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...

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Detalhes bibliográficos

Principais autores:	Alberto Rissone, Erin Jimenez, Kevin Bishop, Blake Carrington, Claire Slevin, Stephen M. Wincovitch, Raman Sood, Fabio Candotti, Shawn M. Burgess
Formato:	Artigo
Idioma:	Inglês
Publicado em:	The Company of Biologists 2019-12-01
coleção:	Disease Models & Mechanisms
Assuntos:	ak2 reticular dysgenesis scid hearing loss zebrafish hair cells lateral line antioxidants
Acesso em linha:	http://dmm.biologists.org/content/12/12/dmm040170
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