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Series GSE86462 Query DataSets for GSE86462
Status Public on Oct 20, 2016
Title HNRNPA2B1 regulates alternative RNA processing in the nervous system and accumulates in granules in ALS IPSC-derived motor neurons [hnRNPA2B1_Arrays_human_iPSC_MN_Stress]
Organism Homo sapiens
Experiment type Expression profiling by array
Summary HnRNPA2B1 encodes an RNA binding protein associated with neurodegenerative disorders. However, its function in the nervous system is unclear. Transcriptome-wide cross-linking and immunoprecipitation in mouse spinal cord discover UAGG motifs enriched within ~2,500 hnRNP A2/B1 binding sites and an unexpected role for hnRNP A2/B1 in alternative polyadenylation. Loss of hnRNP A2/B1 results in alternative splicing, including skipping of an exon in amyotrophic lateral sclerosis (ALS)-associated D-amino acid oxidase (DAO) that reduces D-serine metabolism. Inclusion of the DAO exon is also reduced in transgenic ALS mice models. ALS-associated hnRNP A2/B1 D290V mutant patient fibroblasts and motor neurons differentiated from induced pluripotent stem cells demonstrate gain-of-mutant-dependent splicing differences. Mutant motor neurons also exhibit increased hnRNP A2/B1 localization to cytoplasmic granules during stress, which are abrogated by a small molecule CA43. Our findings and cellular resource identify RNA networks affected in loss of normal and mutated hnRNP A2/B1 with broad relevance to neurodegeneration.
 
Overall design Microarray in human iPSC-MNs from patients with hnRNP A2/B1 D290V, VCP R155H, mutations or controls. Samples treated with puromycin or saline. 3 replicates per condition.
 
Contributor(s) Martinez FJ, Pratt GA, Van Nostrand E, Batra R, Huelga SC, Kapeli K, Freese P, Chun SJ, Ling K, Gelboin-Burkhart C, Fijany L, Wang H, Nussbacher JK, Kim H, Lardelli R, Sundararaman B, Donohue JP, Lykke-Andersen J, Bennett F, Ares M Jr, Burge CB, Taylor JP, Rigo F, Yeo GW
Citation(s) 27773581
Submission date Sep 06, 2016
Last update date Oct 26, 2016
Contact name Gene Yeo
E-mail(s) geneyeo@ucsd.edu
Organization name UCSD
Street address 2880 Torrey Pines Scenic Dr. Room 3805/Yeo Lab
City La Jolla
State/province CA
ZIP/Postal code 92037
Country USA
 
Platforms (1)
GPL17585 [HTA-2_0] Affymetrix Human Transcriptome Array 2.0 [probe set (exon) version]
Samples (42)
GSM2303046 15436.Yeo.1_HTA-2_0
GSM2303047 15437.Yeo.2_HTA-2_0
GSM2303048 15438.Yeo.3_HTA-2_0
This SubSeries is part of SuperSeries:
GSE86464 HNRNPA2B1 regulates alternative RNA processing in the nervous system and accumulates in granules in ALS IPSC-derived motor neurons
Relations
BioProject PRJNA341996

Download family Format
SOFT formatted family file(s) SOFTHelp
MINiML formatted family file(s) MINiMLHelp
Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE86462_RAW.tar 1.0 Gb (http)(custom) TAR (of CEL)
Processed data included within Sample table

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