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Neurons Induced From Fibroblasts of c9ALS/FTD Patients Reproduce the Pathology Seen in the Central Nervous System

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023884%3A_____%2F19%3A00008233" target="_blank" >RIV/00023884:_____/19:00008233 - isvavai.cz</a>

  • Result on the web

    <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6743368/" target="_blank" >https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6743368/</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.3389/fnins.2019.00935" target="_blank" >10.3389/fnins.2019.00935</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Neurons Induced From Fibroblasts of c9ALS/FTD Patients Reproduce the Pathology Seen in the Central Nervous System

  • Original language description

    Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are incurable neurodegenerative conditions. A non-coding hexanucleotide (GGGGCC) repeat expansion in the c9orf72 gene is the most common genetic cause of ALS/FTD. We present a cellular model of c9ALS/FTD where induced neurons (iNeurons) are generated within 2 weeks by direct conversion of patients' dermal fibroblasts through down-regulation of polypyrimidine-tract-binding protein 1 (PTB1). While sense (S) and antisense (AS) intranuclear RNA foci were observed in both fibroblasts and iNeurons, the accumulation of (S) and (AS) repeat-associated non-ATG translation (RANT) products were detected only in iNeurons. Importantly, anti-sense oligonucleotides (ASOs) against the (5) repeat transcript lead to decreased (5) RNA foci staining and a reduction of the corresponding RANT products without affecting its (AS) counterparts. ASOs treatment also rescued the cell viability upon stressful stimulus. The results indicate that iNeurons is an advantageous model that not only recapitulates c9ALS/FTD hallmark features but can also help uncover promising therapeutics.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30103 - Neurosciences (including psychophysiology)

Result continuities

  • Project

  • Continuities

    N - Vyzkumna aktivita podporovana z neverejnych zdroju

Others

  • Publication year

    2019

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Frontiers in Neuroscience

  • ISSN

    1662-453X

  • e-ISSN

  • Volume of the periodical

    13

  • Issue of the periodical within the volume

    September

  • Country of publishing house

    CH - SWITZERLAND

  • Number of pages

    10

  • Pages from-to

  • UT code for WoS article

    000484547600001

  • EID of the result in the Scopus database

    2-s2.0-85072840163