Neuroserpin Portland (Ser52Arg) is trapped as an inactive intermediate that rapidly forms polymers: implications for the epilepsy seen in the dementia FENIB
about
Two non-homologous brain diseases-related genes, SERPINI1 and PDCD10, are tightly linked by an asymmetric bidirectional promoter in an evolutionarily conserved mannerNeuroserpin 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 bodiesThe tempered polymerization of human neuroserpin.Protein misfolding and the serpinopathies.Functional and dysfunctional conformers of human neuroserpin characterized by optical spectroscopies and Molecular DynamicsAccumulation of mutant neuroserpin precedes development of clinical symptoms in familial encephalopathy with neuroserpin inclusion bodies.Identification of a neurovascular signaling pathway regulating seizures in miceEmbelin binds to human neuroserpin and impairs its polymerisation.The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB.alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies.Unravelling the twists and turns of the serpinopathies.Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency.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 stability and activity of human neuroserpin are modulated by a salt bridge that stabilises the reactive centre loop.A hydrophobic patch surrounding Trp154 in human neuroserpin controls the helix F dynamics with implications in inhibition and aggregation.Cholesterol impairment contributes to neuroserpin aggregation.Tissue plasminogen activator, neuroserpin, and seizures.Two latent and two hyperstable polymeric forms of human neuroserpin.Probing neuroserpin polymerization and interaction with amyloid-beta peptides using single molecule fluorescence.Mutation-, aging-, and gene dosage-dependent accumulation of neuroserpin (G392E) in endoplasmic reticula and lysosomes of neurons in transgenic mice.Molecular bases of neuroserpin function and pathology.
P2860
Q21263000-203297EA-F441-480A-B148-DAD174B47B06Q28252760-B3D6A814-9E28-4F18-8442-13D2B90FCC09Q28303740-DDF02302-A319-4C5D-9CFE-BEC4416BB26CQ34193142-CDFF2E16-15F9-4571-B41F-A9AA0D336CE8Q34928474-FC652553-934D-4AD8-B9D8-A95771DD2FA6Q35094717-D18B99C8-737C-42D8-AE9A-F138F702A63EQ35691620-AFC38942-9FCB-47CD-83B6-C0FFA9A7C864Q35935089-F7697E24-056D-48C8-ACD2-9B26F30C8A09Q36432474-D1AA784B-E819-4D8E-858A-8A1DCB715C9EQ36663487-041B20F7-E84D-4CAF-A82C-75EA43144411Q37474870-22C676E9-D339-479D-A1C0-F915F2CD9CD6Q37882046-DFAFF69D-C05A-43BC-9DF6-55312FC1F9C7Q38092851-B6FA2D07-EF52-4CAA-84F8-2B4872C15458Q40404009-4F579F7F-8933-4AD1-B933-9634496F66CAQ40419382-954C43A6-76BB-42CB-A236-E66A2026028DQ41781013-0256F046-0252-422A-84CE-9AC4295522F6Q41854754-F9D34A46-1358-495C-9F2A-123E740E7E78Q42034639-880354FD-6305-41D4-9D68-2C23CBBB2CBDQ42147827-1F4D55AF-B627-4BB8-A94B-F95549FF6A7EQ42183296-F1E3C41E-3810-4077-BD6C-B1C843269496Q42183367-E4E144F2-2021-4DB6-BA28-B0BDBD303CB0Q46305320-0A707AB4-79B3-40B4-913F-CE7F4A9AB749Q53309303-CE53DC20-64C7-4C1A-914F-9AF61782F63E
P2860
Neuroserpin Portland (Ser52Arg) is trapped as an inactive intermediate that rapidly forms polymers: implications for the epilepsy seen in the dementia FENIB
description
2004 nî lūn-bûn
@nan
2004 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի օգոստոսին 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
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@ast
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@en
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@nl
type
label
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@ast
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@en
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@nl
prefLabel
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@ast
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@en
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@nl
P2093
P2860
P1433
P1476
Neuroserpin Portland (Ser52Arg ...... psy seen in the dementia FENIB
@en
P2093
Damian C Crowther
David A Lomas
Didier Belorgey
Lynda K Sharp
P2860
P304
P356
10.1111/J.1432-1033.2004.04270.X
P407
P577
2004-08-01T00:00:00Z