Hemin and sickle cell disease-associated acute chest syndrome development

October 1, 2013, Journal of Clinical Investigation

Acute chest syndrome (ACS) is a complication of sickle cell disease that is characterized by sudden pain and difficulty breathing. Sickle cell disease can also cause red blood cells to suddenly breakdown and release their contents, which may trigger the onset of ACS.

In this issue of the Journal of Clinical Investigation, Solomon Fiifi Ofori-Acquah and colleagues at Emory University asked if hemin, a product released by during lysis, triggers ACS in a mouse model of .

They found that hemin injection caused labored breathing, and rapid death in sickle mice, but not in control mice.

They determined that hemin acts through the immune receptor TLR4, since blocking or deleting this receptor prevented symptoms of hemin injection in sickle mice.

Hemopexin, a protein that sequesters hemin was an effective treatment against hemin-induced lung injury both before and after symptom onset.

These data suggest that therapies aimed at targeting hemin and/or blocking TLR4 signaling may be valuable approaches for treatment or prevention of acute chest syndrome.

Explore further: Arginine therapy shows promise for sickle cell pain

More information: Extracellular hemin crisis triggers acute chest syndrome in sickle mice, J Clin Invest. DOI: 10.1172/JCI64578

Related Stories

Arginine therapy shows promise for sickle cell pain

September 16, 2013
Arginine therapy may be a safe and inexpensive treatment for acute pain episodes in patients with sickle cell disease, according to results of a recent clinical study. The study was the first randomized placebo-controlled ...

Sickle cell disease, sickle cell trait are not the same

December 28, 2012
(HealthDay)—Both sickle cell disease and the condition known as sickle cell trait are genetic blood diseases: You're born with one or the other because of the genes inherited from your parents. Beyond that, the two conditions ...

UCLA stem cell gene therapy for sickle cell disease advances toward clinical trials

July 1, 2013
Researchers at UCLA's Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research have successfully established the foundation for using hematopoietic (blood-producing) stem cells from the bone marrow of patients ...

Beyond the little blue pill: Scientists develop compound that may treat priapism

September 30, 2013
It's not the little blue pill famous for helping men get big results, but for those who need it, the outcome might be even more significant. A new research report published online in The FASEB Journal, offers hope to men ...

Research leads to possible new treatment for sickle cell disease

September 23, 2013
There's a new ray of hope for those battling sickle cell disease thanks to a compound that was discovered at Virginia Commonwealth University.

Recommended for you

Green leafy vegetables may prevent liver steatosis

December 17, 2018
A larger portion of green leafy vegetables in the diet may reduce the risk of developing liver steatosis, or fatty liver. In a study published in PNAS researchers from Karolinska Institutet in Sweden show how a larger intake ...

Discovery of novel mechanisms that cause migraines

December 17, 2018
Researchers at CNRS, Université Côte d'Azur and Inserm have demonstrated a new mechanism related to the onset of migraine. They found how a mutation that causes dysfunction in a protein which inhibits neuronal electrical ...

Small-scale poultry farming could mean big problem in developing countries

December 16, 2018
Small-scale farming in developing countries provides those in rural communities with income and access to protein, but it may have a large impact on antibiotic resistance, according to a new University of Michigan study.

RNA processing and antiviral immunity

December 14, 2018
The RIG-I like receptors (RLRs) are intracellular enzyme sentries that detect viral infection and initiate a first line of antiviral defense. The cellular molecules that activate RLRs in vivo are not clear.

Faster test for Ebola shows promising results in field trials

December 13, 2018
A team of researchers with members from the U.S., Senegal and Guinea, in cooperation with Becton, Dickinson and Company (BD), has developed a faster test for the Ebola virus than those currently in use. In their paper published ...

Urbanisation and air travel leading to growing risk of pandemic

December 13, 2018
Increased arrivals by air and urbanisation are the two main factors leading to a growing vulnerability to pandemics in our cities, a University of Sydney research team has found.

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.