title: A two years longitudinal study of a transgenic Huntington disease monkey.
abstract: BACKGROUND
  A two-year longitudinal study composed of morphometric MRI measures and cognitive behavioral evaluation was performed on a transgenic Huntington's disease (HD) monkey. rHD1, a transgenic HD monkey expressing exon 1 of the human gene encoding huntingtin (HTT) with 29 CAG repeats regulated by a human polyubiquitin C promoter was used together with four age-matched wild-type control monkeys. This is the first study on a primate model of human HD based on longitudinal clinical measurements.
  
  
  RESULTS
  Changes in striatal and hippocampal volumes in rHD1 were observed with progressive impairment in motor functions and cognitive decline, including deficits in learning stimulus-reward associations, recognition memory and spatial memory. The results demonstrate a progressive cognitive decline and morphometric changes in the striatum and hippocampus in a transgenic HD monkey.
  
  
  CONCLUSIONS
  This is the first study on a primate model of human HD based on longitudinal clinical measurements. While this study is based a single HD monkey, an ongoing longitudinal study with additional HD monkeys will be important for the confirmation of our findings. A nonhuman primate model of HD could complement other animal models of HD to better understand the pathogenesis of HD and future development of diagnostics and therapeutics through longitudinal assessment.
authors.affiliation: Division of Neuropharmacology and Neurologic Diseases, Yerkes National Primate Research Center, Emory University, Atlanta, Georgia. awchan@emory.edu.
authors.forename: Anthony Ws
authors.initials: AW
authors.lastname: Chan
authors.forename: Yan
authors.initials: Y
authors.lastname: Xu
authors.forename: Jie
authors.initials: J
authors.lastname: Jiang
authors.forename: Tayeb
authors.initials: T
authors.lastname: Rahim
authors.forename: Dongming
authors.initials: D
authors.lastname: Zhao
authors.forename: Jannet
authors.initials: J
authors.lastname: Kocerha
authors.forename: Tim
authors.initials: T
authors.lastname: Chi
authors.forename: Sean
authors.initials: S
authors.lastname: Moran
authors.forename: Heidi
authors.initials: H
authors.lastname: Engelhardt
authors.forename: Katherine
authors.initials: K
authors.lastname: Larkin
authors.forename: Adam
authors.initials: A
authors.lastname: Neumann
authors.forename: Haiying
authors.initials: H
authors.lastname: Cheng
authors.forename: Chunxia
authors.initials: C
authors.lastname: Li
authors.forename: Katie
authors.initials: K
authors.lastname: Nelson
authors.forename: Heather
authors.initials: H
authors.lastname: Banta
authors.forename: Stuart M
authors.initials: SM
authors.lastname: Zola
authors.forename: Francois
authors.initials: F
authors.lastname: Villinger
authors.forename: Jinjing
authors.initials: J
authors.lastname: Yang
authors.forename: Claudia M
authors.initials: CM
authors.lastname: Testa
authors.forename: Hui
authors.initials: H
authors.lastname: Mao
authors.forename: Xiaodong
authors.initials: X
authors.lastname: Zhang
authors.forename: Jocelyne
authors.initials: J
authors.lastname: Bachevalier
mesh_terms: D000375:Aging / Q000235:genetics / Q000473:pathology; D000818:Animals; D030801:Animals, Genetically Modified; D001921:Brain / Q000378:metabolism* / Q000473:pathology*; D004195:Disease Models, Animal; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics* / Q000473:pathology*; D008137:Longitudinal Studies; D008253:Macaca mulatta; D008297:Male; D009419:Nerve Tissue Proteins / Q000235:genetics*; D009929:Organ Size / Q000235:genetics; D014018:Tissue Distribution
chemical_list: C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins