| The polyA tail facilitates splicing of last introns with weak 3' splice sites via PABPN1. | The polyA tail facilitates splicing of last introns with weak 3' splice sites via PABPN1.
Huang L, Li G, Du C, Jia Y, Yang J, Fan W, Xu YZ, Cheng H, Zhou Y., Free PMC Article | 09/13/2024 |
| PABPN1 loss-of-function causes APA-shift in oculopharyngeal muscular dystrophy. | PABPN1 loss-of-function causes APA-shift in oculopharyngeal muscular dystrophy.
Shademan M, Mei H, van Engelen B, Ariyurek Y, Kloet S, Raz V., Free PMC Article | 04/17/2024 |
| 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 functions as a hub in the assembly of nuclear poly(A) domains that are essential for mouse oocyte development. | PABPN1 functions as a hub in the assembly of nuclear poly(A) domains that are essential for mouse oocyte development.
Dai XX, Pi SB, Zhao LW, Wu YW, Shen JL, Zhang SY, Sha QQ, Fan HY., Free PMC Article | 11/5/2022 |
| 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 |
| results from our study support a model where altered protein interactions with alanine-expanded PABPN1 that lead to loss or gain of function could contribute to pathology in oculopharyngeal muscular dystrophy | Proteomic analysis reveals that wildtype and alanine-expanded nuclear poly(A)-binding protein exhibit differential interactions in skeletal muscle.
Banerjee A, Phillips BL, Deng Q, Seyfried NT, Pavlath GK, Vest KE, Corbett AH., Free PMC Article | 01/11/2020 |
| Data show that a trans-acting factor, RNA binding protein HuR (HuR), which regulate poly(A) binding protein nuclear 1 (Pabpn1) expression specifically in mature muscle in vitro and in vivo. | Post-transcriptional regulation of Pabpn1 by the RNA binding protein HuR.
Phillips BL, Banerjee A, Sanchez BJ, Di Marco S, Gallouzi IE, Pavlath GK, Corbett AH., Free PMC Article | 08/31/2019 |
| PABPN1-17A gave rise to: inhibition of Ca2+ release during ECC, depletion of sarcoplasmic reticulum Ca2+ content, reduced expression of ryanodine receptors, altered nuclear morphology and incapability to stimulate myoblast fusion. | Functional impact of an oculopharyngeal muscular dystrophy mutation in PABPN1.
García-Castañeda M, Vega AV, Rodríguez R, Montiel-Jaen MG, Cisneros B, Zarain-Herzberg A, Avila G., Free PMC Article | 03/31/2018 |
| Matrin 3 (MATR3) is a novel protein interactor of PABPN1. MATR3 also binds and regulates the levels of long non-coding RNA (lncRNA) Neat1 and together with PABPN1 is required for normal paraspeckle function. | Nuclear poly(A) binding protein 1 (PABPN1) and Matrin3 interact in muscle cells and regulate RNA processing.
Banerjee A, Vest KE, Pavlath GK, Corbett AH., Free PMC Article | 11/11/2017 |
| Expression levels of these cytokines were highly correlated in controls, but this correlation pattern was disrupted in OPMD. 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. | 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 |
| educed PABPN1 levels caused a consistent decline in distal PAS utilization in the 3'-UTR of a subset of OPMD-dysregulated genes. This alternative PAS utilization led to up-regulation of Atrogin-1, a key muscle atrophy regulator, but down regulation of proteasomal genes. Additionally reduced PABPN1 levels caused a reduction in proteasomal activity, and transition in MyHC isotope expression pattern in myofibers. | PABPN1-Dependent mRNA Processing Induces Muscle Wasting.
Riaz M, Raz Y, van Putten M, Paniagua-Soriano G, Krom YD, Florea BI, Raz V., Free PMC Article | 05/13/2017 |
| PABPN1 interacts with and is stabilized by heat shock protein 90. | 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 Protein.
Shi C, Huang X, Zhang B, Zhu D, Luo H, Lu Q, Xiong WC, Mei L, Luo S., Free PMC Article | 06/11/2016 |
| These findings demonstrate a role for PABPN1 in rescuing several cytopathological features of TDP-43 proteinopathy by increasing the turnover of pathologic proteins. | PABPN1 suppresses TDP-43 toxicity in ALS disease models.
Chou CC, Alexeeva OM, Yamada S, Pribadi A, Zhang Y, Mo B, Williams KR, Zarnescu DC, Rossoll W., Free PMC Article | 06/4/2016 |
| the first step of the cleavage and polyadenylation reaction, mRNA cleavage, is affected in muscles expressing alanine-expanded PABPN1. We propose that impaired cleavage is an early defect in Oculopharyngeal muscular dystrophy. | Mitochondrial dysfunction reveals the role of mRNA poly(A) tail regulation in oculopharyngeal muscular dystrophy pathogenesis.
Chartier A, Klein P, Pierson S, Barbezier N, Gidaro T, Casas F, Carberry S, Dowling P, Maynadier L, Bellec M, Oloko M, Jardel C, Moritz B, Dickson G, Mouly V, Ohlendieck K, Butler-Browne G, Trollet C, Simonelig M., Free PMC Article | 01/2/2016 |
| These results suggest that PABPN1 levels regulate muscle cell aging and oculopharyngeal muscular dystrophy represents an accelerated muscle aging disorder. | A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle aging.
Anvar SY, Raz Y, Verway N, van der Sluijs B, Venema A, Goeman JJ, Vissing J, van der Maarel SM, 't Hoen PA, van Engelen BG, Raz V., Free PMC Article | 11/16/2013 |
| tristetraprolin inhibits poly(A) tail synthesis by interacting with poly(A)-binding protein nuclear 1 in the nucleus to regulate expression of AU-rich element-containing mRNA | Tristetraprolin inhibits poly(A)-tail synthesis in nuclear mRNA that contains AU-rich elements by interacting with poly(A)-binding protein nuclear 1.
Su YL, Wang SC, Chiang PY, Lin NY, Shen YF, Chang GD, Chang CJ., Free PMC Article | 12/8/2012 |
| PABPN1 plays an essential role in myoblast proliferation and differentiation, suggesting that it is required for muscle regeneration and maintenance in vivo. | Loss of nuclear poly(A)-binding protein 1 causes defects in myogenesis and mRNA biogenesis.
Apponi LH, Leung SW, Williams KR, Valentini SR, Corbett AH, Pavlath GK., Free PMC Article | 06/14/2010 |
| C-terminal domain containing the methylated arginine residues is known to promote PAPBN1 self-association, and arginine methylation has been reported to inhibit self-association of an orthologous protein | Promiscuous modification of the nuclear poly(A)-binding protein by multiple protein-arginine methyltransferases does not affect the aggregation behavior.
Fronz K, Otto S, Kölbel K, Kühn U, Friedrich H, Schierhorn A, Beck-Sickinger AG, Ostareck-Lederer A, Wahle E. | 01/21/2010 |
| PABPN1 has a role in myogenesis | Ectopic expression of a polyalanine expansion mutant of poly(A)-binding protein N1 in muscle cells in culture inhibits myogenesis.
Wang Q, Bag J. | 01/21/2010 |