The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal
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A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease.The N17 domain mitigates nuclear toxicity in a novel zebrafish Huntington's disease model.N17 Modifies mutant Huntingtin nuclear pathogenesis and severity of disease in HD BAC transgenic miceStructure and Topology of the Huntingtin 1–17 Membrane Anchor by a Combined Solution and Solid-State NMR ApproachGeneration and Characterization of Knock-in Mouse Models Expressing Versions of Huntingtin with Either an N17 or a Combined PolyQ and Proline-Rich Region Deletion.Is Huntingtin Dispensable in the Adult Brain?N-terminal Huntingtin Knock-In Mice: Implications of Removing the N-terminal Region of Huntingtin for Therapy.Free-Energy Landscape of the Amino-Terminal Fragment of Huntingtin in Aqueous SolutionUnmasking the roles of N- and C-terminal flanking sequences from exon 1 of huntingtin as modulators of polyglutamine aggregation.Phosphorylation of mutant huntingtin at serine 116 modulates neuronal toxicity.Probing the Huntingtin 1-17 membrane anchor on a phospholipid bilayer by using all-atom simulationsPolyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopyDiscovery of novel isoforms of huntingtin reveals a new hominid-specific exonSUMOylation regulates ciliary localization of olfactory signaling proteins.Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD.Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Span in Vivo.Ciliary Entry of the Hedgehog Transcriptional Activator Gli2 Is Mediated by the Nuclear Import Machinery but Differs from Nuclear Transport in Being Imp-α/β1-IndependentGated entry into the ciliary compartment.Acetylation within the First 17 Residues of Huntingtin Exon 1 Alters Aggregation and Lipid Binding.Molecular connections between nuclear and ciliary import processes.Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis.From pathways to targets: understanding the mechanisms behind polyglutamine disease.Post-Translational Modifications (PTMs), Identified on Endogenous Huntingtin, Cluster within Proteolytic Domains between HEAT Repeats.Evolutionary implications of localization of the signaling scaffold protein parafusin to both cilia and the nucleus.Primary cilia and autophagic dysfunction in Huntington's disease.Mechanisms Regulating Protein Localization.CILIA: before and after.Structure of a single-chain Fv bound to the 17 N-terminal residues of huntingtin provides insights into pathogenic amyloid formation and suppression.Huntingtin N17 domain is a reactive oxygen species sensor regulating huntingtin phosphorylation and localization.Altered Co-Translational Processing Plays a Role in Huntington's Pathogenesis-A Hypothesis.Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract.Depletion of p62 reduces nuclear inclusions and paradoxically ameliorates disease phenotypes in Huntington's model mice.Huntingtin is a scaffolding protein in the ATM oxidative DNA damage response complex.Post-translational modifications clustering within proteolytic domains decrease mutant huntingtin toxicity.Phosphorylation of huntingtin at residue T3 is decreased in Huntington's disease and modulates mutant huntingtin protein conformation.Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases.Protein phosphatase 1 regulates huntingtin exon 1 aggregation and toxicity.A comprehensive in silico analysis of huntingtin and its interactome.Assembly of Huntingtin headpiece into α-helical bundles.Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3.
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P2860
The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal
description
2013 nî lūn-bûn
@nan
2013年の論文
@ja
2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
@zh-mo
2013年論文
@zh-tw
2013年论文
@wuu
2013年论文
@zh
2013年论文
@zh-cn
name
The huntingtin N17 domain is a ...... clear and cilial export signal
@en
type
label
The huntingtin N17 domain is a ...... clear and cilial export signal
@en
prefLabel
The huntingtin N17 domain is a ...... clear and cilial export signal
@en
P2093
P2860
P356
P1476
The huntingtin N17 domain is a ...... clear and cilial export signal
@en
P2093
P2860
P304
P356
10.1093/HMG/DDS554
P577
2013-01-07T00:00:00Z