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American Journal of Orthodontics and Dentofacial Orthopedics, 2007-07-01, Volume 132, Issue 1, Pages 127-128, Copyright © 2007 American Association of Orthodontists

Introduction: Sandhoff’s disease is a rare, genetic, lipid-storage disorder resulting in the progressive deterioration of the central nervous system. It is caused by a deficiency of the enzyme hexosaminidase that results in the accumulation of certain lipids in the brain and other organs of the body. Although Sandhoff’s disease is a severe form of Tay-Sachs disease, which is prevalent primarily in people of European Jewish descent, it is not limited to any ethnic group. Onset of the disorder usually occurs at 6 months of age. Inflammation in GM 2 gangliosidosis is the key element of Sandhoff’s disease.

Objective: By reducing the inflammation with IL-1β receptor antagonist, it was expected that the craniofacial development of the β-hexosaminidase deficient mice would be normalized. The aim of this study was to investigate the effects of a FIV IL-1β receptor antagonist vector in β-hexosaminidase deficient mice.

Methods: The β-hexosaminidase deficient mice in the treated group were given an intraperitoneal injection of the FIV IL-1β receptor antagonist vector during neonatal age. Untreated β-hexosaminidase deficient mice and wild-type mice were used as experimental controls for this study. The craniofacial development of the mice was evaluated by using a cephalometric analysis on β-hexosaminidase deficient mice treated with FIV IL-1β receptor antagonist (n = 3), untreated β-hexosaminidase deficient mice (n = 3), and wild-type controls (n = 3). The data were analyzed by using 1-way ANOVA followed by Tukey post-hoc analysis with a level of significance set at P <.05. Finally, the spheno-occipital suture was evaluated for each mouse by using Alcian Blue-Orange G histochemistry and collagen-2 immunohistochemistry.

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Results and conclusions: The results of the cephalometric analysis showed that the Ba-Rh, Na-Rh, and Na-Ba measurements were ameliorated by this treatment. The histologic analysis showed that the development of the spheno-occipital sutures in the treated knockout mice were normalized and closely resembled those of the wild-type controls.