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A model for reticular dysgenesis shows impaired sensory organ development and hair cell regeneration linked to cellular stress
Mutations in the gene AK2 are responsible for reticular dysgenesis (RD), a rare and severe form of primary immunodeficiency in children. RD patients have a severely shortened life expectancy and without treatment die, generally from sepsis soon after birth. The only available therapeutic option for...
Autores principales: | Rissone, Alberto, Jimenez, Erin, Bishop, Kevin, Carrington, Blake, Slevin, Claire, Wincovitch, Stephen M., Sood, Raman, Candotti, Fabio, Burgess, Shawn M. |
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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/PMC6955229/ https://www.ncbi.nlm.nih.gov/pubmed/31727854 http://dx.doi.org/10.1242/dmm.040170 |
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