Blood: The interplay of the endogenous mesh protein SEC22B and NBEAL2 regulates the formation of alpha particles of macronuclear cells

Gray platelet syndrome,GPS, also known as alpha storage pool disease, is a rare haemorrhagic disease of autosomal recessive genetics, characterized by mild platelet reduction accompanied by large platelets, plateletg glycoproteins are normal, but platelet alpha particles and the proteins they contain are reduced or missingthe study of hereditary platelet disease provides many insights into the development and function of plateletsThe loss of NBEAL2 (neurobeachin-like 2) function can lead to gray platelet syndrome, and the absence of platelet alpha particles indicates that precursor macronuclear cells require NBEAL2 to produce plateletsendoscoscous is a dynamic network that interacts with a large number of intracellular bubbles and organelles and plays a key role in their developmentThe endothelial network of macronuclear cells is very wide, and in this study, researchers explored their role in the occurrence of alpha-particle organisms by focusing on the transporter protein SEC22B on the cell membraneimmunocoprecipitation experiments of marker proteins expressed with giant nuclear cells imMKCL showed that NBEAL2 can bind to SEC22B and NBEAL2 can bind both SEC22B and P-selectorsThe autonuclear endogenous protein and NBEAL2-SEC22B binding were also observed through immunocostosis cocipitated, and a large amount of overlap was detected by the immunofluoriating microscopeSEC22B is positioned in a region of NBEAL2 that spans 1798-1903 amino acids, and two GPS-related fault mutations have been found in the region: E1833K and R1839COnce NBEAL2 has occurred in either of these two mutations, it can no longer bind to SEC22B, which is expressed in the HEK292 cellsCRISPR/Cas9-mediated knockout of the SEC22B of imMKCL cells results in lower NBEAL2 levels and vice versaSEC22B or NBEAL2 expression deficiency can also lead to the failure of alpha particle production and the reduction of granulocyt protein, SEC22B is essential for the occurrence of alpha particle organisms of macronucleocyTes, and interactions with SEC22B and P-selectin promote the important role of NbEAL2 in particle development and protein reprinting stability