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

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dc.contributor.authorDerrick Roberts, A.
dc.contributor.authorFletcher, J.
dc.contributor.authorMoore, L.
dc.contributor.authorByers, S.
dc.date.issued2010
dc.description.abstractMPS 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.
dc.description.statementofresponsibilityAinslie L. K. Roberts, Janice M. Fletcher, Lynette Moore and Sharon Byers
dc.identifier.citationMolecular Genetics and Metabolism, 2010; 101(2-3):208-213
dc.identifier.doi10.1016/j.ymgme.2010.06.008
dc.identifier.issn1096-7192
dc.identifier.issn1096-7206
dc.identifier.orcidByers, S. [0000-0001-5576-3636]
dc.identifier.urihttp://hdl.handle.net/2440/61849
dc.language.isoen
dc.publisherAcademic Press Inc Elsevier Science
dc.relation.grantNHMRC
dc.rights© 2010 Elsevier
dc.source.urihttps://doi.org/10.1016/j.ymgme.2010.06.008
dc.subjectLiver
dc.subjectAnimals
dc.subjectMice
dc.subjectMucopolysaccharidosis III
dc.subjectDisease Models, Animal
dc.subjectRhodamines
dc.subjectGlycosaminoglycans
dc.subjectPregnancy
dc.subjectLitter Size
dc.subjectFemale
dc.titleTrans-generational exposure to low levels of rhodamine B does not adversely affect litter size or liver function in murine mucopolysaccharidosis type IIIA
dc.typeJournal article
pubs.publication-statusPublished

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