GEO DataSets

Analysis of HeLa cells expressing the G392E mutant of neuroserpin. Accumulation of mutant neuroserpin polymers in the ER is associated with familial encephalopathy with neuroserpin inclusion bodies. Results provide insight into pathways initiated by the polymerization of neuroserpin within the ER.

Organism:

Homo sapiens

Type:

Expression profiling by array, transformed count, 2 agent, 4 genotype/variation sets

Platform:

GPL10558

Series:

GSE46230

24 Samples

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(Submitter supplied) HeLa cells were stably transfected using the Tet-On® Advanced Cell Line system with Wildtype, G392E and Delta Neuroserpin with neuroserpin expression induced with doxycycline. Gene expression was examined in the absence or presence of 2 ug/ml doxycycline.

Organism:

Homo sapiens

Type:

Expression profiling by array

Dataset:

GDS5064

Platform:

GPL10558

24 Samples

Download data: TXT

(Submitter supplied) The serpinopathies are human pathologies caused by mutations that promote polymerisation and intracellular deposition of proteins of the serpin superfamily, leading to a poorly understood cell toxicity. The dementia familial encephalopathy with neuroserpin inclusion bodies (FENIB) is caused by polymerisation of the neuronal serpin neuroserpin (NS) within the endoplasmic reticulum (ER) of neurons. We have generated transgenic neural progenitor cell (NPC) cultures from mouse embryonic cerebral cortex, stably expressing the control protein GFP (green fluorescent protein), or human wild type, G392E or deltaNS. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL13112

6 Samples

Download data: TXT