PADGEM (GMP140) is a component of Weibel-Palade bodies of human endothelial cells

PADGEM protein (PADGEM), also known as GMP140, is a platelet alpha- granule membrane protein that is translocated to the external membrane after platelet activation. Although the biosynthesis of this protein was originally thought to be confined to megakaryocytes, the synthesis of PADGEM in endothelial cells was recently demonstrated (McEver et al: Blood 70:1974a, 1987). We now describe the subcellular localization of this protein in endothelial cells. Immunofluorescence staining of permeabilized human umbilical vein endothelial cells with KC4, a well characterized monoclonal antibody to PADGEM, showed positively stained elongated structures similar in distribution and shape to Weibel-Palade bodies. Their identity as Weibel-Palade bodies was confirmed by double label immunofluorescence using KC4 and a polyclonal antiserum to von Willebrand factor (vWf), a protein known to be specifically stored in these organelles. All Weibel-Palade bodies were found to contain PADGEM. In contrast to strong perinuclear staining produced with anti- vWf antibodies, no significant perinuclear staining was obtained with KC4, indicating that relatively little PADGEM is present in the endoplasmic reticulum and in the Golgi apparatus. In endothelial cells treated with secretagogues that stimulate vWf release the elongated structures positive for PADGEM disappeared, further identifying these structures as Weibel-Palade bodies. This observation extends the parallels between Weibel-Palade bodies and alpha-granules and suggests a possible functional association between vWf and PADGEM.     

BUILDING THE FOUNDATION OF EXCELLENCE IN HEART FAILURE NURSING

This column contains the presidential address presented during the Third Annual Meeting of the American Association of Heart Failure Nurses on June 28, 2007, in San Diego, California, titled "Building the Foundation of Excellence in Heart Failure Nursing."     

HLA and Behçet's disease in Northern Spain: Their lack of correlation with arthritis pattern

Summary We have studied the characteristics of arthritis present in 32 patients with Behçet's disease (BD), and how this arthritis is related to the HLA markers class I. 84% of the patients presented arthritis, the most common being monoarthritis as the initial presentation, and oligoarthritis in subsequent episodes. In 63% of the cases, the development was in episodes of acute/subacute arthritis. We found statistically significant association between antigens B-5 and B-51, and the group with BD, with a relative risk of 3.89 and 4.71 respectively. The attempt to relate markers B-5, B-51 and B-27 to the presence of arthritis as well as to its manifestation and further development was not conclusive.     

Peripheral Seeding of Mycotic Aneurysms from an Infected Aortic Stent Graft

Aortic stent graft infection is rare and there are no reported cases of seeded peripheral mycotic aneurysms complicating this condition. We describe the case of a 54 year old man who developed a late stent graft infection at three years, resulting in the peripheral seeding of three mycotic aneurysms with two incidents of rupture. He was successfully treated with extra-anatomic bypass of the aorta and both surgical and endovascular repair of his peripherally seeded mycotic aneurysms.