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  • Pircs, KarolinaLund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Wallenberg Neurocentrum, Lund,Medicinska fakulteten,Molecular Neurogenetics,Lund University Research Groups,Wallenberg Neuroscience Centre, Lund,Faculty of Medicine (author)

Distinct subcellular autophagy impairments in induced neurons from patients with Huntington's disease

  • Article/chapterEnglish2022

Publisher, publication year, extent ...

  • 2021-12-22
  • Oxford University Press (OUP),2022

Numbers

  • LIBRIS-ID:oai:lup.lub.lu.se:7b6bcad7-d1ac-49f3-b38e-2624e13c2988
  • https://lup.lub.lu.se/record/7b6bcad7-d1ac-49f3-b38e-2624e13c2988URI
  • https://doi.org/10.1093/brain/awab473DOI

Supplementary language notes

  • Language:English
  • Summary in:English

Part of subdatabase

Classification

  • Subject category:art swepub-publicationtype
  • Subject category:ref swepub-contenttype

Notes

  • Huntington's disease (HD) is a neurodegenerative disorder caused by CAG expansions in the huntingtin (HTT) gene. Modelling Huntington's disease is challenging, as rodent and cellular models poorly recapitulate the disease as seen in aging humans. To address this, we generated induced neurons (iNs) through direct reprogramming of human skin fibroblasts, which retain age-dependent epigenetic characteristics. HD-iNs displayed profound deficits in autophagy, characterised by reduced transport of late autophagic structures from the neurites to the soma. These neurite-specific alterations in autophagy resulted in shorter, thinner and fewer neurites specifically in HD-iNs. CRISPRi-mediated silencing of HTT did not rescue this phenotype but rather resulted in additional autophagy alterations in ctrl-iNs, highlighting the importance of wild type HTT in normal neuronal autophagy. In summary, our work identifies a distinct subcellular autophagy impairment in adult patient derived Huntington's disease neurons and provides a new rational for future development of autophagy activation therapies.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Drouin-Ouellet, JanelleLund University,Lunds universitet,Utvecklings- och regenerativ neurobiologi,Forskargrupper vid Lunds universitet,Developmental and Regenerative Neurobiology,Lund University Research Groups,University of Montreal(Swepub:lu)med-jdn (author)
  • Horváth, VivienLund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Molecular Neurogenetics,Lund University Research Groups(Swepub:lu)vi3151ho (author)
  • Gil, JeovanisLund University,Lunds universitet,Tumörmikromiljö,Sektion I,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Klinisk kemi, Malmö,Forskargrupper vid Lunds universitet,Biomarkörer och Epi,Institutionen för kliniska vetenskaper, Lund,Tumor microenvironment,Section I,Department of Clinical Sciences, Lund,Faculty of Medicine,Clinical Chemistry, Malmö,Lund University Research Groups,Biomarkers and epidemiology,Department of Clinical Sciences, Lund(Swepub:lu)je2062gi (author)
  • Rezeli, MelindaLund University,Lunds universitet,Avdelningen för Biomedicinsk teknik,Institutionen för biomedicinsk teknik,Institutioner vid LTH,Lunds Tekniska Högskola,Masspektrometri,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,BioMS,Forskargrupper vid Lunds universitet,Clinical Protein Science and Imaging,Biomarkörer och Epi,Sektion I,Institutionen för kliniska vetenskaper, Lund,Department of Biomedical Engineering,Departments at LTH,Faculty of Engineering, LTH,Mass Spectrometry,Section V,Department of Clinical Sciences, Lund,Faculty of Medicine,Lund University Research Groups,Biomarkers and epidemiology,Section I,Department of Clinical Sciences, Lund(Swepub:lu)elma-mrl (author)
  • Garza, RaquelLund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Molecular Neurogenetics,Lund University Research Groups(Swepub:lu)ra7555ga (author)
  • Grassi, Daniela ALund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Molecular Neurogenetics,Lund University Research Groups(Swepub:lu)med-dgi (author)
  • Sharma, YogitaLund University,Lunds universitet,Utvecklings- och regenerativ neurobiologi,Forskargrupper vid Lunds universitet,Developmental and Regenerative Neurobiology,Lund University Research Groups(Swepub:lu)yo4230sh (author)
  • St-Amour, IsabelleLaval University (author)
  • Harris, KateUniversity of Cambridge (author)
  • Jönsson, Marie ELund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Molecular Neurogenetics,Lund University Research Groups(Swepub:lu)mphy-mjo (author)
  • Johansson, Pia ALund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Molecular Neurogenetics,Lund University Research Groups(Swepub:lu)pi2506jo (author)
  • Vuono, RominaUniversity of Cambridge (author)
  • Fazal, Shaline VUniversity of Cambridge (author)
  • Stoker, ThomasUniversity of Cambridge (author)
  • Hersbach, Bob A (author)
  • Sharma, KritikaLund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Molecular Neurogenetics,Lund University Research Groups(Swepub:lu)kr1766sh (author)
  • Lagerwall, Jessica (author)
  • Lagerström, StinaLund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Molecular Neurogenetics,Lund University Research Groups(Swepub:lu)st3263la (author)
  • Storm, PetterLund University,Lunds universitet,Utvecklings- och regenerativ neurobiologi,Forskargrupper vid Lunds universitet,Developmental and Regenerative Neurobiology,Lund University Research Groups(Swepub:lu)med-psm (author)
  • Hébert, Sébastien S (author)
  • Marko-Varga, GyörgyLund University,Lunds universitet,Avdelningen för Biomedicinsk teknik,Institutionen för biomedicinsk teknik,Institutioner vid LTH,Lunds Tekniska Högskola,Clinical Protein Science and Imaging,Forskargrupper vid Lunds universitet,Department of Biomedical Engineering,Departments at LTH,Faculty of Engineering, LTH,Lund University Research Groups(Swepub:lu)akem-gmv (author)
  • Parmar, MalinLund University,Lunds universitet,Utvecklings- och regenerativ neurobiologi,Forskargrupper vid Lunds universitet,Developmental and Regenerative Neurobiology,Lund University Research Groups(Swepub:lu)mphy-mpa (author)
  • Barker, Roger ALund University,Lunds universitet,Utvecklings- och regenerativ neurobiologi,Forskargrupper vid Lunds universitet,Developmental and Regenerative Neurobiology,Lund University Research Groups,University of Cambridge(Swepub:lu)med-rob (author)
  • Jakobsson, JohanLund University,Lunds universitet,Molekylär neurogenetik,Forskargrupper vid Lunds universitet,Molecular Neurogenetics,Lund University Research Groups(Swepub:lu)mphy-jja (author)
  • Molekylär neurogenetikForskargrupper vid Lunds universitet (creator_code:org_t)

Related titles

  • In:Brain : a journal of neurology: Oxford University Press (OUP)145:9, s. 3035-30571460-2156
  • In:Brain: Oxford University Press (OUP)145:9, s. 3035-30570006-8950

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