Categories:   LabSHOTS News Technical Notes

Journal Club #7: Characterizing the Gastrointestinal (GI) Abnormalities in the Mouse Dystonia Musculorum (DSTdt) Model for Hereditary Sensory and Autonomic Neuropathy Type VI (HSAN-VI)

A recent publication from Lynch-Godrei A. et al. from the Neurogastroenterology & Motility Journal characterized the gastrointestinal (GI) abnormalities in the mouse Dystonia musculorum (DSTdt)  model for hereditary sensory and autonomic neuropathy type VI (HSAN-VI) and demonstrated that the recessive dystonin (DST) gene does not correlate with the efficiency of GI absorption into blood plasma as confirmed by Chondrex, Inc.’s D-Xylose Assay Kit.

In humans, HSAN-VI is a recessive genetic disease that results from mutations to the DST gene, leading to joint abnormalities, chronic diarrhea, abdominal pain, and other deteriorating symptoms. The Dstdt-27J mice, which arose by spontaneous mutation, reflect many common symptoms similar to human HSAN-VI, including GI abnormalities. By postnatal day 15, mice have a characteristic accumulation of gas and discoloration of the small intestine which coincides with neurodegeneration of the enteric nervous system (ENS). To characterize and evaluate GI and ENS defects, an array of tests was performed including the D-xylose assay. 

To analyze GI absorption function, mice were administered 1 mg/g bodyweight D-xylose in PBS via oral gavage on postnatal day 15. The absorbed D-xylose levels in plasma were determined at 2- and 4-hours post-administration and levels were not significantly different between wild type and Dstdt-27J mice. On the other hand, the Dstdt-27J mice show thinning of the colon mucous layer and a reduced microbial richness/evenness.

For more information, please refer to the following link.

A. Lynch-Godrei, R. De, R. Yaworski, S. Gagnon, J. Butcher, et al., Characterization of gastrointestinal pathologies in the dystonia musculorum mouse model for hereditary sensory and autonomic neuropathy type VI. Neurogastroenterol Motil 32, e13773 (2020).

In order to evaluate intracellular and intercellular permeability of molecules from the gut to the bloodstream, Chondrex, Inc. provides a D-xylose assay kit and a variety of fluorescent probe labeled Dextran. In addition, Chondrex Inc. provides antibody assay kits against various bacteria and bacterial toxins which may be useful to determine the state of gut barrier functions. Besides evaluation assays, Chondrex, Inc. also carries dextran sulfate sodium (DSS) to induce the mouse colitis model. Please refer to Chondrex, Inc.’s website for more information.

Permeability Evaluation Products
Inflammatory Bowel Disease - DSS-Induced Colitis
Mouse - Anti-Bacterial Antibody Assays
Human Anti-Bacteria and Toxins Antibody Assays


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