Familial dementia caused by polymerization of mutant neuroserpin
about
Two non-homologous brain diseases-related genes, SERPINI1 and PDCD10, are tightly linked by an asymmetric bidirectional promoter in an evolutionarily conserved mannerBinding of retinoic acid by the inhibitory serpin protein C inhibitorRAB26 and RAB3D are direct transcriptional targets of MIST1 that regulate exocrine granule maturationAlpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapySmall molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregatesMegsin gene: its genomic analysis, pathobiological functions, and therapeutic perspectivesInactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational diseaseEndoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpinsAn overview of the serpin superfamilyMolecular Pathological Classification of Neurodegenerative Diseases: Turning towards Precision MedicinePhysiological 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 DeficiencySynergy and Antagonism of Active Constituents of ADAPT-232 on Transcriptional Level of Metabolic Regulation of Isolated Neuroglial CellsTopography of a 2.0 Å structure of α1-antitrypsin reveals targets for rational drug design to prevent conformational diseaseBiochemical characterization of a neuroserpin variant associated with hereditary dementiaBiochemical characterization of Anopheles gambiae SRPN6, a malaria parasite invasion marker in mosquitoesRegulation of seizure spreading by neuroserpin and tissue-type plasminogen activator is plasminogen-independentSerine 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.Protein fiber linear dichroism for structure determination and kinetics in a low-volume, low-wavelength couette flow cellNeuroserpin mutation S52R causes neuroserpin accumulation in neurons and is associated with progressive myoclonus epilepsy.Neonatal Hypoxia Ischaemia: Mechanisms, Models, and Therapeutic Challenges.Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIBProtease Inhibitors in Tick Saliva: The Role of Serpins and Cystatins in Tick-host-Pathogen Interaction.Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization.The tempered polymerization of human neuroserpin.Proteotoxicity in the endoplasmic reticulum: lessons from the Akita diabetic mouse.The necrotic gene in Drosophila corresponds to one of a cluster of three serpin transcripts mapping at 43A1.2.Bioinformatic analyses of male and female Amblyomma americanum tick expressed serine protease inhibitors (serpins).Protein misfolding and the serpinopathies.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 DynamicsTissue plasminogen activator-independent roles of neuroserpin in the central nervous system.Local conformational flexibility provides a basis for facile polymer formation in human neuroserpinThe genetic and molecular bases of monogenic disorders affecting proteolytic systems.Energy landscapes of functional proteins are inherently risky.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.Serpin Inhibition Mechanism: A Delicate Balance between Native Metastable State and Polymerization.Familial encephalopathy with neuroserpin inclusion bodies
P2860
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P2860
Familial dementia caused by polymerization of mutant neuroserpin
description
1999 nî lūn-bûn
@nan
1999 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
1999 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
1999年の論文
@ja
1999年論文
@yue
1999年論文
@zh-hant
1999年論文
@zh-hk
1999年論文
@zh-mo
1999年論文
@zh-tw
1999年论文
@wuu
name
Familial dementia caused by polymerization of mutant neuroserpin
@ast
Familial dementia caused by polymerization of mutant neuroserpin
@en
Familial dementia caused by polymerization of mutant neuroserpin
@nl
type
label
Familial dementia caused by polymerization of mutant neuroserpin
@ast
Familial dementia caused by polymerization of mutant neuroserpin
@en
Familial dementia caused by polymerization of mutant neuroserpin
@nl
prefLabel
Familial dementia caused by polymerization of mutant neuroserpin
@ast
Familial dementia caused by polymerization of mutant neuroserpin
@en
Familial dementia caused by polymerization of mutant neuroserpin
@nl
P2093
P2860
P3181
P1433
P1476
Familial dementia caused by polymerization of mutant neuroserpin
@en
P2093
Bradshaw C
Carrell RW
Collins GH
Elliott PR
Holohan PD
P2860
P2888
P304
P3181
P356
10.1038/43894
10.1038/43897
P407
P577
1999-09-01T00:00:00Z
P6179
1009163891