Oligomerization of polyalanine expanded PABPN1 facilitates nuclear protein aggregation that is associated with cell death
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Promiscuous modification of the nuclear poly(A)-binding protein by multiple protein-arginine methyltransferases does not affect the aggregation behaviorHnRNP A1 and A/B interaction with PABPN1 in oculopharyngeal muscular dystrophyMammalian, yeast, bacterial, and chemical chaperones reduce aggregate formation and death in a cell model of oculopharyngeal muscular dystrophyPolyalanine-independent conformational conversion of nuclear poly(A)-binding protein 1 (PABPN1).Loss of nuclear poly(A)-binding protein 1 causes defects in myogenesis and mRNA biogenesis.Functional insights from the distribution and role of homopeptide repeat-containing proteinsDoxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice.A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1.Evidence that poly(A) binding protein C1 binds nuclear pre-mRNA poly(A) tails.Over-expression of BCL2 rescues muscle weakness in a mouse model of oculopharyngeal muscular dystrophy.Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophyA yeast model for polyalanine-expansion aggregation and toxicityProgressive myopathy in an inducible mouse model of oculopharyngeal muscular dystrophy.The Inhibition of Heat Shock Protein 90 Facilitates the Degradation of Poly-Alanine Expanded Poly (A) Binding Protein Nuclear 1 via the Carboxyl Terminus of Heat Shock Protein 70-Interacting ProteinPABPN1 suppresses TDP-43 toxicity in ALS disease models.A polyalanine tract expansion in Arx forms intranuclear inclusions and results in increased cell death.Alanine Expansions Associated with Congenital Central Hypoventilation Syndrome Impair PHOX2B Homeodomain-mediated Dimerization and Nuclear ImportAn Antibody to Detect Alanine-Expanded PABPN1: A New Tool to Study Oculopharyngeal Muscular DystrophyPABPN1: molecular function and muscle disease.A folded and functional protein domain in an amyloid-like fibril.Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 proteinAssociation of polyalanine and polyglutamine coiled coils mediates expansion disease-related protein aggregation and dysfunctionCystamine suppresses polyalanine toxicity in a mouse model of oculopharyngeal muscular dystrophy.Stimulation of poly(A) polymerase through a direct interaction with the nuclear poly(A) binding protein allosterically regulated by RNA.In vivo aggregation properties of the nuclear poly(A)-binding protein PABPN1.Caspase 8 mediated apoptotic cell death induced by beta-sheet forming polyalanine peptides.How Common Is Disorder? Occurrence of Disordered Residues in Four Domains of Life.Conformational stability of the RNP domain controls fibril formation of PABPN1.Interactions between homopolymeric amino acids (HPAAs).Trinucleotide expansions leading to an extended poly-L-alanine segment in the poly (A) binding protein PABPN1 cause fibril formation.The RNA binding domains of the nuclear poly(A)-binding protein.A proline-tyrosine nuclear localization signal (PY-NLS) is required for the nuclear import of fission yeast PAB2, but not of human PABPN1.Wild-type PABPN1 is anti-apoptotic and reduces toxicity of the oculopharyngeal muscular dystrophy mutation.Functional impact of an oculopharyngeal muscular dystrophy mutation in PABPN1.Polyalanine expansions drive a shift into α-helical clusters without amyloid-fibril formation.Expression of the polyalanine expansion mutant of nuclear poly(A)-binding protein induces apoptosis via the p53 pathway.
P2860
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P2860
Oligomerization of polyalanine expanded PABPN1 facilitates nuclear protein aggregation that is associated with cell death
description
2001 nî lūn-bûn
@nan
2001 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年論文
@yue
2001年論文
@zh-hant
2001年論文
@zh-hk
2001年論文
@zh-mo
2001年論文
@zh-tw
2001年论文
@wuu
name
Oligomerization of polyalanine ...... is associated with cell death
@ast
Oligomerization of polyalanine ...... is associated with cell death
@en
Oligomerization of polyalanine ...... is associated with cell death
@nl
type
label
Oligomerization of polyalanine ...... is associated with cell death
@ast
Oligomerization of polyalanine ...... is associated with cell death
@en
Oligomerization of polyalanine ...... is associated with cell death
@nl
prefLabel
Oligomerization of polyalanine ...... is associated with cell death
@ast
Oligomerization of polyalanine ...... is associated with cell death
@en
Oligomerization of polyalanine ...... is associated with cell death
@nl
P2093
P3181
P356
P1476
Oligomerization of polyalanine ...... is associated with cell death
@en
P2093
P304
P3181
P356
10.1093/HMG/10.21.2341
P407
P577
2001-10-01T00:00:00Z