| pubmed-article:17482562 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C0035820 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C0017337 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C0751383 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C1413495 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C0220781 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C0004083 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C1883254 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C0279266 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C0679622 | lld:lifeskim |
| pubmed-article:17482562 | lifeskim:mentions | umls-concept:C0205314 | lld:lifeskim |
| pubmed-article:17482562 | pubmed:issue | 1 | lld:pubmed |
| pubmed-article:17482562 | pubmed:dateCreated | 2007-5-15 | lld:pubmed |
| pubmed-article:17482562 | pubmed:abstractText | Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) results from a deficiency of CLN3, a protein recently identified within detergent-resistant membranes (DRMs). To study the function of CLN3 within these domains we isolated DRMs from control and JNCL-brain and noted that JNCL-derived DRMs are less buoyant than control. Analysis of DRM phospholipids derived from JNCL-brain revealed a reduction of bis(monoacylglycerol)phosphate. Metabolic labeling of JNCL-fibroblasts demonstrated a reduction in the synthesis of bis(monoacylglycerol)phosphate which was restored following complementation with wild-type-CLN3, substantiating our initial observation in brain. Metabolic labeling of cell lines overexpressing wild-type-CLN3 resulted in increased bis(monoacylglycerol)phosphate synthesis, while overexpression of mutant CLN3-L170P decreased bis(monoacylglycerol)phosphate synthesis. These data illustrate a new finding, a strong correlation between CLN3 protein expression and synthesis of bis(monoacylglycerol)phosphate. | lld:pubmed |
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| pubmed-article:17482562 | pubmed:language | eng | lld:pubmed |
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| pubmed-article:17482562 | pubmed:citationSubset | IM | lld:pubmed |
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| pubmed-article:17482562 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:17482562 | pubmed:month | Jun | lld:pubmed |
| pubmed-article:17482562 | pubmed:issn | 0006-291X | lld:pubmed |
| pubmed-article:17482562 | pubmed:author | pubmed-author:DawsonGlynG | lld:pubmed |
| pubmed-article:17482562 | pubmed:author | pubmed-author:HobertJudith... | lld:pubmed |
| pubmed-article:17482562 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:17482562 | pubmed:day | 22 | lld:pubmed |
| pubmed-article:17482562 | pubmed:volume | 358 | lld:pubmed |
| pubmed-article:17482562 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:17482562 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:17482562 | pubmed:pagination | 111-6 | lld:pubmed |
| pubmed-article:17482562 | pubmed:dateRevised | 2011-9-26 | lld:pubmed |
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| pubmed-article:17482562 | pubmed:year | 2007 | lld:pubmed |
| pubmed-article:17482562 | pubmed:articleTitle | A novel role of the Batten disease gene CLN3: association with BMP synthesis. | lld:pubmed |
| pubmed-article:17482562 | pubmed:affiliation | Committee on Molecular Metabolism and Nutrition, Biological Sciences Division, The University of Chicago, Chicago, IL, USA. hobertj@ccf.org | lld:pubmed |
| pubmed-article:17482562 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:17482562 | pubmed:publicationType | Research Support, N.I.H., Extramural | lld:pubmed |
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