At birth, homozygous null mice are viable, normal in size, and do not display any gross physical or behavioral abnormalities. The lysosomal enzyme alpha-N-acetylglucosaminidase (Naglu) is absent in all tissues. Large amounts of heparan sulfate accumulate in liver and kidney, and lesser amounts in other organs. At one month of age, vacuolated macrophages can be found in most tissues. Epithelial cells in kidney and neurons in some parts of the brain are also affected. The vacuolation becomes more prominent with age. At 4-5 months, the mice show abnormal behavior in an open field test. The life span is 8-12 months. Older animals may have urinary retention and difficulty walking and must be euthanized. The null mice were originally described as fertile, but their fertility has decreasd markedly with repeated back-crossing to an inbred strain. These mice are suitable for examining the pathophysiology of the Sanfilippo syndrome type B [also known as mucopolysaccharidosis type III (MPS III)] and for developing therapies for this lysosomal storage disorder.

These <i> Naglu</i> knock-out mice exhibit massive accumulation of heparan sulfate in liver and kidney and vacuolation in many cells, including macrophages, epithelial cells, and neurons.

Typically mice are recovered in 12-16 weeks. Contact Customer Service to place an order or for more information.

<a target="_blank" href="https://www.jax.org/jax-mice-and-services/customer-support" rel="noreferrer">Contact Customer Service</a> for more information.