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          Institute: MPI für Hirnforschung     Collection: Neurochemistry (Heinrich Betz)     Display Documents



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ID: 10587.0, MPI für Hirnforschung / Neurochemistry (Heinrich Betz)
Rescue of molybdenum cofactor biosynthesis in gephyrin- deficient mice by a Cnx1 transgene
Authors:Grosskreutz, Y.; Betz, H.; Kneussel, M.
Language:English
Date of Publication (YYYY-MM-DD):2003
Title of Journal:Biochemical and Biophysical Research Communications
Journal Abbrev.:Biochem. Biophys. Res. Commun.
Volume:301
Issue / Number:2
Start Page:450
End Page:455
Review Status:Peer-review
Audience:Not Specified
Abstract / Description:Gephyrin is a bifunctional protein which is essential for both synaptic clustering of inhibitory neurotransmitter receptors in the central nervous system and the biosynthesis of the molybdenum cofactor (MoCo) in peripheral tissues. Mice deficient in gephyrin die early postnatally and display a loss of glycine receptors (GlyRs) and many GABA(A) receptor (GABA(A)R) subtypes from postsynaptic sites. In addition, the activities of the MoCo-dependent enzymes xanthine dehydrogenase and sulfite oxidase are reduced to background levels in the liver and intestine of these animals. To genetically separate the different consequences of gephyrin deficiency, we expressed a transgene of the plant homolog Cnxl, known to rescue mammalian MoCo deficiency, on the background of gephyrin knockout mice. Cnxl partially restored sulfite oxidase activity in the liver of the transgenic animals, whereas early lethality and the loss of GlyR clustering were unaltered. Our data suggest that the loss of neurotransmitter receptor clustering at inhibitory synapses causes the early lethality of gephyrin deficient mice. (C) 2003 Elsevier Science (USA). All rights reserved.
Free Keywords:molybdenum cofactor; gephyrin; glycine receptor; GABA(A) receptor; inhibitory synapse; sulfite oxidase; Cnx1
Comment of the Author/Creator:Date: 2003, FEB 7
External Publication Status:published
Document Type:Article
Communicated by:N. N.
Affiliations:MPI für Hirnforschung/Neurochemistry (Abt.: Betz)
Identifiers:ISI:000181099100030 [ID No:1]
ISSN:0006-291X [ID No:2]
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