Progenitor death drives retinal dysplasia and neuronal degeneration in a mouse model of ATRIP-Seckel syndrome

Progenitor death drives retinal dysplasia and neuronal degeneration in a mouse model of ATRIP-Seckel syndrome

Seckel syndrome is a type of microcephalic primordial dwarfism (MPD) that is characterized by growth retardation and neurodevelopmental defects, including reports of retinopathy. Mutations in key mediators of the replication stress response, the mutually dependent partners ATR and ATRIP, are among t...

Full description

Bibliographic Details
Main Authors: Gabriel E. Matos-Rodrigues, Pedro B. Tan, Maurício Rocha-Martins, Clara F. Charlier, Anielle L. Gomes, Felipe Cabral-Miranda, Paulius Grigaravicius, Thomas G. Hofmann, Pierre-Olivier Frappart, Rodrigo A. P. Martins
Format: Article
Language:English
Published: The Company of Biologists 2020-10-01
Series:Disease Models & Mechanisms
Subjects:
apoptosis
dna damage response
neurodevelopment
neurodegeneration
visual system development
photoreceptor
Online Access:http://dmm.biologists.org/content/13/10/dmm045807

Internet

http://dmm.biologists.org/content/13/10/dmm045807

Similar Items