Thromboembolic disease due to thermolabile conformational changes of antithrombin Rouen-VI (187 Asn-->Asp)
about
Uptake of the necrotic serpin in Drosophila melanogaster via the lipophorin receptor-1Identification and analysis of serpin-family genes by homology and synteny across the 12 sequenced Drosophilid genomesProbing serpin reactive-loop conformations by proteolytic cleavageSmall molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregatesImportance of the release of strand 1C to the polymerization mechanism of inhibitory serpinsAn overview of the serpin superfamilySerpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerolMolecular mechanism of a temperature-sensitive phenotype in peroxisomal biogenesis disorderLatent S49P neuroserpin forms polymers in the dementia familial encephalopathy with neuroserpin inclusion bodiesPolymerization of plasminogen activator inhibitor-1.Familial conformational diseases and dementias.Hot news: temperature-sensitive humans explain hereditary disease.The necrotic gene in Drosophila corresponds to one of a cluster of three serpin transcripts mapping at 43A1.2.Blocking formation of large protein aggregates by small peptides.Alpha1-antitrypsin deficiency. 4: Molecular pathophysiologySerpin alpha 1proteinase inhibitor probed by intrinsic tryptophan fluorescence spectroscopyHepatic fibrosis and carcinogenesis in α1-antitrypsin deficiency: a prototype for chronic tissue damage in gain-of-function disorders.Unravelling the twists and turns of the serpinopathies.Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency.Heparin-dependent modification of the reactive center arginine of antithrombin and consequent increase in heparin binding affinity.The functional integrity of the serpin domain of C1-inhibitor depends on the unique N-terminal domain, as revealed by a pathological mutant.Conformational preludes to the latency transition in PAI-1 as determined by atomistic computer simulations and hydrogen/deuterium-exchange mass spectrometry.Polymerization of human angiotensinogen: insights into its structural mechanism and functional significanceL-asparaginase-induced antithrombin type I deficiency: implications for conformational diseases.Thermal instability of compound variants of carnitine palmitoyltransferase II and impaired mitochondrial fuel utilization in influenza-associated encephalopathy.Studies of serpins unfold at a feverish pace.Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis.The biostructural pathology of the serpins: critical function of sheet opening mechanism.Structural modifications of proteins during aging.Genetics and respiratory disease. 2. Alpha 1-antitrypsin deficiency, cirrhosis and emphysema.A hydrophobic patch surrounding Trp154 in human neuroserpin controls the helix F dynamics with implications in inhibition and aggregation.Structural dynamics associated with intermediate formation in an archetypal conformational disease.Probing the local conformational change of alpha1-antitrypsin.6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis.The selective inhibition of serpin aggregation by the molecular chaperone, alpha-crystallin, indicates a nucleation-dependent specificity.All-D-enantiomers of beta-amyloid exhibit similar biological properties to all-L-beta-amyloids.Inhibiting polymerization: new therapeutic strategies for Z alpha1-antitrypsin-related emphysema.alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization.Recurrent mutations in a SERPINC1 hotspot associate with venous thrombosis without apparent antithrombin deficiency.Identification of a 4-mer Peptide Inhibitor that Effectively Blocks the Polymerization of Pathogenic Z α1-Antitrypsin
P2860
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P2860
Thromboembolic disease due to thermolabile conformational changes of antithrombin Rouen-VI (187 Asn-->Asp)
description
1994 nî lūn-bûn
@nan
1994 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1994 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
1994年の論文
@ja
1994年論文
@yue
1994年論文
@zh-hant
1994年論文
@zh-hk
1994年論文
@zh-mo
1994年論文
@zh-tw
1994年论文
@wuu
name
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@ast
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@en
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@nl
type
label
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@ast
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@en
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@nl
prefLabel
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@ast
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@en
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@nl
P2093
P2860
P356
P1476
Thromboembolic disease due to ...... ombin Rouen-VI (187 Asn-->Asp)
@en
P2093
P2860
P304
P356
10.1172/JCI117589
P407
P577
1994-12-01T00:00:00Z