Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2

Highlights • Mice lacking Pcdp1 and Spef2 have hydrocephalus with damage to multiple cell types. • Morphological defects are more severe on the C57BL/6J background than 129S6/SvEvTac. • Mutants have a defect in cilia-driven cerebrospinal fluid flow on both strains. • Genetic modifiers likely influen...

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Veröffentlicht in:	Neuroscience 2014-09, Vol.277, p.552-567
Hauptverfasser:	Finn, R, Evans, C.C, Lee, L
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Brain - pathology Brain - physiopathology Cerebrospinal Fluid - physiology Cfap221 cilia Disease Models, Animal Genetic Predisposition to Disease hydrocephalus Hydrocephalus - pathology Hydrocephalus - physiopathology Immunohistochemistry Kartagener Syndrome - pathology Kartagener Syndrome - physiopathology Mice, 129 Strain Mice, Inbred C57BL Mice, Transgenic Mutation Neurology Neurons - pathology Neurons - physiology Oligodendroglia - pathology Oligodendroglia - physiology Pcdp1 primary ciliary dyskinesia Proteins - genetics Proteins - metabolism Severity of Illness Index Species Specificity Spef2
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Zusammenfassung:	Highlights • Mice lacking Pcdp1 and Spef2 have hydrocephalus with damage to multiple cell types. • Morphological defects are more severe on the C57BL/6J background than 129S6/SvEvTac. • Mutants have a defect in cilia-driven cerebrospinal fluid flow on both strains. • Genetic modifiers likely influence the severity of hydrocephalus in these models.
ISSN:	0306-4522 1873-7544
DOI:	10.1016/j.neuroscience.2014.07.029