Oculopharyngeal muscular dystrophy-like nuclear inclusions are present in normal magnocellular neurosecretory neurons of the hypothalamus
about
Novel variant of neuronal intranuclear rodlet immunoreactive for 40 kDa huntingtin associated protein and ubiquitin in the mouse brainPolyalanine-independent conformational conversion of nuclear poly(A)-binding protein 1 (PABPN1).Hsp70 chaperones and type I PRMTs are sequestered at intranuclear inclusions caused by polyalanine expansions in PABPN1Loss of nuclear poly(A)-binding protein 1 causes defects in myogenesis and mRNA biogenesis.Oculopharyngeal muscular dystrophy as a paradigm for muscle aging.A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1.Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophyA yeast model for polyalanine-expansion aggregation and toxicityMitochondrial dysfunction reveals the role of mRNA poly(A) tail regulation in oculopharyngeal muscular dystrophy pathogenesisA decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle agingControl of mRNA stability contributes to low levels of nuclear poly(A) binding protein 1 (PABPN1) in skeletal muscle.Nucleolar aggresomes as counterparts of cytoplasmic aggresomes in proteotoxic stress. Proteasome inhibitors induce nuclear ribonucleoprotein inclusions that accumulate several key factors of neurodegenerative diseases and cancer.PABPN1: molecular function and muscle disease.Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 proteinReversible aggregation of PABPN1 pre-inclusion structures.Increased E4 activity in mice leads to ubiquitin-containing aggregates and degeneration of hypothalamic neurons resulting in obesity.In vivo aggregation properties of the nuclear poly(A)-binding protein PABPN1.Conformational stability of the RNP domain controls fibril formation of PABPN1.Interactions between homopolymeric amino acids (HPAAs).Novel mouse models of oculopharyngeal muscular dystrophy (OPMD) reveal early onset mitochondrial defects and suggest loss of PABPN1 may contribute to pathology.Blockade of the NFκB pathway drives differentiating glioblastoma-initiating cells into senescence both in vitro and in vivo.Post-transcriptional regulation of Pabpn1 by the RNA binding protein HuR
P2860
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P2860
Oculopharyngeal muscular dystrophy-like nuclear inclusions are present in normal magnocellular neurosecretory neurons of the hypothalamus
description
2004 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 2004
@ast
im April 2004 veröffentlichter wissenschaftlicher Artikel
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scientific journal article
@en
vedecký článok (publikovaný 2004/04/15)
@sk
vědecký článek publikovaný v roce 2004
@cs
wetenschappelijk artikel (gepubliceerd op 2004/04/15)
@nl
наукова стаття, опублікована у квітні 2004
@uk
مقالة علمية (نشرت في 15-4-2004)
@ar
name
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@ast
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@en
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@nl
type
label
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@ast
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@en
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@nl
prefLabel
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@ast
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@en
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@nl
P2093
P356
P1476
Oculopharyngeal muscular dystr ...... ry neurons of the hypothalamus
@en
P2093
Joaquin Navascues
Jose L. Ojeda
Maria T. Berciano
Miguel Lafarga
Nuria T. Villagra
P304
P356
10.1093/HMG/DDH101
P577
2004-04-15T00:00:00Z