title: Muscle-specific expression of a dominant negative desmin mutant in transgenic mice.
abstract: To extend our knowledge of the functions of desmin and vimentin intermediate filaments in the developing organism, a construct encoding a truncated desmin subunit driven by the desmin promoter (pDDV), was introduced into the murine germ line. The resulting mutant desmin subunit was assembly-incompetent and capable of disrupting both preexisting desmin and vimentin filaments in a dominant negative fashion in transfected C2C12 muscle cells and in transgenic mouse muscle tissue. Expression of the pDDV was tissue-specific in transgenic mice. High level expression of pDDV occurred in a small percentage of desmin-containing muscle cells. Immunohistochemical staining of muscle tissue showed a diffuse desmin pattern instead of the dots and clumps into which mutant desmin typically accumulates in undifferentiated C2C12 muscle cells in tissue culture. Disruption of the endogenous desmin filaments in Sartorius muscle results in ultrastructural abnormalities.
authors.affiliation: Department of Biochemistry, University of Nijmegen, The Netherlands.
authors.forename: J M
authors.initials: JM
authors.lastname: Raats
authors.forename: G
authors.initials: G
authors.lastname: Schaart
authors.forename: J B
authors.initials: JB
authors.lastname: Henderik
authors.forename: A
authors.initials: A
authors.lastname: van der Kemp
authors.forename: I
authors.initials: I
authors.lastname: Dunia
authors.forename: E L
authors.initials: EL
authors.lastname: Benedetti
authors.forename: F R
authors.initials: FR
authors.lastname: Pieper
authors.forename: F C
authors.initials: FC
authors.lastname: Ramaekers
authors.forename: H
authors.initials: H
authors.lastname: Bloemendal
mesh_terms: D000818:Animals; D003893:Desmin / Q000235:genetics* / Q000502:physiology; D019084:Fluorescent Antibody Technique, Indirect; D051379:Mice; D008822:Mice, Transgenic; D009132:Muscles / Q000502:physiology* / Q000648:ultrastructure; D016296:Mutagenesis; D010957:Plasmids / Q000378:metabolism; D014162:Transfection; D014746:Vimentin / Q000378:metabolism*
chemical_list: D003893:Desmin; D014746:Vimentin