about
Functional amyloid formation within mammalian tissue.The 8 and 5 kDa fragments of plasma gelsolin form amyloid fibrils by a nucleated polymerization mechanism, while the 68 kDa fragment is not amyloidogenicA hIAPP-derived all-D-amino-acid inhibits hIAPP fibrillation efficiently at membrane surface by targeting α-helical oligomeric intermediatesY65C missense mutation in the WW domain of the Golabi-Ito-Hall syndrome protein PQBP1 affects its binding activity and deregulates pre-mRNA splicingMolecular pathogenesis of megalencephalic leukoencephalopathy with subcortical cysts: mutations in MLC1 cause folding defectsDefective cellular trafficking of missense NPR-B mutants is the major mechanism underlying acromesomelic dysplasia-type MaroteauxSequence determinants of protein aggregation: tools to increase protein solubility.Pancreatic β Cell Mass DeathSorting out the trash: the spatial nature of eukaryotic protein quality controlPharmacoperones: a new therapeutic approach for diseases caused by misfolded G protein-coupled receptorsEmerging novel concept of chaperone therapies for protein misfolding diseasesProtein homeostasis as a therapeutic target for diseases of protein conformationChemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosisHeat shock transcription factor 1 as a therapeutic target in neurodegenerative diseasesQuantifying the molecular origins of opposite solvent effects on protein-protein interactionsAltered Dimer Interface Decreases Stability in an Amyloidogenic ProteinToward Optimization of the Second Aryl Substructure Common to Transthyretin Amyloidogenesis Inhibitors Using Biochemical and Structural Studies †A Substructure Combination Strategy To Create Potent and Selective Transthyretin Kinetic Stabilizers That Prevent Amyloidogenesis and CytotoxicityStructural implications of a G170R mutation of alanine:glyoxylate aminotransferase that is associated with peroxisome-to-mitochondrion mistargetingPotent Kinetic Stabilizers That Prevent Transthyretin-Mediated Cardiomyocyte ProteotoxicityAromatic Sulfonyl Fluorides Covalently Kinetically Stabilize Transthyretin to Prevent Amyloidogenesis while Affording a Fluorescent ConjugateThe Molecular Basis of Pharmacological Chaperoning in Human α-GalactosidaseBifunctional coumarin derivatives that inhibit transthyretin amyloidogenesis and serve as fluorescent transthyretin folding sensorsStilbene Vinyl Sulfonamides as Fluorogenic Sensors of and Traceless Covalent Kinetic Stabilizers of Transthyretin That Prevent AmyloidogenesisStructural snapshots illustrate the catalytic cycle of β-galactocerebrosidase, the defective enzyme in Krabbe disease.Molecular Genetic Characterization of 151 Mut-Type Methylmalonic Aciduria Patients and Identification of 41 Novel Mutations in MUTPartial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasisPhenylbutyric acid rescues endoplasmic reticulum stress-induced suppression of APP proteolysis and prevents apoptosis in neuronal cellsUV-light exposed prion protein fails to form amyloid fibrilsAngiotensin I-converting enzyme Gln1069Arg mutation impairs trafficking to the cell surface resulting in selective denaturation of the C-domainA critical tryptophan and Ca2+ in activation and catalysis of TPPI, the enzyme deficient in classic late-infantile neuronal ceroid lipofuscinosisThe effect of chemical chaperones on the assembly and stability of HIV-1 capsid proteinKinetic and structural evidences on human prolidase pathological mutants suggest strategies for enzyme functional rescueIdentification and glycerol-induced correction of misfolding mutations in the X-linked mental retardation gene CASKTargeting the intrinsically disordered structural ensemble of α-synuclein by small molecules as a potential therapeutic strategy for Parkinson's diseaseA multiparametric computational algorithm for comprehensive assessment of genetic mutations in mucopolysaccharidosis type IIIA (Sanfilippo syndrome)Virtual and In Vitro Screens Reveal a Potential Pharmacophore that Avoids the Fibrillization of Aβ1-42Therapeutic Effect of Berberine on Huntington's Disease Transgenic Mouse ModelMisfolding of proteins with a polyglutamine expansion is facilitated by proteasomal chaperonesThe Fas/Fas ligand death receptor pathway contributes to phenylalanine-induced apoptosis in cortical neurons
P2860
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P2860
description
2003 nî lūn-bûn
@nan
2003 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2003 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
name
Therapeutic approaches to protein-misfolding diseases.
@ast
Therapeutic approaches to protein-misfolding diseases.
@en
Therapeutic approaches to protein-misfolding diseases.
@nl
type
label
Therapeutic approaches to protein-misfolding diseases.
@ast
Therapeutic approaches to protein-misfolding diseases.
@en
Therapeutic approaches to protein-misfolding diseases.
@nl
prefLabel
Therapeutic approaches to protein-misfolding diseases.
@ast
Therapeutic approaches to protein-misfolding diseases.
@en
Therapeutic approaches to protein-misfolding diseases.
@nl
P356
P1433
P1476
Therapeutic approaches to protein-misfolding diseases.
@en
P2093
Jeffery W Kelly
P2888
P304
P356
10.1038/NATURE02265
P407
P50
P577
2003-12-01T00:00:00Z
P5875
P6179
1053582289