SMO-M2 mutation does not support cell-autonomous Hedgehog activity in cerebellar granule cell precursors

Media type: E-Article
Title: SMO-M2 mutation does not support cell-autonomous Hedgehog activity in cerebellar granule cell precursors
Contributor: Petroni, Marialaura; Sahùn Roncero, Maria; Ramponi, Valentina; Fabretti, Francesca; Nicolis Di Robilant, Vittoria; Moretti, Marta; Alfano, Vincenzo; Corsi, Alessandro; De Panfilis, Simone; Giubettini, Maria; Di Giulio, Stefano; Capalbo, Carlo; Belardinilli, Francesca; Coppa, Anna; Sardina, Francesca; Colicchia, Valeria; Pedretti, Flaminia; Infante, Paola; Cardinali, Beatrice; Tessitore, Alessandra; Canettieri, Gianluca; De Smaele, Enrico; Giannini, Giuseppe
imprint: Springer Science and Business Media LLC, 2019
Published in: Scientific Reports
Language: English
DOI: 10.1038/s41598-019-56057-y
ISSN: 2045-2322
Keywords: Multidisciplinary
Origination:
Footnote:
Description: <jats:title>Abstract</jats:title><jats:p>Growth and patterning of the cerebellum is compromised if granule cell precursors do not properly expand and migrate. During embryonic and postnatal cerebellar development, the Hedgehog pathway tightly regulates granule cell progenitors to coordinate appropriate foliation and lobule formation. Indeed, granule cells impairment or defects in the Hedgehog signaling are associated with developmental, neurodegenerative and neoplastic disorders. So far, scant and inefficient cellular models have been available to study granule cell progenitors, <jats:italic>in vitro</jats:italic>. Here, we validated a new culture method to grow postnatal granule cell progenitors as hedgehog-dependent neurospheres with prolonged self-renewal and ability to differentiate into granule cells, under appropriate conditions. Taking advantage of this cellular model, we provide evidence that Ptch1-KO, but not the SMO-M2 mutation, supports constitutive and cell-autonomous activity of the hedgehog pathway.</jats:p>
Access State: Open Access

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