| pubmed-article:19416667 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C0162429 | lld:lifeskim |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C0030664 | lld:lifeskim |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C0025914 | lld:lifeskim |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C0026809 | lld:lifeskim |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C0007765 | lld:lifeskim |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C0011155 | lld:lifeskim |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C0380804 | lld:lifeskim |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C1705994 | lld:lifeskim |
| pubmed-article:19416667 | lifeskim:mentions | umls-concept:C1513492 | lld:lifeskim |
| pubmed-article:19416667 | pubmed:issue | 1 | lld:pubmed |
| pubmed-article:19416667 | pubmed:dateCreated | 2009-5-8 | lld:pubmed |
| pubmed-article:19416667 | pubmed:abstractText | Infantile neuronal ceroid lipofuscinosis (INCL, Infantile Batten Disease) is an inherited, neurodegenerative lysosomal storage disorder. INCL is the result of a CLN1 gene mutation leading to a deficiency in palmitoyl protein thioesterase 1 (PPT1) activity. Studies in the forebrain demonstrate the PPT1-deficient mouse (PPT1-/-) mimics the clinical symptoms and underlying pathology of INCL; however, little is known about changes in cerebellar function or pathology. In this study, we demonstrate Purkinje cell loss beginning at 3 months, which correlates with changes in rotarod performance. Concurrently, we observed an early stage reactive gliosis and a primary pathology in astrocytes, including changes in S100beta and GLAST expression. Conversely, there was a late stage granule cell loss, microglial activation, and demyelination. This study suggests that neuronal-glial interactions are the core pathology in the PPT1-/- cerebellum. In addition, these data identify potential endpoints for use in future efficacy studies for the treatment of INCL. | lld:pubmed |
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| pubmed-article:19416667 | pubmed:language | eng | lld:pubmed |
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| pubmed-article:19416667 | pubmed:citationSubset | IM | lld:pubmed |
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| pubmed-article:19416667 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:19416667 | pubmed:month | May | lld:pubmed |
| pubmed-article:19416667 | pubmed:issn | 1090-2430 | lld:pubmed |
| pubmed-article:19416667 | pubmed:author | pubmed-author:CooperJonatha... | lld:pubmed |
| pubmed-article:19416667 | pubmed:author | pubmed-author:WozniakDavid... | lld:pubmed |
| pubmed-article:19416667 | pubmed:author | pubmed-author:SandsMark SMS | lld:pubmed |
| pubmed-article:19416667 | pubmed:author | pubmed-author:TanYunY | lld:pubmed |
| pubmed-article:19416667 | pubmed:author | pubmed-author:MacauleyShann... | lld:pubmed |
| pubmed-article:19416667 | pubmed:author | pubmed-author:KielarCatheri... | lld:pubmed |
| pubmed-article:19416667 | pubmed:issnType | Electronic | lld:pubmed |
| pubmed-article:19416667 | pubmed:volume | 217 | lld:pubmed |
| pubmed-article:19416667 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:19416667 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:19416667 | pubmed:pagination | 124-35 | lld:pubmed |
| pubmed-article:19416667 | pubmed:dateRevised | 2011-6-1 | lld:pubmed |
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