PubMed_ArguminSci  

PubMed:24269732 JSONTXT 8 Projects

Annnotations TAB TSV DIC JSON TextAE

Id Subject Object Predicate Lexical cue
T1 109-121 DRI_Background denotes The neuronal
T2 144-273 DRI_Background denotes (NCLs, Batten disease) are inherited neurodegenerative lysosomal storage diseases caused by mutations in several different genes.
T3 274-391 DRI_Challenge denotes Mutations in CLN5 cause a variant late-infantile human disease and some cases of juvenile and adult clinical disease.
T4 392-470 DRI_Background denotes NCLs also occur in animals, and a flock of New Zealand Borderdale sheep with a
T5 488-534 DRI_Background denotes mutation has been developed for model studies.
T6 535-757 DRI_Background denotes Dissociated mixed neural cells from CLN5-deficient foetal sheep brains contained no obvious storage bodies at plating but these accumulated rapidly in culture, mainly in microglial cells and also in neurons and astrocytes.
T7 758-904 DRI_Background denotes Accumulation was very obvious after a week, as monitored by fluorescent microscopy and immunostaining for subunit c of mitochondrial ATP synthase.
T8 905-1154 DRI_Background denotes Photography at intervals revealed the dynamic nature of the cultures and a flow of storage bodies between cells, specifically the phagocytosis of storage-body containing cells by microglia and incorporation of the storage bodies into the host cells.
T9 1155-1205 DRI_Background denotes No storage was observed in cultured control cells.
T10 1206-1379 DRI_Background denotes Transduction of cell cultures with a lentiviral vector expressing a C-terminal Myc tagged CLN5 resulted in secretion of post-translationally glycosylated and processed CLN5.
T11 1380-1519 DRI_Outcome denotes Transduction of CLN5-deficient cultures with this construct rapidly reversed storage body accumulation, to less than half in only six days.
T12 1520-1703 DRI_Background denotes These results show that storage body accumulation is reversible with enzyme correction and support the use of these cultures for testing of therapeutics prior to whole animal studies.