Literature DB >> 31203656

Conformational studies of pathogenic expanded polyglutamine protein deposits from Huntington's disease.

Irina Matlahov1, Patrick Ca van der Wel1,2.   

Abstract

Entities:  

Keywords:  Neurodegeneration; aggregation; biophysics; nuclear magnetic resonance; proteins; structural biology

Year:  2019        PMID： 31203656      PMCID： PMC6920524          DOI： 10.1177/1535370219856620

Source DB:  PubMed          Journal:  Exp Biol Med (Maywood)        ISSN： 1535-3699


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  125 in total

1.  IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.

Authors:  Leslie Michels Thompson; Charity T Aiken; Linda S Kaltenbach; Namita Agrawal; Katalin Illes; Ali Khoshnan; Marta Martinez-Vincente; Montserrat Arrasate; Jacqueline Gire O'Rourke; Hasan Khashwji; Tamas Lukacsovich; Ya-Zhen Zhu; Alice L Lau; Ashish Massey; Michael R Hayden; Scott O Zeitlin; Steven Finkbeiner; Kim N Green; Frank M LaFerla; Gillian Bates; Lan Huang; Paul H Patterson; Donald C Lo; Ana Maria Cuervo; J Lawrence Marsh; Joan S Steffan
Journal:  J Cell Biol       Date:  2009-12-21       Impact factor: 10.539

2.  The S/T-Rich Motif in the DNAJB6 Chaperone Delays Polyglutamine Aggregation and the Onset of Disease in a Mouse Model.

Authors:  Vaishali Kakkar; Cecilia Månsson; Eduardo P de Mattos; Steven Bergink; Marianne van der Zwaag; Maria A W H van Waarde; Niels J Kloosterhuis; Ronald Melki; Remco T P van Cruchten; Salam Al-Karadaghi; Paolo Arosio; Christopher M Dobson; Tuomas P J Knowles; Gillian P Bates; Jan M van Deursen; Sara Linse; Bart van de Sluis; Cecilia Emanuelsson; Harm H Kampinga
Journal:  Mol Cell       Date:  2016-04-14       Impact factor: 17.970

3.  Motor disorder in Huntington's disease begins as a dysfunction in error feedback control.

Authors:  M A Smith; J Brandt; R Shadmehr
Journal:  Nature       Date:  2000-02-03       Impact factor: 49.962

4.  Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease.

Authors:  J Carmichael; J Chatellier; A Woolfson; C Milstein; A R Fersht; D C Rubinsztein
Journal:  Proc Natl Acad Sci U S A       Date:  2000-08-15       Impact factor: 11.205

5.  Degradation of mutant huntingtin via the ubiquitin/proteasome system is modulated by FE65.

Authors:  Wan Ning Vanessa Chow; Hon Wing Luk; Ho Yin Edwin Chan; Kwok-Fai Lau
Journal:  Biochem J       Date:  2012-05-01       Impact factor: 3.857

6.  Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease.

Authors:  Kirupa Sathasivam; Andreas Neueder; Theresa A Gipson; Christian Landles; Agnesska C Benjamin; Marie K Bondulich; Donna L Smith; Richard L M Faull; Raymund A C Roos; David Howland; Peter J Detloff; David E Housman; Gillian P Bates
Journal:  Proc Natl Acad Sci U S A       Date:  2013-01-22       Impact factor: 11.205

Review 7.  Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases.

Authors:  Ray Truant; Randy Singh Atwal; Carly Desmond; Lise Munsie; Thu Tran
Journal:  FEBS J       Date:  2008-07-15       Impact factor: 5.542

8.  Self-assembly of Mutant Huntingtin Exon-1 Fragments into Large Complex Fibrillar Structures Involves Nucleated Branching.

Authors:  Anne S Wagner; Antonio Z Politi; Anne Ast; Kenny Bravo-Rodriguez; Katharina Baum; Alexander Buntru; Nadine U Strempel; Lydia Brusendorf; Christian Hänig; Annett Boeddrich; Stephanie Plassmann; Konrad Klockmeier; Juan M Ramirez-Anguita; Elsa Sanchez-Garcia; Jana Wolf; Erich E Wanker
Journal:  J Mol Biol       Date:  2018-03-28       Impact factor: 5.469

9.  Beta conformation of polyglutamine track revealed by a crystal structure of Huntingtin N-terminal region with insertion of three histidine residues.

Authors:  Meewhi Kim
Journal:  Prion       Date:  2013-01-31       Impact factor: 3.931

10.  The structure of a β2-microglobulin fibril suggests a molecular basis for its amyloid polymorphism.

Authors:  Matthew G Iadanza; Robert Silvers; Joshua Boardman; Hugh I Smith; Theodoros K Karamanos; Galia T Debelouchina; Yongchao Su; Robert G Griffin; Neil A Ranson; Sheena E Radford
Journal:  Nat Commun       Date:  2018-10-30       Impact factor: 14.919
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  6 in total

1.  Protofilament Structure and Supramolecular Polymorphism of Aggregated Mutant Huntingtin Exon 1.

Authors:  Jennifer C Boatz; Talia Piretra; Alessia Lasorsa; Irina Matlahov; James F Conway; Patrick C A van der Wel
Journal:  J Mol Biol       Date:  2020-06-27       Impact factor: 5.469

2.  Structural biology: Gaining atomic level insight into the biological function of macromolecules.

Authors:  Thomas B Thompson; Rhett A Kovall
Journal:  Exp Biol Med (Maywood)       Date:  2019-11-13

3.  Quantitative NMR analysis of the kinetics of prenucleation oligomerization and aggregation of pathogenic huntingtin exon-1 protein.

Authors:  Alberto Ceccon; Vitali Tugarinov; Francesco Torricella; G Marius Clore
Journal:  Proc Natl Acad Sci U S A       Date:  2022-07-12       Impact factor: 12.779

Review 4.  Molecular Pathophysiological Mechanisms in Huntington's Disease.

Authors:  Anamaria Jurcau
Journal:  Biomedicines       Date:  2022-06-17

5.  Live-Cell Imaging and Quantification of PolyQ Aggregates by Stimulated Raman Scattering of Selective Deuterium Labeling.

Authors:  Kun Miao; Lu Wei
Journal:  ACS Cent Sci       Date:  2020-03-06       Impact factor: 14.553

Review 6.  Dihedral Angle Measurements for Structure Determination by Biomolecular Solid-State NMR Spectroscopy.

Authors:  Patrick C A van der Wel
Journal:  Front Mol Biosci       Date:  2021-12-06
  6 in total

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