| Phosphorylation of the nuclear poly(A) binding protein (PABPN1) during mitosis protects mRNA from hyperadenylation and maintains transcriptome dynamics. | Phosphorylation of the nuclear poly(A) binding protein (PABPN1) during mitosis protects mRNA from hyperadenylation and maintains transcriptome dynamics.
Gordon JM, Phizicky DV, Schärfen L, Brown CL, Arias Escayola D, Kanyo J, Lam TT, Simon MD, Neugebauer KM., Free PMC Article | 10/9/2024 |
| Dysregulation of RNA-Exosome machinery is directly linked to major cancer hallmarks in prostate cancer: Oncogenic role of PABPN1. | Dysregulation of RNA-Exosome machinery is directly linked to major cancer hallmarks in prostate cancer: Oncogenic role of PABPN1.
Sáez-Martínez P, Porcel-Pastrana F, Montero-Hidalgo AJ, Lozano de la Haba S, Sanchez-Sanchez R, González-Serrano T, Gómez-Gómez E, Martínez-Fuentes AJ, Jiménez-Vacas JM, Gahete MD, Luque RM. | 02/29/2024 |
| PABPN1 aggregation is driven by Ala expansion and poly(A)-RNA binding, leading to CFIm25 sequestration that impairs alternative polyadenylation. | PABPN1 aggregation is driven by Ala expansion and poly(A)-RNA binding, leading to CFIm25 sequestration that impairs alternative polyadenylation.
Guan WL, Jiang LL, Yin XF, Hu HY., Free PMC Article | 09/6/2023 |
| The RNA-binding protein ZC3H11A interacts with the nuclear poly(A)-binding protein PABPN1 and alters polyadenylation of viral transcripts. | The RNA-binding protein ZC3H11A interacts with the nuclear poly(A)-binding protein PABPN1 and alters polyadenylation of viral transcripts.
Kases K, Schubert E, Hajikhezri Z, Larsson M, Devi P, Darweesh M, Andersson L, Akusjärvi G, Punga T, Younis S., Free PMC Article | 09/1/2023 |
| PABPN1 prevents the nuclear export of an unspliced RNA with a constitutive transport element and controls human gene expression via intron retention. | PABPN1 prevents the nuclear export of an unspliced RNA with a constitutive transport element and controls human gene expression via intron retention.
Kwiatek L, Landry-Voyer AM, Latour M, Yague-Sanz C, Bachand F., Free PMC Article | 04/20/2023 |
| Assessment of PABPN1 nuclear inclusions on a large cohort of patients and in a human xenograft model of oculopharyngeal muscular dystrophy. | Assessment of PABPN1 nuclear inclusions on a large cohort of patients and in a human xenograft model of oculopharyngeal muscular dystrophy.
Roth F, Dhiab J, Boulinguiez A, Mouigni HR, Lassche S, Negroni E, Muraine L, Marhic A, Oliver A, Lainé J, Rouche A, O'Ferrall EK, van Engelen B, Ottenheijm C, Greif H, Blumen S, Lacau St Guily J, Perie S, Butler-Browne G, Mouly V, Trollet C., Free PMC Article | 11/12/2022 |
| Novel read-through fusion transcript Bcl2l2-Pabpn1 in glioblastoma cells. | Novel read-through fusion transcript Bcl2l2-Pabpn1 in glioblastoma cells.
Zhang L, Wang D, Han X, Guo X, Cao Y, Xia Y, Gao D., Free PMC Article | 09/17/2022 |
| Activation of the ubiquitin-proteasome system contributes to oculopharyngeal muscular dystrophy through muscle atrophy. | Activation of the ubiquitin-proteasome system contributes to oculopharyngeal muscular dystrophy through muscle atrophy.
Ribot C, Soler C, Chartier A, Al Hayek S, Naït-Saïdi R, Barbezier N, Coux O, Simonelig M., Free PMC Article | 02/19/2022 |
| Regulation of Kv11.1 Isoform Expression by Polyadenylate Binding Protein Nuclear 1. | Regulation of Kv11.1 Isoform Expression by Polyadenylate Binding Protein Nuclear 1.
Stump MR, Nguyen RT, Drgastin RH, Search D, Gong Q, Zhou Z., Free PMC Article | 04/13/2021 |
| PABPN1, a Target of p63, Modulates Keratinocyte Differentiation through Regulation of p63alpha mRNA Translation. | PABPN1, a Target of p63, Modulates Keratinocyte Differentiation through Regulation of p63α mRNA Translation.
Mohibi S, Zhang J, Chen X., Free PMC Article | 04/3/2021 |
| The findings indicate that HuR regulates autophagy by modulating ATG16L1 translation via interaction with circPABPN1 in the intestinal epithelium. | Interaction between HuR and circPABPN1 Modulates Autophagy in the Intestinal Epithelium by Altering ATG16L1 Translation.
Li XX, Xiao L, Chung HK, Ma XX, Liu X, Song JL, Jin CZ, Rao JN, Gorospe M, Wang JY., Free PMC Article | 07/25/2020 |
| Mitochondrial localization of PABPN1 was observed in the muscle fibers of patients with Ooulopharyngeal muscular dystrophy. Moreover, abnormal accumulation of PABPN1 on the inner membrane of mitochondria and reduced expression of OXPHOS complexes were detected in the muscle fibers of transgenic mice expressing expanded human PABPN1 with a 13-alanine stretch. | Mitochondrial localization of PABPN1 in oculopharyngeal muscular dystrophy.
Doki T, Yamashita S, Wei FY, Hara K, Yamamoto T, Zhang Z, Zhang X, Tawara N, Hino H, Uyama E, Kurashige T, Maruyama H, Tomizawa K, Ando Y. | 07/11/2020 |
| We demonstrate that guanabenz acetate (GA) increases both the phosphorylation of the eukaryotic translation initiation factor 2alpha subunit and the splicing of Xbp1, key components of the UPR. Altogether these data show that modulation of protein folding regulation is beneficial for Oculopharyngeal muscular dystrophy (OPMD) and promote the further development of GA or its derivatives for treatment of OPMD in humans | Pharmacological modulation of the ER stress response ameliorates oculopharyngeal muscular dystrophy.
Malerba A, Roth F, Harish P, Dhiab J, Lu-Nguyen N, Cappellari O, Jarmin S, Mahoudeau A, Ythier V, Lainé J, Negroni E, Abgueguen E, Simonelig M, Guedat P, Mouly V, Butler-Browne G, Voisset C, Dickson G, Trollet C. | 02/8/2020 |
| As a crucial player in the DNA double-strand breaks (DSBs) response, nuclear poly(A)-binding protein 1 (PABPN1) relocalizes to DSBs sites and is phosphorylated in an ataxia telangiectasia mutated protein (ATM)-dependent manner. | Nuclear poly(A)-binding protein 1 is an ATM target and essential for DNA double-strand break repair.
Gavish-Izakson M, Velpula BB, Elkon R, Prados-Carvajal R, Barnabas GD, Ugalde AP, Agami R, Geiger T, Huertas P, Ziv Y, Shiloh Y., Free PMC Article | 07/27/2019 |
| PABPN1 is the master regulator in regulating Alternative polyadenylation in Cancer. | Comprehensive Characterization of Alternative Polyadenylation in Human Cancer.
Xiang Y, Ye Y, Lou Y, Yang Y, Cai C, Zhang Z, Mills T, Chen NY, Kim Y, Muge Ozguc F, Diao L, Karmouty-Quintana H, Xia Y, Kellems RE, Chen Z, Blackburn MR, Yoo SH, Shyu AB, Mills GB, Han L., Free PMC Article | 03/2/2019 |
| The expression of Ala-expanded-PABPN1 causes the formation of nuclear aggregates before the onset of muscle weakness in oculopharyngeal muscular dystrophy. | Intranuclear Aggregates Precede Clinical Onset in Oculopharyngeal Muscular Dystrophy.
van der Sluijs BM, Raz V, Lammens M, van den Heuvel LP, Voermans NC, van Engelen BG. | 03/17/2018 |
| The purpose of this study was to characterize the type of PABPN1 expanded alleles in a large cohort of Oculopharyngeal muscular dystrophy individuals from Mexico. | Characterization of PABPN1 expansion mutations in a large cohort of Mexican patients with oculopharyngeal muscular dystrophy (OPMD).
Cruz-Aguilar M, Guerrero-de Ferran C, Tovilla-Canales JL, Nava-Castañeda A, Zenteno JC. | 12/23/2017 |
| We propose that the extensive binding of CircPABPN1 to HuR prevents HuR binding to PABPN1 mRNA and lowers PABPN1 translation, providing the first example of competition between a circRNA and its cognate mRNA for an RBP that affects translation. | Identification of HuR target circular RNAs uncovers suppression of PABPN1 translation by CircPABPN1.
Abdelmohsen K, Panda AC, Munk R, Grammatikakis I, Dudekula DB, De S, Kim J, Noh JH, Kim KM, Martindale JL, Gorospe M., Free PMC Article | 10/21/2017 |
| Whereas PABPN1 strongly increases the activity of its cognate poly(A) polymerase in vitro, Pab2 was unable to stimulate Pla1 to any significant extent. | The nuclear poly(A) binding protein of mammals, but not of fission yeast, participates in mRNA polyadenylation.
Kühn U, Buschmann J, Wahle E., Free PMC Article | 09/9/2017 |
| The described is the mRNA degradation poly(A) tail exosome targeting (PAXT) connection, which comprises the ZFC3H1 Zn-knuckle protein as a central link between MTR4 and the nuclear poly(A)-binding protein PABPN1. | Identification of a Nuclear Exosome Decay Pathway for Processed Transcripts.
Meola N, Domanski M, Karadoulama E, Chen Y, Gentil C, Pultz D, Vitting-Seerup K, Lykke-Andersen S, Andersen JS, Sandelin A, Jensen TH. | 09/2/2017 |
| PABPN1 aggregates are able to trap TNNT3 pre-mRNA, driving it outside nuclear speckles, leading to an altered SC35-mediated splicing. | Nuclear poly(A)-binding protein aggregates misplace a pre-mRNA outside of SC35 speckle causing its abnormal splicing.
Klein P, Oloko M, Roth F, Montel V, Malerba A, Jarmin S, Gidaro T, Popplewell L, Perie S, Lacau St Guily J, de la Grange P, Antoniou MN, Dickson G, Butler-Browne G, Bastide B, Mouly V, Trollet C., Free PMC Article | 06/24/2017 |
| The levels of these 6 cytokines were not altered in expPABPN1 carriers at a pre-symptomatic stage, suggesting that this group of cytokines is a potential biomarker for muscle weakness in OPMD. Correlation pattern of expression levels could be a novel measurer for disease state | Cytokine genes as potential biomarkers for muscle weakness in OPMD.
Riaz M, Raz Y, van der Slujis B, Dickson G, van Engelen B, Vissing J, Raz V. | 06/10/2017 |
| Large cohort study demonstrated that in heterozygous and homozygous patients with oculopharyngeal muscular dystrophy, the mean age at diagnosis and the severity of the clinical symptoms correlate to the number of PABPN1 (GCN) repeats. Homozygous patients showed the worse phenotype, suggesting a gene-dose effect in addition to the repeat number expansion. | Correlation between PABPN1 genotype and disease severity in oculopharyngeal muscular dystrophy.
Richard P, Trollet C, Stojkovic T, de Becdelievre A, Perie S, Pouget J, Eymard B, Neurologists of French Neuromuscular Reference Centers CORNEMUS and FILNEMUS., Free PMC Article | 05/27/2017 |
| we found a polyadenylation-dependent 3' end maturation pathway for the human telomerase RNA that relies on the nuclear poly(A)-binding protein PABPN1 and the poly(A)-specific RNase PARN. | A Polyadenylation-Dependent 3' End Maturation Pathway Is Required for the Synthesis of the Human Telomerase RNA.
Nguyen D, Grenier St-Sauveur V, Bergeron D, Dupuis-Sandoval F, Scott MS, Bachand F. | 09/24/2016 |
| Many ribozymes were assayed and validated, including four ribozymes targeting the transcript of a disease-causing gene (a mutant version of PABPN1). | Automated design of hammerhead ribozymes and validation by targeting the PABPN1 gene transcript.
Kharma N, Varin L, Abu-Baker A, Ouellet J, Najeh S, Ehdaeivand MR, Belmonte G, Ambri A, Rouleau G, Perreault J., Free PMC Article | 08/6/2016 |