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Links from GEO DataSets

Items: 18

1.

Rules governing translation selectivity under limiting ribosome availability

(Submitter supplied) Ribosomopathies are cell-type-specific pathologies related to a ribosomal protein (RP) gene insult. The 5q- syndrome is a somatic ribosomopathy linked to RPS14 gene haploinsufficiency and characterized by a prominent erythroid hypoplasia. Using quantitative proteomic, we show that GATA1 protein expression is low in shRPS14 cells in which ribosome quantities are diminished. Here, we investigated the cause of low GATA1 protein expression in limiting ribosome availability. more...
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL24299
6 Samples
Download data: CEL, TXT
Series
Accession:
GSE124800
ID:
200124800
2.

Integrated analyses of translatome and proteome identify the rules of translation selectivity in RPS14-deficient

(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL24299
21 Samples
Download data: CEL
Series
Accession:
GSE108822
ID:
200108822
3.

mRNA length and 3’UTR structure govern the translation selectivity in RPS14-haploinsufficient erythroblasts [translatome]

(Submitter supplied) 5q- syndrome is a somatic ribosomopathy linked to the monoallelic deletion of the RPS14 gene and characterized by a proeminent erythroid phenotype. The mechanism of anemia involves an impaired differentiation and increased apoptosis of erythroblasts. Here we show that GATA1 protein expression is low in line with a defect in the representation of its mRNA at the ribosome. A global analysis of transcripts on polysomes indicates that translation is selective with a decreased representation of the transcripts with a short coding sequence and UTRs and a highly structured 3’UTR, a subset of transcripts that includes GATA1. more...
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL24299
6 Samples
Download data: CEL
Series
Accession:
GSE108821
ID:
200108821
4.

mRNA length and 3’UTR structure govern the translation selectivity in RPS14-haploinsufficient erythroblasts [transcriptome]

(Submitter supplied) 5q- syndrome is a somatic ribosomopathy linked to the monoallelic deletion of the RPS14 gene and characterized by a proeminent erythroid phenotype. The mechanism of anemia involves an impaired differentiation and increased apoptosis of erythroblasts. Here we show that GATA1 protein expression is low in line with a defect in the representation of its mRNA at the ribosome. A global analysis of transcripts on polysomes indicates that translation is selective with a decreased representation of the transcripts with a short coding sequence and UTRs and a highly structured 3’UTR, a subset of transcripts that includes GATA1. more...
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL24299
9 Samples
Download data: CEL
Series
Accession:
GSE108820
ID:
200108820
5.

Genome-wide expression profiling of cells infected with control or RPS14 shRNAs

(Submitter supplied) We performed genome-wide expression profiling of cells infected with control or RPS14 shRNAs. Keywords: controls vs. shRNA treated
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL3921
24 Samples
Download data: CEL, EXP
Series
Accession:
GSE9487
ID:
200009487
6.

In vitro expansion of normal and Diamond Blackfan anemia-derived peripheral blood erythroid progenitors

(Submitter supplied) The Affymetrix Human Gene 2.0 ST array was used to measure differential expression of RNA isolated from normal and Diamond Blackfan anemia (DBA) erythroid progenitors after ex vivo expansion of circulating, peripheral blood derived hematopoietic stem cells under erythroid growth conditions. The gene-level probe summaries reported in this series were computed using RMA as implemented in the Bioconductor package Oligo v1.36.1. more...
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL16686
37 Samples
Download data: CEL
Series
Accession:
GSE89540
ID:
200089540
7.

5q- Myelodysplastic Syndrome Masquerading as Diamond Blackfan Anemia

(Submitter supplied) Diamond Blackfan anemia is a congenital bone marrow failure syndrome characterized by hypoproliferative anemia, often with associated physical abnormalities. Perturbations of the ribosome appear critically important to the development of DBA, as alterations in 9 different ribosomal protein genes have been identified in multiple unrelated families, along with rarer abnormalities of additional ribosomal proteins. more...
Organism:
Homo sapiens
Type:
SNP genotyping by SNP array; Expression profiling by array
4 related Platforms
14 Samples
Download data: CEL, TXT
Series
Accession:
GSE42570
ID:
200042570
8.

Consequences of Ribosomal Protein Haploinsufficiency in Human Hematopoiesis

(Submitter supplied) Ribosomal protein haploinsufficiency (RPH) underlies diverse human diseases with distinct and specific phenotypes, including Diamond-Blackfan anemia (DBA). Although multiple mechanisms have been proposed for the erythroid-specific hematopoietic defects observed in DBA, only recently has the role of selectively impaired translation been highlighted in these phenotypes. Exactly how and to what extent this impairment of translation occurs is currently unknown. more...
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing; Other
Platform:
GPL16791
14 Samples
Download data: TXT
9.

Heme and GATA1 coordinately regulate erythroid differentiation

(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.
Organism:
Mus musculus
Type:
Expression profiling by high throughput sequencing; Expression profiling by array
Platforms:
GPL19057 GPL6887
132 Samples
Download data
Series
Accession:
GSE94917
ID:
200094917
10.

Heme and GATA1 coordinately regulate erythroid differentiation [expression]

(Submitter supplied) In mice lacking the heme exporter, FLVCR, differentiation fails at the CFU-E/proerythroblast stage from excessive heme and reactive oxygen species. We show that Flvcr1-deleted CFU-E/proerythroblasts have low GATA1 mRNA and GATA1-target gene mRNAs along with increased ribosomal protein mRNAs as a direct result of increased heme. Thus heme increases ribosomal protein transcription when globin production needs to be increased, and when heme is excessive, GATA1 is reduced allowing for normal termination of erythroid differentiation. more...
Organism:
Mus musculus
Type:
Expression profiling by array
Platform:
GPL6887
5 Samples
Download data: TXT
Series
Accession:
GSE94905
ID:
200094905
11.

Heme and GATA1 coordinately regulate erythroid differentiation [RNA-seq]

(Submitter supplied) In mice lacking the heme exporter, FLVCR, differentiation fails at the CFU-E/proerythroblast stage from excessive heme and reactive oxygen species. We show that Flvcr1-deleted CFU-E/proerythroblasts have low GATA1 mRNA and GATA1-target gene mRNAs along with increased ribosomal protein mRNAs as a direct result of increased heme. Thus heme increases ribosomal protein transcription when globin production needs to be increased, and when heme is excessive, GATA1 is reduced allowing for normal termination of erythroid differentiation. more...
Organism:
Mus musculus
Type:
Expression profiling by high throughput sequencing
Platform:
GPL19057
127 Samples
Download data: TXT
Series
Accession:
GSE94898
ID:
200094898
12.

Ribosomal deficiencies in Diamond-Blackfan anemia impair translation of transcripts essential for differentiation of murine and human erythroblasts

(Submitter supplied) Diamond Blackfan Anemia (DBA) is associated with developmental defects and profound anemia. Mutations in genes encoding a ribosomal protein of the small (e.g. Rps19) or large (e.g. Rpl11) ribosomal subunit are found in over half of these patients. The mutations cause ribosomal haploinsufficiency, which reduces overall translation efficiency of cellular mRNAs. We reduced expression of *Rps19* or *Rpl11* in mouse erythroblasts and investigated mRNA polyribosome association, which revealed deregulated translation initiation of specific transcripts. more...
Organism:
Mus musculus
Type:
Expression profiling by array
Platform:
GPL1261
24 Samples
Download data: CEL, TXT
Series
Accession:
GSE22903
ID:
200022903
13.

Gene expression analysis from erythroid progenitors of patients with Diamond-Blackfan anemia

(Submitter supplied) Gene expression analysis from erythroid progenitors (CD34+/CD71(high)/CD45- mononuclear cells from the bone marrow) of patients with Diamond-Blackfan anemia (due to RPS19 mutations) and control individuals.
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL16212
9 Samples
Download data: CEL
Series
Accession:
GSE41817
ID:
200041817
14.

Murine Proerythroblasts (ProEs): WT vs. Bmi1-/-

(Submitter supplied) Transcriptional profiling of ProEs purified from wild type and Bmi1-/- mice.
Organism:
Mus musculus
Type:
Expression profiling by array
Platform:
GPL7042
3 Samples
Download data: TXT
Series
Accession:
GSE63413
ID:
200063413
15.

Murine Myeloid-Erythroid Progenitors (MEPs): WT vs. Bmi1-/-

(Submitter supplied) Transcriptional profiling of MEPs purified from wild type and Bmi1-/- mice.
Organism:
Mus musculus
Type:
Expression profiling by array
Platform:
GPL7042
3 Samples
Download data: TXT
Series
Accession:
GSE63411
ID:
200063411
16.

Defective ribosomal protein gene expression alters transcription, translation, apoptosis, and oncogenic pathways in Diamond-Blackfan anemia.

(Submitter supplied) Transcriptome profile of highly purified multipotential (P), erythroid (E), and myeloid (M) bone marrow progenitors from three RPS19 mutated Diamond-Blackfan anemia and six control human subjects.
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL96
27 Samples
Download data: CEL
Series
Accession:
GSE41599
ID:
200041599
17.

Normal and ineffective erythropoiesis scRNA seq

(Submitter supplied) Ineffective erythropoiesis, the death of maturing erythroid cells, is a common cause of anemia. To better understand why this occurs, we studied the fates and adaptations of single erythroid marrow cells from individuals with Diamond Blackfan anemia (DBA), del(5q) myelodysplastic syndrome (del(5q) MDS), and normal controls, and defined an unhealthy (vs. healthy) differentiation trajectory, using velocity pseudotime and cell surface protein assessment. more...
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing; Other
Platform:
GPL21697
37 Samples
Download data: H5AD
Series
Accession:
GSE222368
ID:
200222368
18.

AB001

(Submitter supplied) Diamond-Blackfan anemia (DBA) is a congenital disorder that results predominantly from mutations in various ribosomal protein genes. In order to determine how these mutations affect the translation of specific mRNAs, we performed microarray analysis of polysomal transcripts isolated from lymphoblast cells derived from DBA patients carrying different haploinsufficient mutations in either RPS19 or RPL11. more...
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL13183
18 Samples
Download data: TXT
Series
Accession:
GSE43117
ID:
200043117
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