Literature DB >> 33625583

Huntington's disease: lessons from prion disorders.

Melanie Alpaugh1,2, Francesca Cicchetti3,4.   

Abstract

Decades of research on the prion protein and its associated diseases have caused a paradigm shift in our understanding of infectious agents. More recent years have been marked by a surge of studies supporting the application of these findings to a broad array of neurodegenerative disorders such as Alzheimer's and Parkinson's diseases. Here, we present evidence to suggest that Huntington's disease, a monogenic disorder of the central nervous system, shares features with prion disorders and that, it too, may be governed by similar mechanisms. We further posit that these similarities could suggest that, like other common neurodegenerative disorders, sporadic forms of Huntington's disease may exist.

Entities:  

Keywords:  Huntingtin; Huntington’s disease-like; Prion-like; Tissue spongiform encephalopathies

Year:  2021        PMID: 33625583     DOI: 10.1007/s00415-021-10418-8

Source DB:  PubMed          Journal:  J Neurol        ISSN: 0340-5354            Impact factor:   4.849


  123 in total

1.  Length of Uninterrupted CAG, Independent of Polyglutamine Size, Results in Increased Somatic Instability, Hastening Onset of Huntington Disease.

Authors:  Galen E B Wright; Jennifer A Collins; Chris Kay; Cassandra McDonald; Egor Dolzhenko; Qingwen Xia; Kristina Bečanović; Britt I Drögemöller; Alicia Semaka; Charlotte M Nguyen; Brett Trost; Fiona Richards; Emilia K Bijlsma; Ferdinando Squitieri; Colin J D Ross; Stephen W Scherer; Michael A Eberle; Ryan K C Yuen; Michael R Hayden
Journal:  Am J Hum Genet       Date:  2019-05-16       Impact factor: 11.025

2.  Evidence for the spread of human-derived mutant huntingtin protein in mice and non-human primates.

Authors:  Philippe Gosset; Alexander Maxan; Melanie Alpaugh; Ludivine Breger; Benjamin Dehay; Zhu Tao; Zhang Ling; Chuan Qin; Giulia Cisbani; Nadia Fortin; Jean-Paul G Vonsattel; Steve Lacroix; Abid Oueslati; Erwan Bezard; Francesca Cicchetti
Journal:  Neurobiol Dis       Date:  2020-05-11       Impact factor: 5.996

3.  Protein misfolding detected early in pathogenesis of transgenic mouse model of Huntington disease using amyloid seeding assay.

Authors:  Sharad Gupta; Shy'Ann Jie; David W Colby
Journal:  J Biol Chem       Date:  2011-12-20       Impact factor: 5.157

4.  A polymorphic DNA marker genetically linked to Huntington's disease.

Authors:  J F Gusella; N S Wexler; P M Conneally; S L Naylor; M A Anderson; R E Tanzi; P C Watkins; K Ottina; M R Wallace; A Y Sakaguchi
Journal:  Nature       Date:  1983 Nov 17-23       Impact factor: 49.962

5.  Transcellular spreading of huntingtin aggregates in the Drosophila brain.

Authors:  Daniel T Babcock; Barry Ganetzky
Journal:  Proc Natl Acad Sci U S A       Date:  2015-09-08       Impact factor: 11.205

6.  mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington's Disease.

Authors:  Anne Ast; Alexander Buntru; Franziska Schindler; Regine Hasenkopf; Aline Schulz; Lydia Brusendorf; Konrad Klockmeier; Gerlinde Grelle; Benjamin McMahon; Hannah Niederlechner; Isabelle Jansen; Lisa Diez; Juliane Edel; Annett Boeddrich; Sophie A Franklin; Barbara Baldo; Sigrid Schnoegl; Severine Kunz; Bettina Purfürst; Annette Gaertner; Harm H Kampinga; A Jennifer Morton; Åsa Petersén; Janine Kirstein; Gillian P Bates; Erich E Wanker
Journal:  Mol Cell       Date:  2018-09-06       Impact factor: 17.970

7.  Huntington's disease cerebrospinal fluid seeds aggregation of mutant huntingtin.

Authors:  Z Tan; W Dai; T G M van Erp; J Overman; A Demuro; M A Digman; A Hatami; R Albay; E M Sontag; K T Potkin; S Ling; F Macciardi; W E Bunney; J D Long; J S Paulsen; J M Ringman; I Parker; C Glabe; L M Thompson; W Chiu; S G Potkin
Journal:  Mol Psychiatry       Date:  2015-06-23       Impact factor: 15.992

8.  Cell-to-cell Transmission of Polyglutamine Aggregates in C. elegans.

Authors:  Dong-Kyu Kim; Kyu-Won Cho; Woo Jung Ahn; Dayana Perez-Acuña; Hyunsu Jeong; He-Jin Lee; Seung-Jae Lee
Journal:  Exp Neurobiol       Date:  2017-12-11       Impact factor: 3.261

9.  Demonstration of prion-like properties of mutant huntingtin fibrils in both in vitro and in vivo paradigms.

Authors:  Maria Masnata; Giacomo Sciacca; Alexander Maxan; Luc Bousset; Hélèna L Denis; Florian Lauruol; Linda David; Martine Saint-Pierre; Jeffrey H Kordower; Ronald Melki; Melanie Alpaugh; Francesca Cicchetti
Journal:  Acta Neuropathol       Date:  2019-02-20       Impact factor: 17.088

10.  Human-to-mouse prion-like propagation of mutant huntingtin protein.

Authors:  Iksoo Jeon; Francesca Cicchetti; Giulia Cisbani; Suji Lee; Endan Li; Jiwoo Bae; Nayeon Lee; Ling Li; Wooseok Im; Manho Kim; Hyun Sook Kim; Seung-Hun Oh; Tae-Aug Kim; Jung Jae Ko; Benoit Aubé; Abid Oueslati; Yun Joong Kim; Jihwan Song
Journal:  Acta Neuropathol       Date:  2016-05-24       Impact factor: 17.088

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