PMID- 11012220 OWN - NLM STAT- MEDLINE DCOM- 20001102 LR - 20081121 IS - 1066-5099 (Print) IS - 1066-5099 (Linking) VI - 14 Suppl 1 DP - 1996 TI - The platelet glycoprotein Ib-V-IX system: regulation of gene expression. PG - 188-93 AB - Platelet glycoproteins (GPs) Ib-V-IX form the surface receptor for von Willebrand factor, and this receptor-ligand interaction mediates the shear-dependent adhesion of platelets to damaged arterial vessel walls. The receptor is a multicomponent structure consisting of four distinct polypeptides (heterodimeric GPIb: Ib alpha-Mr143k and Ib beta-Mr22k; GPV-Mr83k; GPIX-Mr20k), and each of the four cDNAs and genes has been cloned and characterized. The genes appear to have evolved from a common progenitor genomic sequence related to that encoding GPIX. They share simple structures with few introns and possess common consensus regulatory sequences (GATA, ets, Sp-1) in their 5' flanks. Both the GPIb alpha and the GPIX promoters have been analyzed by transfection of reporter constructs into hematopoietic and nonhematopoietic cells. The promoters function in a tissue-specific fashion, and gel shift and mutational analyses indicate that GATA and ets sequences regulate activity. In the case of the GPIX promoter, footprints confirm the role of the ets-related consensus region. Recent studies of GPIb beta transcriptional regulation suggest that an aberrant polyadenylation signal, located in the 3' end of the gene immediately upstream of the GPIb beta gene, allows in vitro expression of a rare extended fusion transcript encoding both the upstream protein and GPIb beta. Little detailed information is available in regard to expression of the GPV gene. In summary, the genes of the GPIb-V-IX system display features of other megakaryocyte/platelet genes, but the unique regulatory events that direct the selective expression of these genes in megakaryocytes remain to be defined. FAU - Roth, G J AU - Roth GJ AD - Hematology Section, Medical and Research Services, Seattle Veterans Administration Medical Center and the University of Washington, 98108, USA. FAU - Yagi, M AU - Yagi M FAU - Bastian, L S AU - Bastian LS LA - eng GR - HL 09265/HL/NHLBI NIH HHS/United States GR - HL 39947/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, U.S. Gov't, Non-P.H.S. PT - Research Support, U.S. Gov't, P.H.S. PT - Review PL - England TA - Stem Cells JT - Stem cells (Dayton, Ohio) JID - 9304532 RN - 0 (DNA, Complementary) RN - 0 (Platelet Glycoprotein GPIb-IX Complex) RN - 0 (Recombinant Fusion Proteins) RN - 24937-83-5 (Poly A) SB - IM MH - Chromosome Mapping MH - DNA Mutational Analysis MH - DNA, Complementary/metabolism MH - Endothelium, Vascular/metabolism MH - *Gene Expression Regulation MH - Genes, Reporter MH - Hematopoietic Stem Cells/metabolism MH - Humans MH - Introns MH - Models, Biological MH - Platelet Aggregation/*genetics MH - Platelet Glycoprotein GPIb-IX Complex/chemistry/*genetics/*metabolism MH - Poly A/metabolism MH - Promoter Regions, Genetic MH - Recombinant Fusion Proteins/metabolism MH - Tissue Distribution MH - Transcription, Genetic MH - Transfection RF - 36 EDAT- 1996/01/01 00:00 MHDA- 2001/02/28 10:01 CRDT- 1996/01/01 00:00 PHST- 1996/01/01 00:00 [pubmed] PHST- 2001/02/28 10:01 [medline] PHST- 1996/01/01 00:00 [entrez] AID - 10.1002/stem.5530140724 [doi] PST - ppublish SO - Stem Cells. 1996;14 Suppl 1:188-93. doi: 10.1002/stem.5530140724.