{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Ohmi K"],"funding":["NIDDK NIH HHS","NINDS NIH HHS"],"pagination":["1902-7"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC149931"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["100(4)"],"pubmed_abstract":["Alpha-N-acetylglucosaminidase deficiency (mucopolysaccharidosis IIIB, MPS IIIB) and alpha-l-iduronidase deficiency (MPS I) are heritable lysosomal storage diseases; neurodegeneration is prominent in MPS IIIB and in severe cases of MPS I. We have obtained morphologic and molecular evidence for the involvement of microglia in brain pathology of mouse models of the two diseases. In the cortex, a subset of microglia (sometimes perineuronal) consists of cells that are probably phagocytic; they have large storage vacuoles, react with MOMA-2 (monoclonal antibody against macrophages) and Griffonia simplicifolia isolectin IB(4), and stain intensely for the lysosomal proteins Lamp-1, Lamp-2, and cathepsin D as well as for G(M3) ganglioside. MOMA-2-positive cells appear at 1 and 6 months in MPS IIIB "],"journal":["Proceedings of the National Academy of Sciences of the United States of America"],"pubmed_title":["Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB."],"pmcid":["PMC149931"],"funding_grant_id":["DK38857","R01 NS022376","NS22376"],"pubmed_authors":["Neufeld EF","Ryazantsev S","Ohmi K","Rajavel KS","Li HH","Greenberg DS"],"additional_accession":[]},"is_claimable":false,"name":"Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB.","description":"Alpha-N-acetylglucosaminidase deficiency (mucopolysaccharidosis IIIB, MPS IIIB) and alpha-l-iduronidase deficiency (MPS I) are heritable lysosomal storage diseases; neurodegeneration is prominent in MPS IIIB and in severe cases of MPS I. We have obtained morphologic and molecular evidence for the involvement of microglia in brain pathology of mouse models of the two diseases. In the cortex, a subset of microglia (sometimes perineuronal) consists of cells that are probably phagocytic; they have large storage vacuoles, react with MOMA-2 (monoclonal antibody against macrophages) and Griffonia simplicifolia isolectin IB(4), and stain intensely for the lysosomal proteins Lamp-1, Lamp-2, and cathepsin D as well as for G(M3) ganglioside. MOMA-2-positive cells appear at 1 and 6 months in MPS IIIB ","dates":{"release":"2003-01-01T00:00:00Z","publication":"2003 Feb","modification":"2025-04-05T00:18:46.798Z","creation":"2019-03-26T23:29:37Z"},"accession":"S-EPMC149931","cross_references":{"pubmed":["12576554"],"doi":["10.1073/pnas.252784899"]}}