Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB
| Autor: | Jonathan D. Cooper, Brian W. Bigger, Maria Mercè Canal, Fiona L. Wilkinson, J. Ed Wraith, Rob Wynn |
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| Rok vydání: | 2010 |
| Předmět: |
medicine.medical_specialty
Vasopressin Photoperiod Central nervous system Neuropeptide Motor Activity Biology Mice Mucopolysaccharidosis III Behavioral Neuroscience Internal medicine Image Processing Computer-Assisted medicine Lysosomal storage disease Animals Circadian rhythm Analysis of Variance Behavior Animal Suprachiasmatic nucleus Age Factors medicine.disease Actigraphy Immunohistochemistry Circadian Rhythm Arginine Vasopressin Disease Models Animal Endocrinology medicine.anatomical_structure Light effects on circadian rhythm Hypothalamus Suprachiasmatic Nucleus Vasoactive Intestinal Peptide |
| Zdroj: | Behavioural Brain Research. 209:212-220 |
| ISSN: | 0166-4328 |
| DOI: | 10.1016/j.bbr.2010.01.045 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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