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Series GSE52929 Query DataSets for GSE52929
Status Public on Dec 04, 2013
Title Sel1L is Indispensable for Mammalian ERAD, ER Homeostasis and Survival
Organism Mus musculus
Experiment type Expression profiling by array
Summary Sel1L is an adaptor protein for the E3 ligase Hrd1 involved in endoplasmic reticulum-associated degradation (ERAD). Its physiological importance in mammalian ERAD, however, remains to be established. Here, using the inducible Sel1L knockout mouse and cell models, we provide the first in vivo evidence that Sel1L is indispensable for Hrd1 stability, ER homeostasis and survival. Acute loss of Sel1L leads to premature death in adult mice within 3 weeks with profound pancreatic atrophy. Contrary to current belief, our data show that mammalian Sel1L is required for Hrd1 stability and ERAD function both in vitro and in vivo. Sel1L deficiency disturbs ER homeostasis, activates ER stress, attenuates translation and promotes cell death. Serendipitously, using biochemical approach coupled with mass spectrometry, we found that Sel1L deficiency causes the aggregation of both small and large ribosomal subunits. Thus, Sel1L is an indispensable component of mammalian ERAD and ER homeostasis, which is essential for protein translation, pancreatic function, cellular and organismal survival.
Overall design Pancreas tissue of wild type and inducible Sel1L knockout mice were subjected to gene expression analysis.
Contributor(s) Sun S, Shi G, Han X, Francisco AB, Ji Y, Mendonça N, Liu X, Locasale JW, Simpson KW, Duhamel G, Kersten S, Yates III JR, Long Q, Qi L
Citation(s) 24453213
Submission date Dec 03, 2013
Last update date Apr 18, 2017
Contact name Guido Hooiveld
Organization name Wageningen University
Department Div. Human Nutrition & Health
Lab Nutrition, Metabolism & Genomics Group
Street address HELIX, Stippeneng 4
City Wageningen
ZIP/Postal code NL-6708WE
Country Netherlands
Platforms (1)
GPL11533 [MoGene-1_1-st] Affymetrix Mouse Gene 1.1 ST Array [transcript (gene) version]
Samples (8)
GSM1278268 pancreas_wildtype_rep1
GSM1278269 pancreas_wildtype_rep2
GSM1278270 pancreas_wildtype_rep3
BioProject PRJNA230475

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Supplementary file Size Download File type/resource
GSE52929_RAW.tar 35.0 Mb (http)(custom) TAR (of CEL)
Processed data included within Sample table

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