Strain Characteristics |
MPR rats have smaller bodies, shorter limbs, facial dysmorphia, dysostosis multiplex, and increased urinary excretion of glucosaminoglycans (GAGs) and these features become evident after 3 weeks of age (Yoshida, 1993). Biochemical analyses demonstrated that the excreted CAG was dermantan sulfate and the activity of hepatic arylsulfatase B was lower than that of normal rats. MPR rats have a 1-bp insertion (507insC) which resulting in a frame shift mutation in the arylsulfatase B (<i>Arsb</i>) gene on rat chromosome 2 (Kunieda, 1995). The MPR is a disease model for mucopolysaccharidosis VI. (Jun 3, 2010) |
References |
Yoshida M, Noguchi J, Ikadai H, Takahashi M, Nagase S. Arylsulfatase B-deficient mucopolysaccharidosis in rats. J Clin Invest. 91(3):1099-104, 1993.
Kunieda T, Simonaro CM, Yoshida M, Ikadai H, Levan G, Desnick RJ, Schuchman EH. Mucopolysaccharidosis type VI in rats: isolation of cDNAs encoding arylsulfatase B, chromosomal localization of the gene, and identification of the mutation. Genomics. 10;29(3):582-7, 1995. |