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Cooperation

Institute of Bioorganic Chemistry,
Polish Academy of Sciences
Z. Noskowskiego 12/14
61-704 Poznań
tel centrala: (+48) 61 852 85 03
fax: (+48) 61 852 05 32
e-mail: ibch@ibch.poznan.pl

Sekretariat Dyrektora, tel: 61 852 89 19

Address

Department of Molecular Neurobiology

Dr. habil. Maciej Figiel

Head of Department
mfigiel@ibch.poznan.pl
ext. 1150

Karolina Świtońska MSc, BEng

PhD student

Joanna Delimata MSc
PhD student

Piotr Piasecki MSc
PhD student

Ewelina Jesion MSc

PhD student

Kalina Wiatr MSc, BEng
PhD student

Żaneta Kalinowska MSc
PhD student

PhD students:

  • Szlachcic WJ, Wiatr K, Trzeciak M, Figlerowicz M, Figiel M.The Generation of Mouse and Human Huntington Disease iPS Cells Suitable for In vitro Studies on Huntingtin Function.Front Mol Neurosci. 10:253 (2017)

  • Wiatr K, Szlachcic WJ, Trzeciak M, Figlerowicz M, Figiel M.Huntington disease as neurodevelopmental disorder and early phenotypes of the disease in stem cells.Mol Neurobiol. 2017; May 11. doi: 10.1007/s12035-017-0477-7.

  • Szlachcic WJ, Switonski PM, Kurkowiak M, Wiatr K, Figiel M.Mouse polyQ database: a new online resource for research using mouse models of neurodegenerative diseases.Mol Brain. 8(1):69 (2015)

  • Szlachcic WJ, Switonski PM, Krzyzosiak WJ, Figlerowicz M, Figiel M. Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway.Dis Model Mech. 8(9):1047-57 (2015)

  • Switonski PM, Szlachcic WJ, Krzyzosiak WJ, Figiel M.A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD.Neurobiol Dis.73:174-88 (2015)

  • Figiel M, Krzyzosiak WJ, Switonski PM, Szlachcic WJ.Chapter 64: Mouse Models of SCA3 and Other Polyglutamine Repeat Ataxias.In Movement Disorders, Second Edition. Ed. LeDoux M. ISBN: 9780124051959 DOI: 10.1016/B978-0-12-405195-9.00064-0 (2015)

Selected publications

  • Allele selective, CAG-targeted RNAi-based strategy to lower mutant polyQ proteins in polyglutamine ataxias; National Ataxia Foundation; Minneapolis, MN, USA; 688790; Pioneer SCA3/MJD Translational Research Award; Maciej Figiel and Yvon Trottier

  • Discovering disorders of brain development in Huntington disease as a consequence of possible total huntingtin deficiency in HD, using juvenile HD iPSC and fused brain organoids; National Science Center, Grant number: 2018/31/B/NZ3/03621

  • Allele-specific lowering of mutant polyQ proteins as treatment for Huntington disease, spinocerebellar ataxia type 3 and spinocerebellar ataxia type 7; National Centre for Research and Development; Grant number: ERA-NET-E-RARE-3/III/TreatPolyQ/08/2018

  • Gene Therapy for Cerebellar Ataxias: restoring cholesterol metabolism by targeting brain cholesterol 24 hydroxylase (CYP46A1); National Centre for Research and Development; Grant number: ERA-NET-E-RARE-3/III/SCA-CYP/09/2018

  • Defining new mechanisms of neurodegeneration using the first model of a knock-in SCA3 / MJD; National Science Center (Krakow); Grant number: 2013/10/E/NZ4/00621

Research activity

  • Neurodegenerative disorders,
  • neurodevelopmental disorders,
  • mouse models,
  • induced pluripotent stem cells,
  • iPSC, SCA3, MJD,
  • #spinocerebellar ataxia type 3,
  • Machado Joseph Disease,
  • Huntington disease

  • New mechanisms of neurodegeneration in poliq model diseases.

  • Characterization of SCA3/MJD KI91 knock-in mouse model.

  • Identification of changes in the level of mRNA and proteins, and modifications (phosphoproteins, ubiquitinated proteins) in different stages of diseases development in the KI91 model.

  • Behavioral characterization of the KI91 mice.

  • Identyfication of new molecular therapeutic targets in poliQ diseases.

  • Therapy of polyQ diseases.

  • Generation of iPSC models of neurodegenerativ diseases.

  • Neurodegenerative disease = neurodevelopmental disease. The study of the pathogenesis 

  • of neurodegenerativ disease on the development axis: stem cells - neuronal stem cells - mature neuronal cells.

  • Identyfication and validation of RNA species with altered expression level in human Huntington disease iPSC.

Research area

Keywords

Research Projects

Ewelina Jesion MSc

biologist

Technical Staff:

Urszula Kozłowska
adiunkt

Dr. Bart Krist
asystent

Dr. Magdalena Surdyka
adiunkt

Researchers:

Jakub Kubiś
PhD student