{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T13:24:46Z","timestamp":1791206686812,"version":"4.1.0"},"reference-count":44,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2010,3,17]],"date-time":"2010-03-17T00:00:00Z","timestamp":1268784000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2010,3,17]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The neuronal ceroid lipofuscinoses (NCLs) constitute a group of autosomal recessive neurodegenerative diseases affecting children. To date, the disease pathogenesis remains unknown, although the role of lysosomal impairment is widely recognized across the different diseases. Recently, the creation of simple models of juvenile NCL (Batten disease) has provided additional insights into the disease mechanism at the molecular level. We report defects in metabolism identified in the Schizosacchromyces pombe yeast model, where btn1, the orthologue of CLN3, has been deleted, using a metabolomics approach based on high resolution 1H and 13C NMR spectroscopy. Such changes represent the first documented metabolic changes associated with deletion of btn1. A decrease in extracellularglucose and increases in the concentration of extracellularethanol and alaninelabelling demonstrate increased glycolytic flux that may arise from vacuolar impairment, whilst amino acid changes were detected which were also in accordance with defective vacuolar functionality. That these changes were detected using a metabolomic based approach advocates its use to further analyse other yeast models of human disease to better understand the function of orthologuegenes.<\/jats:p>\n                  <jats:p\/>","DOI":"10.1039\/b915670d","type":"journal-article","created":{"date-parts":[[2010,3,18]],"date-time":"2010-03-18T05:30:22Z","timestamp":1268890222000},"page":"1093-1102","source":"Crossref","is-referenced-by-count":21,"title":["Deletion of\n                    <i>btn<\/i>\n                    1, an orthologue of\n                    <i>CLN<\/i>\n                    3, increases glycolysis and perturbs amino acidmetabolism in the fission yeast model of Batten disease"],"prefix":"10.1093","volume":"6","author":[{"given":"Michael R","family":"Pears","sequence":"first","affiliation":[{"name":"Department of Biochemistry, University of Cambridge, Hopkins Building, Tennis Court Road a , Cambridge, CB2 1QW, UK \u00a0 jlg40@cam.ac.uk \u00a0 +44 (0)1223 764948"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Codlin","sequence":"additional","affiliation":[{"name":"MRC Laboratory for Molecular Cell Biology, University College London b , Gower Street, London, WC1E 6BT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca L","family":"Haines","sequence":"additional","affiliation":[{"name":"MRC Laboratory for Molecular Cell Biology, University College London b , Gower Street, London, WC1E 6BT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian J","family":"White","sequence":"additional","affiliation":[{"name":"MRC Laboratory for Molecular Cell Biology, University College London b , Gower Street, London, WC1E 6BT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell J","family":"Mortishire-Smith","sequence":"additional","affiliation":[{"name":"Johnson & Johnson PR&D c , Turnhoutseweg 30, Beerse B-2340 Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara E","family":"Mole","sequence":"additional","affiliation":[{"name":"Department of Genetics, Evolution and Environment, University College London e , Gower Street, London, WC1E 6BT, UK"},{"name":"Molecular Medicine Unit, UCL Institute of Child Health, University College London d , UK"},{"name":"MRC Laboratory for Molecular Cell Biology, University College London b , Gower Street, London, WC1E 6BT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian L","family":"Griffin","sequence":"additional","affiliation":[{"name":"The Cambridge Centre for Systems Biology, University of Cambridge, Hopkins Building, Tennis Court Road f , Cambridge, CB2 1GA, UK"},{"name":"Department of Biochemistry, University of Cambridge, Hopkins Building, Tennis Court Road a , Cambridge, CB2 1QW, UK \u00a0 jlg40@cam.ac.uk \u00a0 +44 (0)1223 764948"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2010,3,17]]},"reference":[{"key":"2026012913220451100_cit1","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1111\/j.1750-3639.1998.tb00142.x","volume":"8","author":"Goebel","year":"1998","journal-title":"Brain Pathol."},{"key":"2026012913220451100_cit2","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1111\/j.1750-3639.2004.tb00499.x","volume":"14","author":"Goebel","year":"2004","journal-title":"Brain Pathol."},{"key":"2026012913220451100_cit3","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.bbadis.2006.05.006","volume":"1762","author":"Siintola","year":"2006","journal-title":"Biochim. 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