Defective platelet autocrine signaling in HPS.
In this issue of Blood, Meng et al and Sharda et al use the Hermansky-Pudlak syndrome (HPS) as a model to show that adenosine 5′-diphosphate (ADP) released by dense granules serves as an autocrine signal to potentiate platelet release of α-granule and lysosome cargo and protein disulfide isomerase (PDI), all of which serve to stabilize thrombus formation.
UNIVERSITY OF ARKANSAS FOR MEDICAL SCIENCES.
Brian
http://orcid.org/0000-0002-4644-2504
B
Storrie
D000818:Animals; D001792:Blood Platelets / Q000201:enzymology* / Q000502:physiology*; D042783:Endothelial Cells / Q000201:enzymology*; D005260:Female; D022861:Hermanski-Pudlak Syndrome / Q000097:blood* / Q000201:enzymology*; D006801:Humans; D008297:Male; D019704:Protein Disulfide-Isomerases / Q000378:metabolism*; D013927:Thrombosis / Q000097:blood* / Q000201:enzymology*
D019704:Protein Disulfide-Isomerases