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Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB.

Canal, Maria M; Wilkinson, Fiona L; Cooper, Jonathan D; Ed Wraith, J; Wynn, Rob; Bigger, Brian W

Behavioural brain research. 2010;209(2):212-20.

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Abstract

Mucopolysaccharidosis IIIB (MPSIIIB) is a lysosomal storage disease characterised by progressive central nervous system degeneration in patients, with death usually in the late teens. Serious behavioural problems have been reported in children at the early stages of the disease, such as hyperactivity and severe sleep disturbances, which suggest alterations in circadian rhythms. We investigated the circadian rhythm of locomotor activity of young and old MPSIIIB mice, under a 24-h light-dark (LD) cycle and under constant darkness (DD), and also examined neuropeptide expression in the suprachiasmatic nucleus (SCN), site of the principal biological pacemaker. We show that MPSIIIB mice have higher activity levels during the light (resting) phase of the LD cycle, together with weaker circadian rhythms, and a longer active phase due to a late peak of activity, in both LD and DD. In addition, young MPSIIIB mice showed shorter phase delays in response to a light pulse in DD. Increased lysosomal storage, neuroinflammation and changes in the expression of Arginine Vasopressin and Vasointestinal Polypeptide, two circadian neuropeptides, were observed in the SCN, which may be in part responsible for the changes in circadian behaviour observed in MPSIIIB mice. These findings suggest an alteration of the circadian system in MPSIIIB mice, and may inform better clinical management of circadian, sleep and behavioural disturbances in patients with MPSIII.

Bibliographic metadata

Content type:
Publication type:
Publication form:
Published date:
Language:
eng
Abbreviated journal title:
ISSN:
Place of publication:
Netherlands
Volume:
209
Issue:
2
Pagination:
212-20
Digital Object Identifier:
10.1016/j.bbr.2010.01.045
Pubmed Identifier:
20138090
Pii Identifier:
S0166-4328(10)00078-1
Access state:
Active

Institutional metadata

University researcher(s):

Record metadata

Manchester eScholar ID:
uk-ac-man-scw:80168
Created by:
Bigger, Brian
Created:
4th May, 2010, 16:06:42
Last modified by:
Bigger, Brian
Last modified:
4th May, 2010, 16:14:10

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