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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
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
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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