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Title

Junctional adhesion molecule (JAM)-C deficient C57BL/6 mice develop a severe hydrocephalus

Authors
Wyss, Lena
Schäfer, Julia
Liebner, Stefan
Mittelbronn, Michel
Deutsch, Urban
Enzmann, Gaby
Adams, Ralf H
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Published in PLOS ONE. 2012, vol. 7, no. 9, p. e45619
Abstract 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 3(rd) 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.
Keywords AnimalsBase SequenceDNA PrimersFluorescent Antibody TechniqueHydrocephalus/geneticsJunctional Adhesion Molecule C/genetics/physiologyMiceMice, Inbred C57BLMice, Transgenic
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PMID: 23029139
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Article (Published version) (6 MB) - public document Free access
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Research group Molécules d'adhésion et processus de migration cellulaire (167)
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WYSS, Lena et al. Junctional adhesion molecule (JAM)-C deficient C57BL/6 mice develop a severe hydrocephalus. In: PLOS ONE, 2012, vol. 7, n° 9, p. e45619. https://archive-ouverte.unige.ch/unige:31442

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Deposited on : 2013-11-27

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