Figure 1.
Pathophysiology underlying low VWF levels. Hepatic macrophages, liver sinusoidal ECs (LSECs), and hepatocytes contribute to regulating VWF clearance. A number of cell surface receptors have also been described. On LSECs these include stabilin-2 (STAB2) and C-type lectin domain family 4 member M (CLEC4M). Macrophage receptors include Siglec-5, the low-density lipoprotein receptor-related protein-1 (LRP1), the scavenger receptor class A member I (SR-A1), and macrophage galactose-type lectin (MGL). Finally, the asialoglycoprotein receptor (ASGPR) on hepatocytes has also been implicated. PTM, posttranslational modification.