Results 91 to 100 of about 721,729 (294)

Hereditary Angioedema due to C1-Inhibitor Deficiency in Pediatric Patients in Croatia – First National Study, Diagnostic and Prophylactic Challenges

open access: yesActa Clinica Croatica, 2019
Hereditary angioedema (HAE) is a rare autosomal dominant disease with deficiency (type I) or dysfunction (type II) of C1 inhibitor, caused by mutations in the C1-INH gene, characterized by recurrent submucosal or subcutaneous edemas including skin ...
Ljerka Karadža-Lapić   +7 more
doaj   +1 more source

Molecular Genetics of C1 Inhibitor

open access: yesImmunobiology, 1998
More than 100 different C1 inhibitor gene mutations have been described in hereditary angioedema (HAE) patients. Sixty-nine mutations have been reported in patients with the quantitative C1 inhibitor defect (type 1 HAE) in two recent large-scale studies. These changes were found distributed over all exons and exon/intron boundaries.
openaire   +2 more sources

Mixed‐Metal Promotion in a Manganese‐Molybdenum Oxynitride as Catalyst to Integrate C─C and C─N Coupling Reactions for the Direct Synthesis of Acetonitrile from Syngas and Ammonia

open access: yesAdvanced Materials, EarlyView.
Transition metal oxy/carbo‐nitrides show great promise as catalysts for sustainable processes. A Mn‐Mo mixed‐metal oxynitride attains remarkable performance for the direct synthesis of acetonitrile, an important commodity chemical, via sequential C─N and C─C coupling from syngas (C1) and ammonia (N1) feedstocks.
M. Elena Martínez‐Monje   +7 more
wiley   +1 more source

Dexime: A Selectively Enzyme‐Degradable Hydrogel for Protein Therapeutic Release

open access: yesAdvanced Materials, EarlyView.
A dextrin‐oxime hydrogel (dexime) is produced using ketone or aldehyde modified dextrin and tetra‐oxyamine modified poly(ethylene glycol). The rheological and mechanical properties of dexime are tunable. Dexime is injectable, cytocompatible, hydrolytically stable, and selectively degradable by α‐amylase.
Quinton E. A. Sirianni   +5 more
wiley   +1 more source

Effect of mutations within the coding region of C1-INH gene on protein function in families with HAE

open access: yes, 2005
C1 esterase inhibitor (C1-INH) is a serine protease inhibitor (serpin). Serpins are a superfamily of proteins that fold into a conserved structure and utilize a unique suicide substrate-like inhibitory mechanism.
M. Cicardi   +3 more
core  

Turning Unpredictable Biomolecule Adsorption to Controlled Corona Formation: Focus on Carbon Nanomaterials

open access: yesAdvanced Materials, EarlyView.
Controlling the protein corona formation onto carbon nanomaterials (CNMs) enhances their functionalities as platforms for cancer theranostics. Here, we reviewed the effects of the intrinsic and acquired properties of CNMs on protein corona formation, the consequent biological and toxicological outcomes, and the strategies to reshape corona formation ...
Yajuan Zou   +5 more
wiley   +1 more source

Clinical profile of patients with C1-inhibitor deficiency from Eastern India

open access: yesIndian Journal of Allergy Asthma and Immunology, 2016
C1-inhibtor deficiency or hereditary angioedema is a rare, autosomal dominant disorder that is characterized by severe episodic attacks of angioedema that can affect any part of the body.
Sujoy Khan
doaj   +1 more source

Self-administered C1 esterase inhibitor concentrates for the management of hereditary angioedema: usability and patient acceptance

open access: yes, 2016
Huamin Henry Li Institute for Asthma and Allergy, Chevy Chase, MD, USA Abstract: Hereditary angioedema (HAE) is a rare genetic disease characterized by episodic subcutaneous or submucosal swelling. The primary cause for the most common form of HAE is a
Li HH
core  

Plasma levels of C1- inhibitor complexes and cleaved C1- inhibitor in patients with hereditary angioneurotic edema [PDF]

open access: yes, 1990
C1- inhibitor (C1(-)-Inh) catabolism in plasma of patients with hereditary angioneurotic edema (HANE) was assessed by measuring the complexes formed by C1(-)-Inh with its target proteases (C1-s, Factor XIIa, and kallikrein) and a modified (cleaved ...
M Cugno   +13 more
core   +1 more source

Targeted Modulation of d‐Band Center in MoS2 Interlayer With n‐Type Co/Fe Dopants Accelerating Sulfur Reaction Kinetics in Lithium‐Sulfur Batteries

open access: yesAdvanced Materials, EarlyView.
A targeted modulation of the MoS2 electronic structure is achieved via substitutional n‐type Co/Fe co‐doping. This strategy triggers S‐mediated d‐p hybridization, optimizing the binding affinity with polysulfides to establish a volcano‐shaped relationship between the d‐band center and catalytic activity.
Junhyuk Ji   +6 more
wiley   +1 more source

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