Literature DB >> 24880044

Microfluidic platform to evaluate migration of cells from patients with DYT1 dystonia.

Flavia C Nery1, Nadia A Atai1,2, Cintia C da Hora1, Edward Y Kim3, Jasmin Hettich1, Thorsten R Mempel3, Xandra O Breakefield1, Daniel Irimia4.   

Abstract

BACKGROUND: Microfluidic platforms for quantitative evaluation of cell biologic processes allow low cost and time efficient research studies of biological and pathological events, such as monitoring cell migration by real-time imaging. In healthy and disease states, cell migration is crucial in development and wound healing, as well as to maintain the body's homeostasis. NEW
METHOD: The microfluidic chambers allow precise measurements to investigate whether fibroblasts carrying a mutation in the TOR1A gene, underlying the hereditary neurologic disease--DYT1 dystonia, have decreased migration properties when compared to control cells.
RESULTS: We observed that fibroblasts from DYT1 patients showed abnormalities in basic features of cell migration, such as reduced velocity and persistence of movement. COMPARISON WITH EXISTING
METHOD: The microfluidic method enabled us to demonstrate reduced polarization of the nucleus and abnormal orientation of nuclei and Golgi inside the moving DYT1 patient cells compared to control cells, as well as vectorial movement of single cells.
CONCLUSION: We report here different assays useful in determining various parameters of cell migration in DYT1 patient cells as a consequence of the TOR1A gene mutation, including a microfluidic platform, which provides a means to evaluate real-time vectorial movement with single cell resolution in a three-dimensional environment.
Copyright © 2014 Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Cell polarization; DYT1; Dystonia; Microfluidic device; Migration; TorsinA

Mesh:

Substances:

Year:  2014        PMID: 24880044      PMCID: PMC4120252          DOI: 10.1016/j.jneumeth.2014.05.027

Source DB:  PubMed          Journal:  J Neurosci Methods        ISSN: 0165-0270            Impact factor:   2.390


  56 in total

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3.  4-Phenylbutyrate attenuates the ER stress response and cyclic AMP accumulation in DYT1 dystonia cell models.

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