Trans-generational exposure to low levels of rhodamine B does not adversely affect litter size or liver function in murine mucopolysaccharidosis type IIIA

Molecular Genetics and Metabolism
Ainslie RobertsSharon Byers

Abstract

MPS IIIA is a lysosomal storage disorder caused by mutations in the sulphamidase gene, resulting in the accumulation of heparan sulphate glycosaminoglycans (HS GAGs). Symptoms predominantly manifest in the CNS and there is no current therapy that effectively addresses neuropathology in MPS IIIA patients. Recent studies in MPS IIIA mice have shown that rhodamine B substrate deprivation therapy (SDT) (also termed substrate reduction therapy/SRT) inhibits GAG biosynthesis and, improves both somatic and CNS disease pathology. Acute overexposure to high doses of rhodamine B results in liver toxicity and is detrimental to reproductive ability. However, the long-term effects of decreasing GAG synthesis, at the low dose sufficient to alter neurological function are unknown. A trans-generational study was therefore initiated to evaluate the continuous exposure of rhodamine B treatment in MPS IIIA mice over 4 generations, including treatment during pregnancy. No alterations in litter size, liver histology or liver function were observed. Overall, there are no long-term issues with the administration of rhodamine B at the low dose tested and no adverse effects were noted during pregnancy in mice.

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Citations

Apr 24, 2013·Journal of Inherited Metabolic Disease·N Joan Abbott
Mar 1, 2012·IUBMB Life·Alicja Węgrzyn
Jul 8, 2016·International Journal of Molecular Sciences·Maria Francisca CoutinhoSandra Alves
Sep 19, 2020·Journal of Inherited Metabolic Disease·Berna Seker YilmazJulien Baruteau
May 1, 2020·Frontiers in Molecular Biosciences·Jacob M FavretDaesung Shin
Nov 21, 2012·International Journal of Molecular Medicine·Giancarlo ParentiMariacarolina Salerno

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