TY  - JOUR
A1  - Wyss, Lena
A1  - Schäfer, Julia
A1  - Liebner, Stefan
A1  - Mittelbronn, Michel Guy André
A1  - Deutsch, Urban
A1  - Enzmann, Gaby
A1  - Adams, Ralf Heinrich
A1  - Aurrand-Lions, Michel
A1  - Plate, Karl
A1  - Imhof, Beat A.
A1  - Engelhardt, Britta
T1  - Junctional adhesion molecule (JAM)-C deficient C57BL/6 mice develop a severe hydrocephalus
T2  - PLoS One
N2  - The junctional adhesion molecule (JAM)-C is a widely expressed adhesion molecule regulating cell adhesion, cell polarity and inflammation. JAM-C expression and function in the central nervous system (CNS) has been poorly characterized to date. Here we show that JAM-C−/− mice backcrossed onto the C57BL/6 genetic background developed a severe hydrocephalus. An in depth immunohistochemical study revealed specific immunostaining for JAM-C in vascular endothelial cells in the CNS parenchyma, the meninges and in the choroid plexus of healthy C57BL/6 mice. Additional JAM-C immunostaining was detected on ependymal cells lining the ventricles and on choroid plexus epithelial cells. Despite the presence of hemorrhages in the brains of JAM-C−/− mice, our study demonstrates that development of the hydrocephalus was not due to a vascular function of JAM-C as endothelial re-expression of JAM-C failed to rescue the hydrocephalus phenotype of JAM-C−/− C57BL/6 mice. Evaluation of cerebrospinal fluid (CSF) circulation within the ventricular system of JAM-C−/− mice excluded occlusion of the cerebral aqueduct as the cause of hydrocephalus development but showed the acquisition of a block or reduction of CSF drainage from the lateral to the 3rd ventricle in JAM-C−/− C57BL/6 mice. Taken together, our study suggests that JAM-C−/− C57BL/6 mice model the important role for JAM-C in brain development and CSF homeostasis as recently observed in humans with a loss-of-function mutation in JAM-C.
Y1  - 2012
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/26344
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-263445
SN  - 1932-6203
VL  - 7
IS  - (9):e45619
PB  - PLoS
CY  - Lawrence, Kan.
ER  -