title: Induction of specific storage organelles by von Willebrand factor propolypeptide.
abstract: Endothelial cells store the multimeric adhesive glycoprotein von Willebrand factor (vWf), which promotes the formation of a platelet plug at the site of vessel injury. To investigate the packaging of vWf into the granules called Weibel-Palade bodies, we expressed pro-vWf cDNA and cDNA lacking the prosequence in a variety of cell lines. Storage granules formed only in cells that contain a regulated pathway of secretion. Furthermore, packaging required the prosequence. Pro-vWf, lacking the C-terminal region involved in interchain disulfide bonding, formed granules. We conclude that the signal for storage is universal in that an adhesive glycoprotein can be stored by a hormone-secreting cell; the storage of vWf is independent of its covalent multimeric structure; the unusual rod shape of Weibel-Palade bodies is due to vWf; and the vWf propolypeptide is necessary for the formation of vWf storage granules.
authors.affiliation: New England Medical Center, Boston, Massachusetts.
authors.forename: D D
authors.initials: DD
authors.lastname: Wagner
authors.forename: S
authors.initials: S
authors.lastname: Saffaripour
authors.forename: R
authors.initials: R
authors.lastname: Bonfanti
authors.forename: J E
authors.initials: JE
authors.lastname: Sadler
authors.forename: E M
authors.initials: EM
authors.lastname: Cramer
authors.forename: B
authors.initials: B
authors.lastname: Chapman
authors.forename: T N
authors.initials: TN
authors.lastname: Mayadas
mesh_terms: D000818:Animals; D015153:Blotting, Western; D002460:Cell Line; D004247:DNA / Q000235:genetics; D005455:Fluorescent Antibody Technique; D006801:Humans; D008854:Microscopy, Electron; D015388:Organelles / Q000378:metabolism* / Q000648:ultrastructure; D010957:Plasmids; D011498:Protein Precursors / Q000235:genetics*; D011499:Protein Processing, Post-Translational; D015183:Restriction Mapping; D014162:Transfection; D014841:von Willebrand Factor / Q000235:genetics* / Q000302:isolation & purification
chemical_list: D011498:Protein Precursors; D014841:von Willebrand Factor; C067003:von Willebrand factor propolypeptide; D004247:DNA