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Researcher Investigates An Incurable Blood Disorder Often Diagnosed In Children

Treating a persistent blood disease that affects children can result in an adjustment in energy production in stem cells, suggests research from the University of West Virginia.

Wei Du, an assistant professor at the Faculty of Pharmacy, studies the relationship between stem cell energy production and the development of Fanconi anemia. The disease prevents the bone marrow from producing the blood cells needed to fight disease, bleeding, and oxygen transport. This also makes it harder to repair damaged DNA.

“Almost every child with Fanconi’s anemia will eventually develop leukemia,” said Du, who co-directs the Alexander B. Osborn Hematopoietic Malignant Transplantation Program at the WVU Cancer Institute. According to St. Jude Children’s Research Hospital, the average life expectancy of people with the disease is between 20 and 30 years.

Du and his research team have identified that in animal models of Fanconi anemia, stem cells tend to employ aerobic or oxygen-based processes to generate energy. This is strange; usually, stem cells in blood and bone marrow prefer an anaerobic method which is not based on oxygen.

This metabolic distinction suggests that the various symptoms of Fanconi’s anemia, such as tiredness, shortness of breath, frequent bruising, and bleeding may depend on the production of energy at the cellular level.

The researchers identified a specific signal pathway, called the TIGAR p53 axis, which was overexpressed in anemic models.

The current standard of care for Fanconi’s anemia includes bone marrow transplantation, but, as Du explained, it works less than a third of the time. “In people with Fanconi anemia, blood cells are not the only ones with a mutation,” she said. “The same applies to other cells in other parts of the body that promote the survival of stem cells.” For this reason, the patient’s body allows the replication of healthy transplanted cells and their anemia persists.

Susan Wallace
Susan Wallace Subscriber
JR. CONTENT WRITER At Global Industry News

With a degree in Medical Scientists, Susan Wallace serves as a Junior content writer and is naïve to the world of writing. She is given the accountability to write blogs and reports about the Health field. It comprises vaccination reports & new drug discoveries, invention & innovation, clinical trials, drug approvals, and much more. Susan, in spare time, helps NGOs in her nearby area to give the kids free study-related tutorials and other assistance.

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