Scott Emmert, A. et al. Dis.Model Mech.12, pii: dmm040972 (2019)

Hydrocephalus, one of the most common congenital abnormalities affecting the nervous system, is defined by an excessive accumulation of cerebrospinal fluid (CSF) in the brain. Surgical treatments are available to prevent brain damage, but a better understanding of the condition could lead to new therapeutic strategies.

Investigators from Cincinnati Children’s Hospital Medical Center generated a rat model of hydrocephalus by using a CRISPR/Cas9 approach to introduce a gene mutation within the Ccdc39 gene. Ccdc39prh/prh mutants demonstrated progressive postnatal hydrocephalus, impaired neural differentiation and glymphatic-mediated CSF circulation, as well as increased inflammation responses compared with wild-type rats. Ccdc39prh/prh rats should be useful to understand the mechanisms underlying impaired neocorticogenesis, glymphatic fluid exchange and cognitive and motor development in neonatal hydrocephalus.