TY - JOUR
T1 - Drosophila models of prionopathies
T2 - insight into prion protein function, transmission, and neurotoxicity
AU - Fernandez-Funez, Pedro
AU - Sanchez-Garcia, Jonatan
AU - Rincon-Limas, Diego E.
N1 - Publisher Copyright:
© 2017 Elsevier Ltd
PY - 2017/6/1
Y1 - 2017/6/1
N2 - Prion diseases (PrD) are unique neurodegenerative conditions with sporadic, genetic, and infectious etiologies. The agent responsible for these pathologies is a misfolded conformation of the prion protein (PrP). Although a process of autocatalytic “conversion” is known to mediate disease transmission, important gaps still remain regarding the physiological function of PrP and its relevance to pathogenesis, the molecular and cellular mechanisms mediating neurotoxicity and transmission, and the PrP conformations responsible for neurotoxicity. New Drosophila models expressing mammalian PrP have revealed physiological insight into PrP function and opened the door to significant progress in prion transmission and PrP neurotoxicity. Importantly, flies expressing human PrP showing a robust eye phenotype will allow performing genetic screens to uncover novel mechanisms mediating PrP neurotoxicity.
AB - Prion diseases (PrD) are unique neurodegenerative conditions with sporadic, genetic, and infectious etiologies. The agent responsible for these pathologies is a misfolded conformation of the prion protein (PrP). Although a process of autocatalytic “conversion” is known to mediate disease transmission, important gaps still remain regarding the physiological function of PrP and its relevance to pathogenesis, the molecular and cellular mechanisms mediating neurotoxicity and transmission, and the PrP conformations responsible for neurotoxicity. New Drosophila models expressing mammalian PrP have revealed physiological insight into PrP function and opened the door to significant progress in prion transmission and PrP neurotoxicity. Importantly, flies expressing human PrP showing a robust eye phenotype will allow performing genetic screens to uncover novel mechanisms mediating PrP neurotoxicity.
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U2 - 10.1016/j.gde.2017.03.013
DO - 10.1016/j.gde.2017.03.013
M3 - Review article
C2 - 28415023
AN - SCOPUS:85017477001
SN - 0959-437X
VL - 44
SP - 141
EP - 148
JO - Current Opinion in Genetics and Development
JF - Current Opinion in Genetics and Development
ER -