about
ERp44, a novel endoplasmic reticulum folding assistant of the thioredoxin familyHDAC6 and microtubules are required for autophagic degradation of aggregated huntingtinA CADASIL-mutated Notch 3 receptor exhibits impaired intracellular trafficking and maturation but normal ligand-induced signaling.Altered ribostasis: RNA-protein granules in degenerative disordersNeuronal response in Alzheimer's and Parkinson's disease: the effect of toxic proteins on intracellular pathwaysRoles for the ubiquitin-proteasome pathway in protein quality control and signaling in the retina: implications in the pathogenesis of age-related macular degenerationDestabilizing protein polymorphisms in the genetic background direct phenotypic expression of mutant SOD1 toxicityDeciphering the structure, growth and assembly of amyloid-like fibrils using high-speed atomic force microscopyNovel polyglutamine model uncouples proteotoxicity from agingDistinct machinery is required in Saccharomyces cerevisiae for the endoplasmic reticulum-associated degradation of a multispanning membrane protein and a soluble luminal protein.Distinct retrieval and retention mechanisms are required for the quality control of endoplasmic reticulum protein folding.Cell death in the nervous systemThe neuroendocrine protein 7B2 suppresses the aggregation of neurodegenerative disease-related proteinsSeipinopathy: a novel endoplasmic reticulum stress-associated diseaseTrafficking and quality control of the gonadotropin releasing hormone receptor in health and diseaseThe threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegansElucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation.Ultrafast colorimetric determination of predominant protein structure evolution with gold nanoplasmonic particles.Addressing the Role of Conformational Diversity in Protein Structure PredictionEpithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis.Investigating the role of T-cell avidity and killing efficacy in relation to type 1 diabetes prediction.Effect of osmolytes on the conformation and aggregation of some amyloid peptides: CD spectroscopic data.Amyloidogenesis abolished by proline substitutions but enhanced by lipid binding.Organizational diversity among distinct glycoprotein endoplasmic reticulum-associated degradation programsReducing Igf-1r levels leads to paradoxical and sexually dimorphic effects in HD mice.Update and perspectives on congenital disorders of glycosylation.Recent progress in histochemistry and cell biology.The unfolded protein response: no longer just a special teams player.Pharmacological chaperone-mediated in vivo folding and stabilization of the P23H-opsin mutant associated with autosomal dominant retinitis pigmentosaDilated cardiomyopathy caused by aberrant endoplasmic reticulum quality control in mutant KDEL receptor transgenic mice.Polyglutamine amyloid core boundaries and flanking domain dynamics in huntingtin fragment fibrils determined by solid-state nuclear magnetic resonance.Scrapie-like prion protein accumulates in aggresomes of cyclosporin A-treated cells.Unraveling the contribution of pancreatic beta-cell suicide in autoimmune type 1 diabetes.Blocking formation of large protein aggregates by small peptides.Resveratrol inhibits the formation of multiple-layered β-sheet oligomers of the human islet amyloid polypeptide segment 22-27Genetics and molecular pathology of Stargardt-like macular degeneration.Expression of one important chaperone protein, heat shock protein 27, in neurodegenerative diseases.Ubiquitin-proteasome pathway and cellular responses to oxidative stress.Activation of endoplasmic reticulum stress response during the development of ischemic heart disease.Is all of the endoplasmic reticulum created equal? The effects of the heterogeneous distribution of endoplasmic reticulum Ca2+-handling proteins.
P2860
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P2860
description
2000 nî lūn-bûn
@nan
2000年の論文
@ja
2000年学术文章
@wuu
2000年学术文章
@zh
2000年学术文章
@zh-cn
2000年学术文章
@zh-hans
2000年学术文章
@zh-my
2000年学术文章
@zh-sg
2000年學術文章
@yue
2000年學術文章
@zh-hant
name
Conformational disease.
@en
Conformational disease.
@nl
type
label
Conformational disease.
@en
Conformational disease.
@nl
prefLabel
Conformational disease.
@en
Conformational disease.
@nl
P356
P1433
P1476
Conformational disease.
@en
P2093
P2888
P304
P356
10.1038/35041139
P50
P577
2000-11-01T00:00:00Z