sn-deficient mice and scored axonal degeneration and neuronal integrity using immunohistochemistry, electron microscopy and optical coherence tomography. Degenerative alterations in the retinotectal pathway of Ppt1(-/-)Sn(-/-) and Cln3(-/-)Sn(-/-) mice were significantly reduced. Ppt1(-/-)Sn(-/-) mice also showed a substantially improved clinical phenotype and extended lifespan, attenuated numbers of M1-polarized microglia/macrophages and reduced expression levels of proinflammatory cytokines. This was accompanied by an increased frequency of CD8+CD122+ T-lymphocytes in the CNS of Ppt1(-/-)Sn(-/-) mice, the regulatory phenotype of which was demonstrated by impaired survival of CD8+CD122-effector T-lymphocytes in co-culture experiments. We show for the first time that increased Sn expression on microglia/macrophages contributes to neural perturbation in two distinct models of CLN disease. Our data also indicate that a rarely described CD8+CD122+ T-cell population can regulate the corresponding diseases. These studies provide insights into CLN pathogenesis and may guide in designing immuno-regulatory treatment strategies."xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.org/dc/terms/identifier | "doi:10.1002/glia.22962"xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/author | "Martini R."xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/author | "Stadler D."xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/author | "Lutz M.B."xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/author | "Schilling T."xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/author | "Groh J."xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/author | "Ribechini E."xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/date | "2016"xsd:gYear |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/name | "Glia"xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/pages | "792-809"xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/title | "Sialoadhesin promotes neuroinflammation-related disease progression in two mouse models of CLN disease."xsd:string |
| http://purl.uniprot.org/citations/26775238 | http://purl.uniprot.org/core/volume | "64"xsd:string |
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