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Information on the principal investigator and host institution

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Defining new neurodegeneration mechanisms using the first SCA3/MJD knock-in mouse model.

2013/10/E/NZ4/00621

Keywords:

neurodegeneration mouse model knock-in SCA3 MJD ataxin-3 ataxia,

Descriptors:

  • NZ3_5: Molecular neurobiology
  • NZ3_6: Cell neurobiology
  • NZ4_9: Systems neurobiology

Panel:

NZ4 - Biology of tissues, organs and organisms: morphology and functions of animal's and human's systems, organs and organisms, experimental medicine, basics of neurology

Host institution :

Instytut Chemii Bioorganicznej PAN

woj. wielkopolskie

Other projects carried out by the institution 

Principal investigator (from the host institution):

dr hab. Maciej Figiel 

Number of co-investigators in the project: 6

Call: SONATA BIS 3 - announced on 2013-06-14

Amount awarded: 1 498 900 PLN

Project start date (Y-m-d): 2014-07-08

Project end date (Y-m-d): 2020-07-07

Project duration:: 72 months (the same as in the proposal)

Project status: Project settled

Information in the final report

  • Publication in academic press/journals (7)
  1. Identification of Altered Developmental Pathways in Human Juvenile HD iPSC With 71Q and 109Q Using Transcriptome Profiling
    Authors:
    Świtońska K, Szlachcic WJ, Handschuh L, Wojciechowski P, Marczak Ł, Stelmaszczuk M, Figlerowicz M, Figiel M
    Academic press:
    Frontiers in Cellular Neuroscience (rok: 2019, tom: 12, strony: 528), Wydawca: Frontiers
    Status:
    Published
    DOI:
    10.3389/fncel.2018.00528 - link to the publication
  2. Mouse polyQ database: a new online resource for research using mouse models of neurodegenerative diseases.
    Authors:
    Wojciech J. Szlachcic, Pawel M. Switonski, Malgorzata Kurkowiak, Kalina Wiatr, Maciej Figiel
    Academic press:
    Molecular Brain (rok: 2015, tom: 8(1), strony: 69), Wydawca: BioMed Central
    Status:
    Published
    DOI:
    10.1186/s13041-015-0160-8 - link to the publication
  3. Broad Influence of Mutant Ataxin-3 on the Proteome of the Adult Brain, Young Neurons, and Axons Reveals Central Molecular Processes and Biomarkers in SCA3/MJD Using Knock-In Mouse Model
    Authors:
    Kalina Wiatr, Łukasz Marczak, Jean-Baptiste Perot, Emmanuel Brouillet, Julien Flament, Maciej Figiel
    Academic press:
    Frontiers in Molecular Neuroscience (rok: 2021, tom: 14:658339, strony: -), Wydawca: Frontiers Media SA
    Status:
    Published
    DOI:
    10.3389/fnmol.2021.658339 - link to the publication
  4. Altered Levels of Proteins and Phosphoproteins, in the Absence of Early Causative Transcriptional Changes, Shape the Molecular Pathogenesis in the Brain of Young Presymptomatic Ki91 SCA3/MJD Mouse
    Authors:
    Wiatr K, Piasecki P, Marczak Ł, Wojciechowski P, Kurkowiak M, Płoski R, Rydzanicz M, Handschuh L, Jungverdorben J, Brüstle O, Figlerowicz M, Figiel M
    Academic press:
    Molecular Neurobiology (rok: 2019, tom: 56(12), strony: 8168-8202), Wydawca: Springer Nature
    Status:
    Published
    DOI:
    10.1007/s12035-019-01643-4 - link to the publication
  5. Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway
    Authors:
    Wojciech J. Szlachcic, Pawel M. Switonski, Wlodzimierz J. Krzyzosiak, Marek Figlerowicz, Maciej Figiel
    Academic press:
    Disease Models and Mechanisms (rok: 2015, tom: 8, strony: 1047-1057), Wydawca: The Company of Biologists
    Status:
    Published
    DOI:
    10.1242/dmm.019406 - link to the publication
  6. The Generation of Mouse and Human Huntington Disease iPS Cells Suitable for In vitro Studies on Huntingtin Function
    Authors:
    Wojciech J. Szlachcic, Kalina Wiatr, Marta Trzeciak, Marek Figlerowicz and Maciej Figiel
    Academic press:
    Frontiers in Molecular Neuroscience (rok: 2017, tom: 10, strony: Article 253), Wydawca: Frontiers
    Status:
    Published
    DOI:
    10.3389/fnmol.2017.00253 - link to the publication
  7. Huntington disease as a neurodevelopmental disorder and early signs of the disease in stem cells.
    Authors:
    Kalina Wiatr, Wojciech J. Szlachcic, Marta Trzeciak, Marek Figlerowicz, Maciej Figiel
    Academic press:
    Molecular Neurobiology (rok: 2018, tom: 55, strony: 3351-3371), Wydawca: SpringerNature
    Status:
    Published
    DOI:
    10.1007/s12035-017-0477-7 - link to the publication

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