Hereditary angioedema (HAE) is a rare autosomal dominant genetic disease characterized by repetitive subcutaneous or submucosal angioedema, activation of the kinin system, and increased vascular permeability.
Yinshi Guo +6 more
doaj +1 more source
Hyperosmotic stress induces PARP1‐mediated HPF1‐dependent mono(ADP‐ribosyl)ation
Sorbitol‐induced hyperosmotic stress rapidly induces reversible mono(ADP‐ribosyl)ation (MARylation) on PARP1 without the signs of genotoxic signaling. We show that PARP1 autoMARylation is HPF1 dependent and forms hydroxylamine‐resistant O‐glycosidic linkages.
Anna Georgina Kopasz +11 more
wiley +1 more source
The ubiquitin‐proteasome system and autophagy as guardians of the cellular proteome
This Perspective covers the three principles governing the crosstalk between the ubiquitin‐proteasome system and autophagy in cellular proteostasis: (1) a shared ubiquitin code routing substrates via shuttle factors or autophagy receptors; (2) spatial compartmentalization into phase‐separated degradation hubs and organelle‐specific modules (exemplified
Ivan Dikic
wiley +1 more source
C1 Inhibitor Deficiency and Angioedema of the Small Intestine Masquerading as Crohn’s Disease
A case of C1 inhibitor deficiency presenting as localized edema of the small intestine is described. A 16-year-old, previously healthy woman presented with recurrent attacks of abdominal pain and vomiting following minor abdominal trauma.
Kelly W Burak, Gary R May
doaj +1 more source
Serial change of C1 inhibitor in patients with sepsis: a prospective observational study
Background C1 inhibitor (C1-INH), which belongs to the superfamily of serine protease inhibitors, regulates the complement system and also the plasma kallikrein-kinin, fibrinolytic, and coagulation systems.
Tomoya Hirose +7 more
doaj +1 more source
Electron transfer between complexes III and IV in S. cerevisiae mitochondrial membranes
Mitochondrial oxidative phosphorylation in S. cerevisiae mitoplasts is limited by complex IV catalytic capacity, rather than two‐dimensional cytochrome c diffusion. At physiological cytochrome c : supercomplex ratios at salinity equivalent to that of 20 mm monovalent salt, activity is maximized, indicating that this low ionic strength accurately mimics
Ana Paula Lobez +2 more
wiley +1 more source
Presence of C1-inhibitor polymers in a subset of patients suffering from hereditary angioedema. [PDF]
Hereditary angioedema (HAE) is a potentially life-threatening disease caused by mutations in the gene encoding the serine protease inhibitor (serpin) C1 inhibitor (C1-inh).
Daniel Elenius Madsen +5 more
doaj +1 more source
C1-esterase inhibitor reduces reperfusion injury after lung transplantation
BACKGROUND: Activation of the complement system and polymorphonuclear neutrophilic leukocytes plays a major role in mediating reperfusion injury after lung transplantation.
Schäfers, Hans-Joachim +9 more
core +1 more source
Structural insights and therapeutic targets in Acinetobacter baumannii capsule biosynthesis
Hypervirulent KL49 A. baumannii's capsular polysaccharide contains the nonulosonic acid 8‐epi‐Leg5,7Ac2, synthesized by epimerization via ElaA, ElaB, and ElaC. Crystal structures of ElaA, ElaB, and ElaC reveal their role in CMP‐Leg5,7Ac2 synthesis and regioselective C8 epimerization.
Woo Cheol Lee +7 more
wiley +1 more source
Effects of weak anchoring on C1 and C2 chevron structures
We present a theoretical study of the effect of weak anchoring on the transition between C1 and C2 chevron structures in smectic C liquid crystals. We employ a continuum theory which allows for variable cone, azimuthal and layer tilt angles.
Mottram, Nigel +4 more
core +1 more source

