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Series GSE248598 Query DataSets for GSE248598
Status Public on May 22, 2024
Title Deficiency in peroxisomes underlies failures in neonatal hepatic immune cell hematopoiesis and antigen presentation in a severe Zellweger disease model
Organism Mus musculus
Experiment type Expression profiling by high throughput sequencing
Summary Peroxisome Biogenesis disorders (PBDs) represent a group of metabolic conditions that cause severe developmental defects. Peroxisomes are essential metabolic organelles, present in virtually every eukaryotic cell and mediating key processes in immunometabolism. To date, the full spectrum of PBDs remains to be identified, and the impact PBDs have on immune function is unexplored. This study presents a characterization of the hepatic immune compartment of a neonatal PBD mouse model at single-cell resolution to establish the importance and function of peroxisomes in developmental hematopoiesis. We report that hematopoietic defects are a feature in a severe PBD murine model. Finally, we identify a role for peroxisomes in the regulation of the major histocompatibility class II expression and antigen presentation to CD4+ T cells in dendritic cells. This study adds to our understanding of the mechanisms of PBDs and expands our knowledge of the role of peroxisomes in immunometabolism.
Overall design Liver immune cells from pex2 homozygous null mutant, pex2 heterozygous, and wildtype neonatal mice were isolated by Fluorescence-activeated cell sorting (FACs) according to fluorescence-conjugted anti-CD45 antibody labelling and viability staining and analyzed using scRNAseq.
Contributor(s) Parsons BD, Medina-Luna D, Scur M, Pinelli M, Gamage GS, Chilvers RA, Hamon Y, Ahmed I, Savary S, Makrigiannis AP, Braverman NE, Alcazar JR, Latz E, Karakach T, Di Cara F
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Submission date Nov 24, 2023
Last update date May 22, 2024
Contact name Francesca Di Cara
Organization name Dalhousie University
Street address 5850/5980 University Avenue
City Halifax
State/province Nova Scotia
ZIP/Postal code B3K 6R8
Country Canada
Platforms (1)
GPL24247 Illumina NovaSeq 6000 (Mus musculus)
Samples (3)
GSM7917813 liver CD45+ cells, pex2 WT, Rep 1
GSM7917814 liver CD45+ cells, pex2 Het, Rep 1
GSM7917815 liver CD45+ cells, pex2 KO, Rep1
BioProject PRJNA1044828

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Supplementary file Size Download File type/resource
GSE248598_RAW.tar 320.8 Mb (http)(custom) TAR (of MTX, TSV)
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Raw data are available in SRA
Processed data provided as supplementary file

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