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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...
Autores principales: | Iyer, Sangeetha, Mast, Joshua D., Tsang, Hillary, Rodriguez, Tamy P., DiPrimio, Nina, Prangley, Madeleine, Sam, Feba S., Parton, Zachary, Perlstein, Ethan O. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Company of Biologists Ltd
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6899034/ https://www.ncbi.nlm.nih.gov/pubmed/31615832 http://dx.doi.org/10.1242/dmm.040576 |
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