Additional file 3.
Facilitated lysosomal trafficking of the mutated CLN5 by PPT1 overexpression in SH-SY5Y cells. Human neuroblastoma cells (SH-SY5Y), were transiently transfected with wt CLN1/PPT1 and CLN5-Fin, carrying the most common vLINCL(Fin) causing mutation (A-E), or with CLN5-Fin alone (F-H). The cells were fixed with methanol 48 h post transfection, stained and analyzed by confocal microscopy. When CLN5-Fin was co-expressed with wt PPT1, CLN5-Fin was able to traffic to lysosomes with PPT1 (A-E). When CLN5-Fin was expressed alone, it retained in the ER (F-H). Scale bar 10 μm.
Format: PDF Size: 3MB Download file
This file can be viewed with: Adobe Acrobat Reader
Lyly et al. BMC Cell Biology 2009 10:83 doi:10.1186/1471-2121-10-83