Research Topics
Genomes and Genes | Claudia StendelSummaryAffiliation: Swiss Federal Institute of Technology Country: Switzerland Publications
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Detail Information
Publications
SH3TC2, a protein mutant in Charcot-Marie-Tooth neuropathy, links peripheral nerve myelination to endosomal recyclingClaudia Stendel
Institute of Cell Biology, Department of Biology, ETH Zurich, Zurich, Switzerland
Brain 133:2462-74. 2010..Our data indicate that the SH3TC2/Rab11 interaction is relevant for peripheral nerve pathophysiology and place endosomal recycling on the list of cellular mechanisms involved in Schwann cell myelination...
Peripheral nerve demyelination caused by a mutant Rho GTPase guanine nucleotide exchange factor, frabin/FGD4Claudia Stendel
Institute of Cell Biology, ETH Zurich, Schafmattstrasse 18, CH 8093 Zurich, Switzerland
Am J Hum Genet 81:158-64. 2007..These data, together with the ability of frabin to induce Cdc42-mediated cell-shape changes in transfected Schwann cells, suggest that Rho GTPase signaling is essential for proper myelination of the peripheral nervous system...
Autosomal-dominant distal myopathy associated with a recurrent missense mutation in the gene encoding the nuclear matrix protein, matrin 3Jan Senderek
Institute of Cell Biology, ETH Zurich, 8093 Zurich, Switzerland
Am J Hum Genet 84:511-8. 2009..Our data establish proof of principle that the nuclear matrix is crucial for normal skeletal muscle structure and function and put VCPDM on the growing list of monogenic disorders associated with the nuclear proteome...
Small Rho GTPases are key regulators of peripheral nerve biology in health and diseaseSven Krause
Institute of Cell Biology, Department of Biology, ETH Zurich, Zurich, Switzerland
J Peripher Nerv Syst 13:188-99. 2008....
Mutations in a gene encoding a novel SH3/TPR domain protein cause autosomal recessive Charcot-Marie-Tooth type 4C neuropathyJan Senderek
Department of Human Genetics, Aachen University of Technology, Aachen, Germany
Am J Hum Genet 73:1106-19. 2003..Comparative sequence alignments indicate that members of this protein family contain multiple SH3 and TPR domains that are likely involved in the formation of protein complexes...
Cloning, expression and characterization of the murine orthologue of SBF2, the gene mutated in Charcot-Marie-Tooth disease type 4B2Jutta Kirfel
Institute of Pathology, University of Bonn, Bonn, Germany
Gene Expr Patterns 6:978-84. 2006..Taken together, this study provides information about the mRNA expression and subcellular localization of Sbf2 and as such helps in further understanding its function in development and disease...
