In a recent study published in Nature Communications, researchers at the University of Virginia School of Medicine led by Shengyi Iris Sun, PhD, an associate professor in the Department of Pharmacology, uncovered how one of these quality control systems, called the ER-associated degradation (ERAD) pathway, helps regulate the production and breakdown of fibrinogen, a key protein involved in blood clotting. They identified the SEL1L-HRD1 complex within the ERAD pathway as crucial for preventing a rare liver disorder, known as hepatic fibrinogen storage disease (HFSD).
The researchers showed that when the SEL1L-HRD1 complex is deficient in liver cells, it leads to the formation of abnormal fibrinogen retention and deposits. They further revealed that the SEL1L-HRD1 ERAD complex mediates the breakdown misfolded fibrinogen protein components, allowing only correctly folded fibrinogen to form.
“Our findings highlight the importance of SEL1L-HRD1 in maintaining normal fibrinogen production,” said Dr. Sun. “By clearing misfolded fibrinogen, this complex supports the healthy formation and release of this essential protein.”
Read more about Dr. Sun’s research here, and at her lab website here.
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