Results 211 to 220 of about 1,197,187 (269)

Chemoenzymatic Radiosynthesis of a Gluconate Transporter‐Targeted In Vivo Bacterial Sensor From Clinical [18F]FDG

Angewandte Chemie, EarlyView.
An efficient radiosynthesis of fluorine‐18 labeled gluconic acid ([18F]FGA) was developed using two readily available materials, 2‐deoxy‐2‐[18F]fluoro‐D‐glucose ([18F]FDG) and glucose oxidase. [18F]FGA is highly specific for bacterial gluconate metabolism mediated by gluconate permease (GntP) and gluconate kinase (GntK), with rapid clearance, low off ...
Sang Hee Lee, Jung Min Kim, Joseph Blecha, Robert R. Flavell, Michael A. Ohliger, Joanne Engel, Youngho Seo, David M. Wilson   +7 more
wiley   +1 more source

Unraveling A4GALT Mechanism and Its Modulation With Adamantyl‐Galactosylceramide Analogues: Advancing Fabry Disease Therapeutic Strategies

Angewandte Chemie, EarlyView.
A 310‐helix‐mediated conformational switch promotes a front‐face SNi‐like catalysis by human A4GALT. Mechanism‐guided design identifies AdaGalCer as a selective modulator of globotriaosylceramide (Gb3) biosynthesis, opening a clear route toward new Fabry disease therapeutics.
Nicky de Koster, Òscar Vidal‐Gironès, Rowan de Graaf, Ken Kok, Lindsey T. Lelieveld, Jackelien Hoogervorst, Maria J. Ferraz, José Pablo Rivas‐Fernández, Rob F. Lammers, Herman S. Overkleeft, Johannes M. F. G. Aerts, Rolf G. Boot, Carme Rovira, Marta Artola   +13 more
wiley   +1 more source

Highlights from the 7th C1 Inhibitor Deficiency Workshop, Budapest, Hungary

, 2011
Späth, P. J.
core  

C1-inhibitor deficiency and angioedema

Molecular Immunology, 2001
C1-inhibitor deficiency can be inherited or acquired; both conditions lead to recurrent angioedema that can be life threatening when the larynx is involved (hereditary angioedema, HAE; acquired angioedema, AAE). The genetic defect is due to the heterozygous deficiency of C1-Inh that is transmitted as an autosomal dominant trait.
A. Carugati, E. Pappalardo, L.C. Zingale, M. Cicardi   +3 more
openaire   +4 more sources

Monocyte C1‐inhibitor synthesis in patients with C1‐inhibitor deficiency

European Journal of Clinical Investigation, 1989
Abstract. Monocytes of seven out of eight patients with type 1 C1‐inhibitor (C1‐inh) deficiency (HAE) produced 40% as much C1‐inh as monocytes from normal donors (controls). In contrast, monocytes from three patients with type 2 and three patients with acquired C1‐inh deficiency produced similar amounts of Cl‐inh as controls. Recombinant γ‐interferon (
D F, Lappin, A R, McPhaden, P L, Yap, P E, Carter, G D, Birnie, J E, Fothergill, K, Whaley   +6 more
openaire   +3 more sources

A National Survey of Hereditary Angioedema and Acquired C1 Inhibitor Deficiency in the United Kingdom [PDF]

Journal of Allergy and Clinical Immunology: in Practice, 2023
BackgroundDetailed demographic data on people with hereditary angioedema and acquired C1 inhibitor deficiency in the UK are relatively limited. Better demographic data would be beneficial in planning service provision, identifying areas of improvement ...
Patrick F K Yong, Alex Richter, Catherine Stroud   +2 more
exaly   +13 more sources

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Deficiencies of C1 inhibitor

Best Practice & Research Clinical Gastroenterology, 2005
Hereditary and acquired deficiencies of the C1 inhibitor result in a single prominent symptom, namely angioedema. Angioedema may involve the skin, the gastrointestinal tract or the upper airway. Genetically determined defects in C1INH cause hereditary angioedema.
Fred S, Rosen, Alvin E, Davis
openaire   +2 more sources

Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency

Journal of Allergy and Clinical Immunology, 2006
BACKGROUND: Administration of C1-inhibitor concentrate is effective for prophylaxis and treatment of severe angioedema attacks caused by C1-inhibitor deficiency.
G Choi
exaly   +2 more sources

C1 inhibitor deficiency: management

Clinical and Experimental Dermatology, 2005
This is the second of two articles on C1 inhibitor deficiency based on a recent UK consensus document covering its diagnosis and management in adults and children. This summary focuses on the management of the disorder including prophylaxis, emergency treatment and special situations such as pregnancy and dental care.
M M, Gompels, R J, Lock
openaire   +2 more sources

Acquired C1 Inhibitor Deficiency

Immunology and Allergy Clinics of North America, 2017
Acquired angioedema due to C1-INH deficiency (C1-INH-AAE) can occur when there are acquired (not inherited) deficiencies of C1-INH. A quantitative or functional C1-INH deficiency with negative family history and low C1q is diagnostic of C1-INH-AAE. The most common conditions associated with C1-INH-AAE are autoimmunity and B-cell lymphoproliferative ...
Iris M, Otani, Aleena, Banerji
openaire   +2 more sources