Browse
Submit Data
Databases
API
Help

Dataset Information

11 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology.

ABSTRACT: Sickle cell disease (SCD) is a severe genetic blood disorder characterized by hemolytic anemia, episodic vaso-occlusion, and progressive organ damage. Current management of the disease remains symptomatic or preventative. Specific treatment targeting major complications such as vaso-occlusion is still lacking. Recent studies have identified various cellular and molecular factors that contribute to the pathophysiology of SCD. Here, we review the role of these elements and discuss the opportunities for therapeutic intervention.

SUBMITTER: Zhang D

PROVIDER: S-EPMC4760086 | biostudies-literature |

REPOSITORIES: biostudies-literature

ACCESS DATA

Json Xml

Similar Datasets

MicroRNA expression profile of platelets in sickle cell disease

Project description:Circulating platelets from Sickle cell disease (SCD) patients express distinct gene expression patterns that regulate function. The objective of this study is to identify a role of post-transcriptional regulation of the platelet transcriptional signaling by microRNAs. Comparison of microRNA expression in platelets from SCD patients and control subjects, from 2 cohorts-University of Pittsburgh and National Institutes of Health.

2013-06-20 | E-GEOD-41574 | biostudies-arrayexpress

MicroRNA expression profile of platelets in sickle cell disease

2013-06-20 | GSE41574 | GEO

The proteome of neutrophils in sickle cell disease

Project description:Polymorphonuclear neutrophils are key actors in the pathophysiology of sickle cell disease, but specific factors underlying their activation and sustained inflammation are not well documented. In the present study, we investigated the proteome of neutrophils by a label-free global comparative approach between 4 non-treated sickle patients (SS genotype) at steady state and 4 healthy donors. We identified 101 proteins differentially expressed in SS and normal neutrophils. We found overexpression of CD64 and under-expression of CD62L suggestive of an activated and aged neutrophil profile in SS patients. Comparison of the two proteomes revealed a strong involvement of the type 1 interferon (IFN) response pathway with a 3- to 84-fold increase of type 1 IFN-induced proteins in SS neutrophils, and overexpression of STAT1 and STAT2. Thus, we next determined the plasmatic concentration of type 1 IFNs (IFNα and IFNβ) using the digital-ELISA technology and found a significant higher concentration of IFNα in the plasma from half of our SS patients compared to controls. Overall, a dramatic high-level expression of IFNα signaling proteins in neutrophils from SS patients suggests auto-inflammatory-like phenotype in sickle cell disease at steady state. This finding could open the way to new anti-inflammatory therapies.

2020-11-11 | PXD014457 | Pride

Sickle Cell Disease-Genetics, Pathophysiology, Clinical Presentation and Treatment.

Project description: Not available

| S-EPMC7510211 | biostudies-literature

HbS Binding to GP1b? Activates Platelets in Sickle Cell Disease.

Project description: Not available

| S-EPMC5148012 | biostudies-literature

Targeting sickle cell disease root-cause pathophysiology with small molecules.

Project description: Not available

| S-EPMC6717594 | biostudies-literature

Pathophysiology and treatment of pulmonary hypertension in sickle cell disease.

Project description: Not available