title: Effects of desmin gene knockout on mice heart mitochondria.
abstract: In heart tissue from mice lacking the intermediate filament (IF) desmin, mitochondria show an abnormal shape and distribution (Thornell et al., 1997). In the present study we have isolated heart mitochondria from desmin null (D-/-) and control (D+/+) mice, and analyzed their composition by SDS-PAGE, immunoblotting, and enzyme measurements. We found both in vitro and in situ that the conventional kinesin, the microtubule-associated plus-end directed motor, was frequently associated with D+/+ heart mitochondria, but not with D-/- heart mitochondria, suggesting that the positioning of mitochondria in heart is a dynamic event involving the IF desmin, the molecular motor kinesin, and, most likely, the microtubules (MT) network. Furthermore, an increased capacity in energy production was found, as indicated by a threefold higher creatine kinase activity in heart mitochondria from D-/- compared to D+/+ mice. We also observed a significantly lower amount of cytochrome c in heart mitochondria from D-/- mice, and a relocalization of Bcl-2, which may indicate an apoptotic condition in the cell leading to the earlier reported pathological events, such as cardiomyocytes degeneration and calcinosis of the heart (Thornell et al., 1997).
authors.affiliation: Groupe de Biologie des Interactions Cellulaires, UMR CNRS 6558, Poitiers, France. Monica@dbb.su.se
authors.forename: M
authors.initials: M
authors.lastname: Lindén
authors.forename: Z
authors.initials: Z
authors.lastname: Li
authors.forename: D
authors.initials: D
authors.lastname: Paulin
authors.forename: T
authors.initials: T
authors.lastname: Gotow
authors.forename: J F
authors.initials: JF
authors.lastname: Leterrier
mesh_terms: D000818:Animals; D003402:Creatine Kinase / Q000378:metabolism; D020285:Cryoelectron Microscopy; D003574:Cytochrome c Group / Q000032:analysis; D003893:Desmin / Q000235:genetics / Q000502:physiology*; D004591:Electrophoresis, Polyacrylamide Gel; D015151:Immunoblotting; D016547:Kinesins / Q000032:analysis; D051379:Mice; D018345:Mice, Knockout; D008870:Microtubules; D008929:Mitochondria, Heart / Q000737:chemistry* / Q000187:drug effects* / Q000473:pathology; D011506:Proteins / Q000032:analysis; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism
chemical_list: D003574:Cytochrome c Group; D003893:Desmin; D011506:Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D003402:Creatine Kinase; D016547:Kinesins