Drug screens of NGLY1 deficiency in worm and fly models reveal catecholamine, NRF2 and anti-inflammatory-pathway activation as potential clinical approaches

N-glycanase 1 (NGLY1) deficiency is an ultra-rare and complex monogenic glycosylation disorder that affects fewer than 40 patients globally. NGLY1 deficiency has been studied in model organisms such as yeast, worms, flies and mice. Proteasomal and mitochondrial homeostasis gene networks are controll...

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Sparad:
Bibliografiska uppgifter
I publikationen:Dis Model Mech
Huvudupphovsmän: Iyer, Sangeetha, Mast, Joshua D., Tsang, Hillary, Rodriguez, Tamy P., DiPrimio, Nina, Prangley, Madeleine, Sam, Feba S., Parton, Zachary, Perlstein, Ethan O.
Materialtyp: Artigo
Språk:Inglês
Publicerad: The Company of Biologists Ltd 2019
Ämnen:
Research Article
Länkar:https://ncbi.nlm.nih.gov/pmc/articles/PMC6899034/
https://ncbi.nlm.nih.gov/pubmed/31615832
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/dmm.040576
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