| http://purl.uniprot.org/citations/31701076 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/31701076 | http://www.w3.org/2000/01/rdf-schema#comment | "Mutations in the liver glycogen phosphorylase (Pygl) gene are associated with the diagnosis of glycogen storage disease type VI (GSD-VI). To understand the pathogenesis of GSD-VI, we generated a mouse model with Pygl deficiency (Pygl -/-). Pygl -/- mice exhibit hepatomegaly, excessive hepatic glycogen accumulation, and low hepatic free glucose along with lower fasting blood glucose levels and elevated blood ketone bodies. Hepatic glycogen accumulation in Pygl -/- mice increases with age. Masson's trichrome and picrosirius red staining revealed minimal to mild collagen deposition in periportal, subcapsular, and/or perisinusoidal areas in the livers of old Pygl -/- mice (>40 weeks). Consistently, immunohistochemical analysis showed the number of cells positive for alpha smooth muscle actin (α-SMA), a marker of activated hepatic stellate cells, was increased in the livers of old Pygl -/- mice compared with those of age-matched wild-type (WT) mice. Furthermore, old Pygl -/- mice had inflammatory infiltrates associated with hepatic vessels in their livers along with up-regulated hepatic messenger RNA levels of C-C chemokine ligand 5 (Ccl5/Rantes) and monocyte chemoattractant protein 1 (Mcp-1), indicating inflammation, while age-matched WT mice did not. Serum levels of aspartate aminotransferase and alanine aminotransferase were elevated in old Pygl -/- mice, indicating liver damage. Conclusion: Pygl deficiency results in progressive accumulation of hepatic glycogen with age and liver damage, inflammation, and collagen deposition, which can increase the risk of liver fibrosis. Collectively, the Pygl-deficient mouse recapitulates clinical features in patients with GSD-VI and provides a model to elucidate the mechanisms underlying hepatic complications associated with defective glycogen metabolism."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.org/dc/terms/identifier | "doi:10.1002/hep4.1426"xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Smyth J.A."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Wu R."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Brown L.M."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Cho J.H."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Lee Y.M."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Estrella A."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Weinstein D.A."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Chengsupanimit T."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/author | "Wilson L.H."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/date | "2019"xsd:gYear |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/name | "Hepatol Commun"xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/pages | "1544-1555"xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/title | "Liver Glycogen Phosphorylase Deficiency Leads to Profibrogenic Phenotype in a Murine Model of Glycogen Storage Disease Type VI."xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://purl.uniprot.org/core/volume | "3"xsd:string |
| http://purl.uniprot.org/citations/31701076 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/31701076 |
| http://purl.uniprot.org/citations/31701076 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/31701076 |
| http://purl.uniprot.org/uniprot/Q9ET01#attribution-E53BF8B2896E872BFB9F72417CF5FA45 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/31701076 |
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| http://purl.uniprot.org/uniprot/#_Q3UEJ6-mappedCitation-31701076 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/31701076 |
| http://purl.uniprot.org/uniprot/#_Q3TJQ7-mappedCitation-31701076 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/31701076 |
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| http://purl.uniprot.org/uniprot/#_Q9ET01-mappedCitation-31701076 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/31701076 |