Polyalanine-independent conformational conversion of nuclear poly(A)-binding protein 1 (PABPN1).

The Journal of Biological Chemistry
Reno WinterElisabeth Schwarz

Abstract

Oculopharyngeal muscular dystrophy is a late-onset disease caused by an elongation of a natural 10-alanine segment within the N-terminal domain of the nuclear poly(A)-binding protein 1 (PABPN1) to maximally 17 alanines. The disease is characterized by intranuclear deposits consisting primarily of PABPN1. In previous studies, we could show that the N-terminal domain of PABPN1 forms amyloid-like fibrils. Here, we analyze fibril formation of full-length PABPN1. Unexpectedly, fibril formation was independent of the presence of the alanine segment. With regard to fibril formation kinetics and resistance against denaturants, fibrils formed by full-length PABPN1 had completely different properties from those formed by the N-terminal domain. Fourier transformed infrared spectroscopy and limited proteolysis showed that fibrillar PABPN1 has a structure that differs from native PABPN1. Circumstantial evidence is presented that the C-terminal domain is involved in fibril formation.

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Citations

Nov 17, 2015·Nature Structural & Molecular Biology·Saskia PollingDanny M Hatters
Dec 9, 2015·Nature Structural & Molecular Biology·Regina M Murphy
Aug 13, 2015·Protein Science : a Publication of the Protein Society·Jens LieboldElisabeth Schwarz
Apr 23, 2013·The FEBS Journal·Ayan BanerjeeAnita H Corbett
Apr 11, 2013·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Anja ButtstedtElisabeth Schwarz
Apr 25, 2013·Biological Chemistry·Reno WinterElisabeth Schwarz
Jul 9, 2014·Biological Chemistry·Steffen Damm, Elisabeth Schwarz
Jun 26, 2018·Nucleic Acids Research·Brittany L PhillipsAnita H Corbett
Apr 18, 2021·Scientific Reports·Francesca GenovaMaria Longeri

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