PABPN1: molecular function and muscle disease
نویسندگان
چکیده
منابع مشابه
PABPN1-Dependent mRNA Processing Induces Muscle Wasting
Poly(A) Binding Protein Nuclear 1 (PABPN1) is a multifunctional regulator of mRNA processing, and its expression levels specifically decline in aging muscles. An expansion mutation in PABPN1 is the genetic cause of oculopharyngeal muscle dystrophy (OPMD), a late onset and rare myopathy. Moreover, reduced PABPN1 expression correlates with symptom manifestation in OPMD. PABPN1 regulates alternati...
متن کاملA decline in PABPN1 induces progressive muscle weakness in Oculopharyngeal muscle dystrophy and in muscle aging
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 and is ubiquitously expressed. Here we investigated how symptoms in OPMD initiate only at midlife and why a subset of skeletal muscles is predominantly affected. Genome-wide RNA expression profiles from Vastus lateralis mu...
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chapter one is devoted to a moderate discussion on preliminaries, according to our requirements. chapter two which is based on our work in (24) is devoted introducting weighted semigroups (s, w), and studying some famous function spaces on them, especially the relations between go (s, w) and other function speces are invesigated. in fact this chapter is a complement to (32). one of the main fea...
15 صفحه اولRespiratory muscle function in health and disease.
1982;81;82-90 Chest JM Luce and BH Culver Respiratory muscle function in health and disease http://chestjournal.org services can be found online on the World Wide Web at: The online version of this article, along with updated information and ). ISSN: 0012-3692. http://www.chestjournal.org/misc/reprints.shtml ( without the prior written permission of the copyright holder distributed rights reser...
متن کاملPABPN1 suppresses TDP-43 toxicity in ALS disease models.
TAR DNA-binding protein 43 (TDP-43) is a major disease protein in amyotrophic lateral sclerosis (ALS) and related neurodegenerative diseases. Both the cytoplasmic accumulation of toxic ubiquitinated and hyperphosphorylated TDP-43 fragments and the loss of normal TDP-43 from the nucleus may contribute to the disease progression by impairing normal RNA and protein homeostasis. Therefore, both the...
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ژورنال
عنوان ژورنال: FEBS Journal
سال: 2013
ISSN: 1742-464X
DOI: 10.1111/febs.12294