PABPN 1: molecular function and muscle disease

A Banerjee, LH Apponi, GK Pavlath… - The FEBS …, 2013 - Wiley Online Library
The polyadenosine RNA binding protein polyadenylate‐binding nuclear protein 1 (PABPN
1) plays key roles in post‐transcriptional processing of RNA. Although PABPN 1 is …

Poly (A) RNA‐binding proteins and polyadenosine RNA: new members and novel functions

CP Wigington, KR Williams, MP Meers… - Wiley …, 2014 - Wiley Online Library
Poly (A) RNA‐binding proteins (Pabs) bind with high affinity and specificity to polyadenosine
RNA. Textbook models show a nuclear Pab, PABPN1, and a cytoplasmic Pab, PABPC …

Poly (A) binding protein nuclear 1 levels affect alternative polyadenylation

E de Klerk, A Venema, SY Anvar… - Nucleic acids …, 2012 - academic.oup.com
The choice for a polyadenylation site determines the length of the 3′-untranslated region
(3′-UTRs) of an mRNA. Inclusion or exclusion of regulatory sequences in the 3′-UTR may …

Activation of the ubiquitin-proteasome system contributes to oculopharyngeal muscular dystrophy through muscle atrophy

C Ribot, C Soler, A Chartier, S Al Hayek… - PLoS …, 2022 - journals.plos.org
Oculopharyngeal muscular dystrophy (OPMD) is a late-onset disorder characterized by
progressive weakness and degeneration of specific muscles. OPMD is due to extension of a …

Regulated Intron Retention and Nuclear Pre-mRNA Decay Contribute to PABPN1 Autoregulation

D Bergeron, G Pal, YB Beaulieu, B Chabot… - … and cellular biology, 2015 - Taylor & Francis
The poly (A)-binding protein nuclear 1 is encoded by the PABPN1 gene, whose mutations
result in oculopharyngeal muscular dystrophy, a late-onset disorder for which the molecular …

Alternative polyadenylation: An enigma of transcript length variation in health and disease

NK Mohanan, F Shaji, GR Koshre… - Wiley Interdisciplinary …, 2022 - Wiley Online Library
Alternative polyadenylation (APA) is a molecular mechanism during a pre‐mRNA
processing that involves usage of more than one polyadenylation site (PA‐site) generating …

Mitochondrial dysfunction reveals the role of mRNA poly (A) tail regulation in oculopharyngeal muscular dystrophy pathogenesis

A Chartier, P Klein, S Pierson, N Barbezier… - PLoS …, 2015 - journals.plos.org
Oculopharyngeal muscular dystrophy (OPMD), a late-onset disorder characterized by
progressive degeneration of specific muscles, results from the extension of a polyalanine …

Novel mouse models of oculopharyngeal muscular dystrophy (OPMD) reveal early onset mitochondrial defects and suggest loss of PABPN1 may contribute to …

KE Vest, BL Phillips, A Banerjee… - Human molecular …, 2017 - academic.oup.com
Oculopharyngeal muscular dystrophy (OPMD) is a late onset disease caused by polyalanine
expansion in the poly (A) binding protein nuclear 1 (PABPN1). Several mouse models have …

Ubiquitin‐binding domains: Mechanisms of ubiquitin recognition and use as tools to investigate ubiquitin‐modified proteomes

D Scott, NJ Oldham, J Strachan, MS Searle… - …, 2015 - Wiley Online Library
Ubiquitin‐binding domains (UBDs) are modular units found within ubiquitin‐binding proteins
that mediate the non‐covalent recognition of (poly) ubiquitin modifications. A variety of …

A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle aging

SY Anvar, Y Raz, N Verwey, B van der Sluijs… - Aging (Albany …, 2013 - pmc.ncbi.nlm.nih.gov
Oculopharyngeal muscular dystrophy (OPMD) is caused by trinucleotide repeat expansion
mutations in Poly (A) binding protein 1 (PABPN1). PABPN1 is a regulator of mRNA stability …