Gretz, J.E., Norbury, C.C., Anderson, A.O., Proudfoot, A.E., and Arthur O. Anderson1 and Stephen Shaw2 Shaw, S. (2000). J. Exp. Med. 192, 1425–1440. Department of Clinical Pathology Hayakawa, M., Kobayashi, M., and Hoshino, T. (1988). Arch. Histol. US Army Medical Research Institute Cytol. 51, 233–240. of Infectious Diseases Ingulli, E., Ulman, D.R., Lucido, M.M., and Jenkins, M.K. (2002). J. F...

Rho GTPases are members of the Ras superfamily of GTPases that regulate a wide variety of cellular functions. While Rho GTPase pathways have been implicated in various pathological conditions in humans, to date coding mutations in only the hematopoietic specific GTPase, RAC2, have been found to cause a human disease, a severe phagocytic immunodeficiency characterized by life-threatening infecti...

The syndrome of leukocyte adhesion deficiency (LAD) combined with a severe Glanzmann-type bleeding disorder has been recognized as a separate disease entity. The variability in clinical and cell biological terms has remained largely unclear. We present data on 9 cases from 7 unrelated families, with 3 patients being actively followed for more than 12 years. The disease entity, designated LAD-1/...

A five-year case study of a child with leukocyte adhesion deficiency is presented. The report describes the generalized progressive periodontitis and intraoral infections associated with both the primary and permanent dentition. The associated therapeutic challenges and largely unsuccessful treatment regimens utilized in the attempts to arrest the periodontal disease are described.

BACKGROUND Ecthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme neutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients. Leukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of the innate immunity, is an autosomal recessive disorder characterized in its severe phenotype by a comp...

Abstract Background To investigate the microRNA (miRNA)-gene interactions underlying leukocyte functions and characteristics, especially potential serum biomarkers, implicated in traditional Chinese medicine (TCM)-defined Pi-qi-deficiency syndrome (PQDS) Pi-wei damp-heat (PDHS) resulting from chronic atrophic gastritis (CAG). Methods Using RNA/miRNA-sequencing approach, compared with healthy co...

INCE THE EARLY studies of Metchnikoff in the S 1880s,’ a large body of data has been accumulated that established a crucial role for circulating polymorphonuclear leukocytes (PMN) in host defense against pyogenic infections.’*2 These studies also amply documented a pathogenic role for these cells in host tissue damage in many noninfectious diseases.2 The same cellular processes underlie this co...

von Willebrand factor (VWF) levels are elevated and a disintegrin-like and metalloprotease with thrombospondin type I repeats-13 (ADAMTS13) activity is decreased in both acute and chronic inflammation. We hypothesized that by cleaving hyperactive ultralarge VWF (ULVWF) multimers, ADAMTS13 down-regulates both thrombosis and inflammation. Using intravital microscopy, we show that ADAMTS13 deficie...

Integrins are a widely expressed family of cell-matrix and cell-cell adhesion receptors that also have signalling functions. They are heterodimers consisting of non-covalently linked α and β subunits. There are 8 known β subunits and 14 known α subunits (Hynes, 1987, 1992). Many α subunits can associate with only a single β subunit, but recent results indicate that certain α subunits associate ...