Functional evaluation of human stem cell-derived striatal medium spiny and cortical neurons in control and in Huntington’s disease

Functional evaluation of human stem cell-derived striatal medium spiny and cortical neurons in control and in Huntington’s disease

Research Group: Gerardo Biella, Mauro Toselli

Neurodegenerative diseases are characterized by selective degeneration of cells in the nervous system. In Huntington’s disease (HD) has been described the degeneration of the medium spiny neurons (MSNs) of the striatum. At present does not exist a therapy to cure HD. A possible therapeutic approach is the cell replacement therapy. The differentiation of stem cell towards MSNs could be a good source of cell. We use physiological method (patch clamp) to define the functional properties of stem cell differentiated towards striatal neuron. The same protocol is also applied to differentiate stem cell (iPSCs) derived from HD patients. These MSNs are used to model in vitro the disease and to identify a possible physiological readouts.

Recent Publications:

  • Onorati M, Castiglioni V, Biasci D,  Cesana E, Menon R, Vuono R, Talpo F, Goya RL, Lyons PA,Bulfamante GP, Muzio L, Martino G, Toselli M, Farina C, Barker RA, Biella G, Cattaneo E Molecular and Functional Definition of the Developing Human Striatum. Nature neuroscience. 2014 Dec;17(12):1804-15. doi: 10.1038/nn.3860. Epub 2014 Nov 10 NC: 18
  • Carri A, Onorati M, Lelos MJ, Castiglioni V, Faedo A, Menon R, Camnasio S, Vuono R, Spaiardi P, Talpo F, Toselli M, Martino G, Barker RA, Dunnett SB, Biella G, Cattaneo E. Developmentally coordinated extrinsic signals drive human pluripotent stem cell differentiation toward authentic DARPP-32+ medium-sized spiny neurons. Development. 2013 Jan 15;140(2):301-12. doi: 10.1242/dev.084608. PubMed PMID: 23250204. NC: 86
  • Biella G, Di Febo F, Goffredo D, Moiana A, Taglietti V, Conti L, Cattaneo E, Toselli M . Differentiating embryonic stem-derived neural stem cells show a maturation-dependent pattern of voltage-gated sodium current expression and graded action potentials. Neuroscience. 2007 Oct 12;149(1):38-52, ISSN: 0306-4522. NC: 40