Behavioural characterisation of the α-mannosidosis guinea pig

A. J. Robinson, A. C. Crawley, D. Auclair, P. F. Weston, C. Hirte, K. M. Hemsley, J. J. Hopwood

Research output: Contribution to journalArticlepeer-review

7 Citations (Scopus)

Abstract

α-Mannosidosis is a lysosomal storage disorder resulting from a functional deficiency of the lysosomal enzyme α-mannosidase. This deficiency results in the accumulation of various oligosaccharides in the lysosomes of affected individuals, causing somatic pathology and progressive neurological degeneration that results in cognitive deficits, ataxia, and other neurological symptoms. We have a naturally occurring guinea pig model of this disease which exhibits a deficiency of lysosomal α-mannosidase and has a similar clinical presentation to human α-mannosidosis. Various tests were developed in the present study to characterise and quantitate the loss of neurological function in α-mannosidosis guinea pigs and to follow closely the progression of the disease. General neurological examinations showed progressive differences in α-mannosidosis animals from approximately 1 month of age. Significant differences were observed in hind limb gait width from 2 months of age and significant cognitive (memory and learning) deficits were observed from 3 months of age. Evoked response tests showed an increase in somatosensory P1 peak latency in α-mannosidosis guinea pigs from approximately 2 months of age, as well as progressive hearing loss using auditory brainstem evoked responses. The α-mannosidosis guinea pig therefore appears to exhibit many of the characteristics of the human disease, and will be useful in evaluating therapies for treatment of central nervous system pathology. Crown

Original languageEnglish
Pages (from-to)176-184
Number of pages9
JournalBEHAVIOURAL BRAIN RESEARCH
Volume186
Issue number2
DOIs
Publication statusPublished - 25 Jan 2008
Externally publishedYes

Keywords

  • α-Mannosidosis
  • Behavioural test
  • Guinea pig
  • Lysosomal storage disorder

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