PABPN 1: molecular function and muscle disease
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 …
1) plays key roles in post‐transcriptional processing of RNA. Although PABPN 1 is …
[HTML][HTML] Oculopharyngeal muscular dystrophy: recent advances in the understanding of the molecular pathogenic mechanisms and treatment strategies
A Abu-Baker, GA Rouleau - … et Biophysica Acta (BBA)-Molecular Basis of …, 2007 - Elsevier
Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by
progressive eyelid drooping, swallowing difficulties and proximal limb weakness. OPMD is …
progressive eyelid drooping, swallowing difficulties and proximal limb weakness. OPMD is …
Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic …
C Trollet, SY Anvar, A Venema… - Human molecular …, 2010 - academic.oup.com
Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by
ptosis, dysphagia and proximal limb weakness. Autosomal-dominant OPMD is caused by a …
ptosis, dysphagia and proximal limb weakness. Autosomal-dominant OPMD is caused by a …
Poly (A) binding protein C1 is essential for efficient L1 retrotransposition and affects L1 RNP formation
Poly (A) binding proteins (PABPs) specifically bind the polyadenosine tail of mRNA and
have been shown to be important for RNA polyadenylation, translation initiation, and mRNA …
have been shown to be important for RNA polyadenylation, translation initiation, and mRNA …
H3K4me3 recognition by the COMPASS complex facilitates the restoration of this histone mark following DNA replication
A Serra-Cardona, S Duan, C Yu, Z Zhang - Science advances, 2022 - science.org
During DNA replication, parental H3-H4 marked by H3K4me3 are transferred almost equally
onto leading and lagging strands of DNA replication forks. Mutations in replicative helicase …
onto leading and lagging strands of DNA replication forks. Mutations in replicative helicase …
Phosphorylation of the nuclear poly (A) binding protein (PABPN1) during mitosis protects mRNA from hyperadenylation and maintains transcriptome dynamics
JM Gordon, DV Phizicky, L Schärfen… - Nucleic Acids …, 2024 - academic.oup.com
Polyadenylation controls mRNA biogenesis, nucleo-cytoplasmic export, translation and
decay. These processes are interdependent and coordinately regulated by poly (A)-binding …
decay. These processes are interdependent and coordinately regulated by poly (A)-binding …
PABPN1 polyalanine tract deletion and long expansions modify its aggregation pattern and expression
AF Klein, M Ebihara, C Alexander, MJ Dicaire… - Experimental cell …, 2008 - Elsevier
Expansions of a (GCN) 10/polyalanine tract in the Poly (A) Binding Protein Nuclear 1
(PABPN1) cause autosomal dominant oculopharyngeal muscular dystrophy (OPMD). In …
(PABPN1) cause autosomal dominant oculopharyngeal muscular dystrophy (OPMD). In …
Interactions between homopolymeric amino acids (HPAAs)
Y Oma, Y Kino, K Toriumi, N Sasagawa… - Protein …, 2007 - Wiley Online Library
Many human proteins contain consecutive amino acid repeats, known as homopolymeric
amino acid (HPAA) tracts. Some inherited diseases are caused by proteins in which HPAAs …
amino acid (HPAA) tracts. Some inherited diseases are caused by proteins in which HPAAs …
Soluble expanded PABPN1 promotes cell death in oculopharyngeal muscular dystrophy
C Messaed, PA Dion, A Abu-Baker, D Rochefort… - Neurobiology of …, 2007 - Elsevier
Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disease caused by
the expansion of a polyalanine repeat (GCG) 8–13 in exon 1 of the PABPN1 gene. Skeletal …
the expansion of a polyalanine repeat (GCG) 8–13 in exon 1 of the PABPN1 gene. Skeletal …
Nuclear speckles are involved in nuclear aggregation of PABPN1 and in the pathophysiology of oculopharyngeal muscular dystrophy
Nuclear speckles are essential nuclear compartments involved in the assembly, delivery
and recycling of pre-mRNA processing factors, and in the post-transcriptional processing of …
and recycling of pre-mRNA processing factors, and in the post-transcriptional processing of …