Association between conformational mutations in neuroserpin and onset and severity of dementia
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Two non-homologous brain diseases-related genes, SERPINI1 and PDCD10, are tightly linked by an asymmetric bidirectional promoter in an evolutionarily conserved mannerRAB26 and RAB3D are direct transcriptional targets of MIST1 that regulate exocrine granule maturationMutations in SERPINB7, encoding a member of the serine protease inhibitor superfamily, cause Nagashima-type palmoplantar keratosisSmall molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregatesCrystal structure of protein Z-dependent inhibitor complex shows how protein Z functions as a cofactor in the membrane inhibition of factor XAn overview of the serpin superfamilyPhysiological and pathological roles of tissue plasminogen activator and its inhibitor neuroserpin in the nervous systemDeficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin DeficiencySerpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerolNeuroserpin binds Abeta and is a neuroprotective component of amyloid plaques in Alzheimer diseaseMutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulumLatent S49P neuroserpin forms polymers in the dementia familial encephalopathy with neuroserpin inclusion bodiesProcessing of surfactant protein C requires a type II transmembrane topology directed by juxtamembrane positively charged residuesSerine proteases, serine protease inhibitors, and protease-activated receptors: roles in synaptic function and behaviorIdentification of a novel targeting sequence for regulated secretion in the serine protease inhibitor neuroserpin.Neonatal Hypoxia Ischaemia: Mechanisms, Models, and Therapeutic Challenges.Neuroserpin polymorphisms and stroke risk in a biracial population: the stroke prevention in young women studyNeuroserpin 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.A recurrent de novo mutation in KCNC1 causes progressive myoclonus epilepsy.Protein misfolding and the serpinopathies.The endoplasmic reticulum (ER)-associated degradation system regulates aggregation and degradation of mutant neuroserpin.Tissue plasminogen activator-independent roles of neuroserpin in the central nervous system.Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.The aggregation-prone intracellular serpin SRP-2 fails to transit the ER in Caenorhabditis elegans.Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies.Accumulation of mutant neuroserpin precedes development of clinical symptoms in familial encephalopathy with neuroserpin inclusion bodies.Serpin Inhibition Mechanism: A Delicate Balance between Native Metastable State and Polymerization.Alpha1-antitrypsin deficiency. 4: Molecular pathophysiologyIdentification of a neurovascular signaling pathway regulating seizures in miceExpression of SERPINA3s in cattle: focus on bovSERPINA3-7 reveals specific involvement in skeletal muscle.Embelin binds to human neuroserpin and impairs its polymerisation.What we owe to alpha(1)-antitrypsin and to Carl-Bertil Laurell.The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB.Update on research, diagnosis and management of alphal-antitrypsin deficiency.Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulumNeuroserpin 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.
P2860
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P2860
Association between conformational mutations in neuroserpin and onset and severity of dementia
description
2002 nî lūn-bûn
@nan
2002 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2002 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2002年の論文
@ja
2002年論文
@yue
2002年論文
@zh-hant
2002年論文
@zh-hk
2002年論文
@zh-mo
2002年論文
@zh-tw
2002年论文
@wuu
name
Association between conformati ...... onset and severity of dementia
@ast
Association between conformati ...... onset and severity of dementia
@en
Association between conformati ...... onset and severity of dementia
@nl
type
label
Association between conformati ...... onset and severity of dementia
@ast
Association between conformati ...... onset and severity of dementia
@en
Association between conformati ...... onset and severity of dementia
@nl
prefLabel
Association between conformati ...... onset and severity of dementia
@ast
Association between conformati ...... onset and severity of dementia
@en
Association between conformati ...... onset and severity of dementia
@nl
P2093
P1433
P1476
Association between conformati ...... onset and severity of dementia
@en
P2093
Antony E Shrimpton
Bernardino Ghetti
Bruno Baumann
Charles B Bradshaw
Daniel A Lawrence
Daria Larocca
David A Lomas
Felicitas Lacbawan
George H Collins
Lieselotte Gerhard
P304
P356
10.1016/S0140-6736(02)09293-0
P407
P577
2002-06-29T00:00:00Z