Stories about: microfluidics

A “CRISPR” view of Sturge-Weber syndrome is coming into focus

Colorized laboratory image showing tissue model containing the Sturge-Weber syndrome genetic mutation
Living blood vessels inside a microfluidic chip containing the genetic mutation (green) responsible for Sturge-Weber syndrome (Credit: Bischoff lab)

Three-dimensional modeling and CRISPR-Cas9 gene editing technology are giving scientists a new view into Sturge-Weber syndrome, a rare congenital disorder that causes small blood vessels, called capillaries, to be malformed. These capillary malformations can cause port wine birthmarks on the face and neck, and in some cases, abnormal vasculature in the brain that can spark seizures.

Last year, a Boston Children’s Hospital research team — led by Joyce Bischoff, PhD, of the Vascular Biology Programdiscovered that the genetic mutation responsible for Sturge-Weber syndrome dwells in endothelial cells lining the affected capillaries in the brain. The team had previously found the same mutation present in the endothelial cells of skin capillaries of patients’ port wine birthmarks.

Together, their studies suggest that mutated endothelial cells could be causing surrounding cells to behave abnormally.

To explore this emerging hypothesis, Bischoff’s team is seeking lifelike ways of mimicking these hallmark capillary malformations in the laboratory.

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Mimicking milieus to produce platelets…for science and transfusion

Researchers and doctors dream of being able to artificially produce platelets (in the blood bag above) at clinically useful scales. A device that mimics the environments in which platelets mature could help them get there. (Toytoy/Wikimedia Commons)

The platelet – a crucial cog in our blood’s clotting machinery – is in high demand. Trauma, chemotherapy, and surgery patients often need platelet transfusions to keep their blood working properly. So too do people with genetic disorders like Wiskott-Aldrich syndrome that prevent them from producing enough platelets on their own and cause thrombocytopenia.

However, platelets are in short supply compared to other blood products, in part due to their short shelf life.

“Platelets only last in the body for about 10 days at a time,” explains Jonathan Thon, a fellow in the laboratory of Joe Italiano, a member of Children’s Vascular Biology Program. “In a blood bank, red blood cells can be stored in a refrigerator for 42 days, and plasma can be frozen for years. But platelets need to be stored at room temperature, and only for a short time for fear of bacterial contamination.” Which means that few platelets are available for those who need them – a situation that screams for a means of artificial platelet production.

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