Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum
about
Two non-homologous brain diseases-related genes, SERPINI1 and PDCD10, are tightly linked by an asymmetric bidirectional promoter in an evolutionarily conserved mannerEndoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpinsCrystallographic and Cellular Characterisation of Two Mechanisms Stabilising the Native Fold of α1-Antitrypsin: Implications for Disease and Drug DesignNeuroserpin binds Abeta and is a neuroprotective component of amyloid plaques in Alzheimer diseaseLatent S49P neuroserpin forms polymers in the dementia familial encephalopathy with neuroserpin inclusion bodiesIdentification of a novel targeting sequence for regulated secretion in the serine protease inhibitor neuroserpin.Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIBA novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.The tempered polymerization of human neuroserpin.Three new alpha1-antitrypsin deficiency variants help to define a C-terminal region regulating conformational change and polymerization.Protein misfolding and the serpinopathies.Association between neuroserpin and molecular markers of brain damage in patients with acute ischemic stroke.The endoplasmic reticulum (ER)-associated degradation system regulates aggregation and degradation of mutant neuroserpin.Functional and dysfunctional conformers of human neuroserpin characterized by optical spectroscopies and Molecular DynamicsAAV-mediated overexpression of neuroserpin in the hippocampus decreases PSD-95 expression but does not affect hippocampal-dependent learning and memory.The aggregation-prone intracellular serpin SRP-2 fails to transit the ER in Caenorhabditis elegans.Accumulation of mutant neuroserpin precedes development of clinical symptoms in familial encephalopathy with neuroserpin inclusion bodies.A cure for traffic jams: small molecule chaperones in the endoplasmic reticulum.A single-chain variable fragment intrabody prevents intracellular polymerization of Z α1-antitrypsin while allowing its antiproteinase activityEmbelin binds to human neuroserpin and impairs its polymerisation.Sequestration of mutated alpha1-antitrypsin into inclusion bodies is a cell-protective mechanism to maintain endoplasmic reticulum function.The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB.Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulumPolymers and inflammation: disease mechanisms of the serpinopathiesNeuroserpin polymers activate NF-kappaB by a calcium signaling pathway that is independent of the unfolded protein response.alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies.Hepatic fibrosis and carcinogenesis in α1-antitrypsin deficiency: a prototype for chronic tissue damage in gain-of-function disorders.α(1)-antitrypsin deficiency and inflammation.Unravelling the twists and turns of the serpinopathies.Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency.The unfolded protein response modulates toxicity of the expanded glutamine androgen receptor.A critical role of Hrd1 in the regulation of optineurin degradation and aggresome formation.Inhibitory serpins. New insights into their folding, polymerization, regulation and clearance.Inhibition of proteasome by bortezomib causes intracellular aggregation of hepatic serpins and increases the latent circulating form of antithrombin.pH-dependent stability of neuroserpin is mediated by histidines 119 and 138; implications for the control of beta-sheet A and polymerization.Protein accumulation in the endoplasmic reticulum as a non-equilibrium phase transition.The pathological Trento variant of alpha-1-antitrypsin (E75V) shows nonclassical behaviour during polymerization.Gp78 E3 Ubiquitin Ligase: Essential Functions and Contributions in Proteostasis.The stability and activity of human neuroserpin are modulated by a salt bridge that stabilises the reactive centre loop.Sterol metabolism regulates neuroserpin polymer degradation in the absence of the unfolded protein response in the dementia FENIB.
P2860
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P2860
Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum
description
2004 nî lūn-bûn
@nan
2004 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2004年の論文
@ja
2004年論文
@yue
2004年論文
@zh-hant
2004年論文
@zh-hk
2004年論文
@zh-mo
2004年論文
@zh-tw
2004年论文
@wuu
name
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@ast
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@en
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@nl
type
label
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@ast
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@en
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@nl
prefLabel
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@ast
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@en
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@nl
P2860
P356
P1476
Mutants of neuroserpin that ca ...... thin the endoplasmic reticulum
@en
P2093
David A Lomas
Karin Römisch
P2860
P304
P356
10.1074/JBC.M313166200
P407
P577
2004-07-02T00:00:00Z