Depletion of Complexin II does not affect disease progression in a mouse model of Huntington's disease (HD); support for role for complexin II in behavioural pathology in a mouse model of HD

Brain Res Bull. 2007 Apr 30;72(2-3):108-20. doi: 10.1016/j.brainresbull.2006.10.017. Epub 2006 Nov 13.

Abstract

Huntington's disease (HD) is a progressive, inherited, neurological disorder with a complicated phenotype that is characterised by movement abnormalities, cognitive impairments and psychiatric symptoms. Although HD is a neurodegenerative disease, recent evidence indicates that neurological dysfunction, rather than frank neurodegeneration contributes to, and may even cause early symptoms in the absence of neurodegeneration. One protein that may contribute to neurological dysfunction in HD is complexin II. Complexins are presynaptic proteins that are believed to modulate neurotransmitter release. Complexin II levels are reduced in human HD striatum and cortex, and a progressive depletion of complexin II mRNA and protein has also been shown in the R6/2 mouse model of HD. Interestingly, complexin II knockout mice share behavioural deficits in reversal learning in common with R6/2 mice. Further, the two strains both show abnormalities in long-term potentiation. This evidence led us to wonder whether or not loss of complexin II underlies some of the behavioural deficits seen in R6/2 mice. To investigate this, we crossbred complexin II knockout mice with R6/2 mice to generate a double mutant mouse. The behavioural phenotype of R6/2 mice on a null complexin II background was characterised and was compared to that seen in control mice. Complete knockout of complexin II did not significantly affect the phenotype of R6/2 mice. This indicates that loss of complexin II is part of the mechanism underlying the R6/2 phenotype. Whether it is causal or compensatory remains to be determined.

MeSH terms

  • Adaptor Proteins, Vesicular Transport
  • Animals
  • Behavior, Animal / physiology*
  • Disease Models, Animal
  • Disease Progression
  • Female
  • Huntington Disease / genetics
  • Huntington Disease / physiopathology*
  • Long-Term Potentiation / physiology
  • Male
  • Maze Learning / physiology
  • Mice
  • Mice, Knockout
  • Motor Activity / physiology*
  • Nerve Tissue Proteins / deficiency*
  • Nerve Tissue Proteins / genetics
  • Phenotype
  • Polymerase Chain Reaction

Substances

  • Adaptor Proteins, Vesicular Transport
  • Nerve Tissue Proteins
  • complexin II