Biomedical subjects

Deborah Brown

Publications and source records attributed to Deborah Brown.

3 recordsLinked to original sources

PubMed2006

Molecular and clinical spectrum of type I plasminogen deficiency: A series of 50 patients.

Severe type I plasminogen (PLG) deficiency has been causally linked to a rare chronic inflammatory disease of the mucous membranes that may be life threatening. Here we report clinical manifestations, PLG plasma levels, and molecular genetic status of the PLG gene of 50 patients. The most common clinical manifestations among these patients were ligneous conjunctivitis (80%) and ligneous gingivitis (34%), followed by less common manifestations such as ligneous vaginitis (8%), and involvement of the respiratory tract (16%), the ears (14%), or the gastrointestinal tract (2%). Four patients showed congenital occlusive hydrocephalus, 2 with Dandy-Walker malformation of cerebellum. Venous thrombosis was not observed. In all patients, plasma PLG levels were markedly reduced. In 38 patients, distinct mutations in the PLG gene were identified. The most common genetic alteration was a K19E mutation found in 34% of patients. Transient in vitro expression of PLG mutants R134K, delK212, R216H, P285T, P285A, T319_N320insN, and R776H in transfected COS-7 cells revealed significantly impaired secretion and increased degradation of PLG. These results demonstrate impaired secretion of mutant PLG proteins as a common molecular pathomechanism in type I PLG deficiency.

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PubMed2003

Graduate health professions education: an interdisciplinary university - community partnership model 1996 - 2001.

INTRODUCTION: In 1996, East Tennessee State University (ETSU) reinforced its historical commitment to multidisciplinary community engagement by developing a graduate level community partnerships program in the Division of Health Sciences. While the university's earlier health partnership efforts relied primarily on curricular innovation, the approach to graduate health professions education was to seed a series of curricular enhancements and interdisciplinary, community-based learning experiences and service into traditional curricula. This paper presents the experience of one school in crafting a regional network that became the basis of a division-wide graduate level teaching and learning initiative. INNOVATIONS AND EVALUATION: Carefully selected planning and implementation techniques enabled multidisciplinary practitioners and community members from across a 20-county region to participate with university faculty in training ETSU learners in community-based medical care. By year four of the project, curricular "enhancements" were institutionalized in over five departments across the Division and engaged 1160 medical residents and graduate learners in a give - get model of health education. Programme evaluation methodology was collaboratively defined and documentation of programme effort and outcomes regularly reported and strategically reviewed. CONCLUSIONS: Programme evaluation demonstrates mutual benefit to community and university. Faculty involvement in programme activity increased fourfold and community involvement in training of health professions graduate learners increased threefold by year four. Educational innovations were adopted into traditional curricula, thousands of hours of clinical services were provided to underserved communities and the university-community team forged by network links continues to promote multidisciplinary interests through joint public policy endeavors.

Appalachian Region↗

PubMed2002

Structure and function of membrane rafts.

Lipids do not always mix uniformly in membranes, but can cluster to form microdomains. We will consider one type of microdomain that can form in cell membranes. These are enriched in cholesterol and sphingolipids, and are referred to as rafts. Rafts probably exist in membranes in the liquid-ordered phase or a phase with similar properties. We will briefly review membrane lipid phase behavior, and the differences between liquid-crystalline, liquid-ordered, and gel-phase membrane bilayer domains. We will present evidence suggesting that phospholipid-rich, liquid-crystalline phase domains and sphingolipid-rich, liquid-ordered phase domains (rafts) can exist in equilibrium in biological membranes, especially the plasma membrane. Preferential partitioning of membrane proteins into rafts can affect function. Among the proteins that are targeted to rafts are those anchored in the outer leaflet of the membrane through covalent attachment to a special glycolipid, glycosyl phosphatidylinositol (GPI). Other proteins that are linked to saturated acyl chains, such as those that are directly acylated with two or more palmitate chains, or a palmitate and a myristate chain, are also targeted to rafts. Targeting of GPI-anchored proteins and other proteins to rafts plays a role in signal transduction in hematopoietic cells, and possibly also in sorting in intracellular membranes and regulation of cell-surface proteolysis in other mammalian cells.

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