title: Motor phenotype is not associated with vascular dysfunction in symptomatic Huntington's disease transgenic R6/2 (160 CAG) mice.
abstract: Whereas Huntington's disease (HD) is unequivocally a neurological disorder, a critical mass of emerging studies highlights the occurrence of peripheral pathology like cardiovascular defects in both animal models and humans. The overt impairment in cardiac function is normally expected to be associated with peripheral vascular dysfunction, however whether this assumption is reasonable or not in HD is still unknown. In this study we functionally characterized the vascular system in R6/2 mouse model (line 160 CAG), which recapitulates several features of human pathology including cardiac disease. Vascular reactivity in different arterial districts was determined by wire myography in symptomatic R6/2 mice and age-matched wild type (WT) littermates. Disease stage was assessed by using well-validated behavioural tests like rotarod and horizontal ladder task. Surprisingly, no signs of vascular dysfunction were detectable in symptomatic mice and no link with motor phenotype was found.
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: A
authors.initials: A
authors.lastname: Di Pardo
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: A
authors.initials: A
authors.lastname: Carrizzo
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: A
authors.initials: A
authors.lastname: Damato
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: S
authors.initials: S
authors.lastname: Castaldo
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: E
authors.initials: E
authors.lastname: Amico
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: L
authors.initials: L
authors.lastname: Capocci
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: M
authors.initials: M
authors.lastname: Ambrosio
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: F
authors.initials: F
authors.lastname: Pompeo
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: C
authors.initials: C
authors.lastname: De Sanctis
authors.affiliation: IRCCS Multimedica, Milano (Italy).
authors.forename: C C
authors.initials: CC
authors.lastname: Spinelli
authors.affiliation: IRCCS Multimedica, Milano (Italy).|University of Salerno, Salerno (Italy).
authors.forename: A A
authors.initials: AA
authors.lastname: Puca
authors.affiliation: University of Salerno, Salerno (Italy).
authors.forename: P
authors.initials: P
authors.lastname: Remondelli
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).
authors.forename: V
authors.initials: V
authors.lastname: Maglione
authors.affiliation: IRCCS Neuromed, Pozzilli (Italy).|University of Salerno, Salerno (Italy).
authors.forename: C
authors.initials: C
authors.lastname: Vecchione
mesh_terms: D000818:Animals; D001158:Arteries / Q000502:physiology*; D004195:Disease Models, Animal; D004576:Electromyography; D006801:Humans; D000071058:Huntingtin Protein / Q000235:genetics*; D006816:Huntington Disease / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D051379:Mice; D008822:Mice, Transgenic; D018482:Muscle, Skeletal / Q000503:physiopathology*; D009154:Mutation; D010641:Phenotype; D018924:Vascular Capacitance
chemical_list: C086055:HTT protein, human; D000071058:Huntingtin Protein