PUBLICATION

Systematic transcriptome analysis of the zebrafish model of diamond-blackfan anemia induced by RPS24 deficiency

Authors
Song, B., Zhang, Q., Zhang, Z., Wan, Y., Jia, Q., Wang, X., Zhu, X., Leung, A.Y., Cheng, T., Fang, X., Yuan, W., Jia, H.
ID
ZDB-PUB-140906-5
Date
2014
Source
BMC Genomics   15: 759 (Journal)
Registered Authors
Jia, Haibo, Leung, Anskar
Keywords
none
Datasets
GEO:GSE54259, GEO:GSE54270, GEO:GSE54152
MeSH Terms
  • Anemia, Diamond-Blackfan/genetics*
  • Anemia, Diamond-Blackfan/metabolism
  • Animals
  • Cluster Analysis
  • Disease Models, Animal
  • Erythroid Precursor Cells/metabolism
  • Gene Expression Profiling*
  • Gene Expression Regulation
  • Gene Knockout Techniques
  • Gene Regulatory Networks
  • Hematopoiesis/genetics
  • Hematopoietic Stem Cells/metabolism
  • Humans
  • MicroRNAs/genetics
  • MicroRNAs/metabolism
  • Morpholinos/genetics
  • Morpholinos/metabolism
  • Phenotype
  • Ribosomal Proteins/deficiency
  • Ribosomal Proteins/genetics
  • Ribosomal Proteins/metabolism
  • Signal Transduction
  • Transcriptome*
  • Tumor Suppressor Protein p53
  • Zebrafish/genetics
PubMed
25189322 Full text @ BMC Genomics
Abstract
Diamond-Blackfan anemia (DBA) is a class of human diseases linked to defective ribosome biogenesis that results in clinical phenotypes. Genetic mutations in ribosome protein (RP) genes lead to DBA phenotypes, including hematopoietic defects and physical deformities. However, little is known about the global regulatory network as well as key miRNAs and gene pathways in the zebrafish model of DBA.
In this study, we establish the DBA model in zebrafish using an RPS24 morpholino and found that RPS24 is required for both primitive hematopoiesis and definitive hematopoiesis processes that are partially mediated by the p53 pathway. Several deregulated genes and miRNAs were found to be related to hematopoiesis, vascular development and apoptosis in RPS24-deficient zebrafish via RNA-seq and miRNA-seq data analysis, and a comprehensive regulatory network was first constructed to identify the mechanisms of key miRNAs and gene pathways in the model. Interestingly, we found that the central node genes in the network were almost all targeted by significantly deregulated miRNAs. Furthermore, the enforced expression of miR-142-3p, a uniquely expressed miRNA, causes a significant decrease in primitive erythrocyte progenitor cells and HSCs.
The present analyses demonstrate that the comprehensive regulatory network we constructed is useful for the functional prediction of new and important miRNAs in DBA and will provide insights into the pathogenesis of mutant rps24-mediated human DBA disease.
Genes / Markers
Figures
Show all Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping