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Gene Review

GUSB  -  glucuronidase, beta

Canis lupus familiaris

 
 
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Disease relevance of GUSB

 

High impact information on GUSB

  • Intravenous injection of a retroviral vector expressing canine GUSB into neonatal MPS VII mice resulted in transduction of 6 to 35% of hepatocytes, which secreted GUSB into blood [2].
  • Serum GUSB activity was stable for 6 months at 600 (low expression) to 10,000 (high expression) U/ml, and enzyme was modified appropriately with mannose 6-phosphate [2].
  • However, better delivery of GUSB to chondrocytes will be necessary to achieve more profound effects in cartilage [3].
  • Although chondrocytes did not have detectable GUSB activity and had little reduction in lysosomal storage, the thickness of the growth plate was reduced toward normal [3].
  • Both myocardium and aorta had significant levels of GUSB and reduction in GAGs [4].
 

Analytical, diagnostic and therapeutic context of GUSB

  • The average serum GUSB activity (3531 U/ml) is the highest long-term expression reported for MPS VII mice after gene therapy [2].

References

  1. Cloning of the canine beta-glucuronidase cDNA, mutation identification in canine MPS VII, and retroviral vector-mediated correction of MPS VII cells. Ray, J., Bouvet, A., DeSanto, C., Fyfe, J.C., Xu, D., Wolfe, J.H., Aguirre, G.D., Patterson, D.F., Haskins, M.E., Henthorn, P.S. Genomics (1998) [Pubmed]
  2. Evaluation of pathological manifestations of disease in mucopolysaccharidosis VII mice after neonatal hepatic gene therapy. Xu, L., Mango, R.L., Sands, M.S., Haskins, M.E., Ellinwood, N.M., Ponder, K.P. Mol. Ther. (2002) [Pubmed]
  3. Neonatal retroviral vector-mediated hepatic gene therapy reduces bone, joint, and cartilage disease in mucopolysaccharidosis VII mice and dogs. Mango, R.L., Xu, L., Sands, M.S., Vogler, C., Seiler, G., Schwarz, T., Haskins, M.E., Ponder, K.P. Mol. Genet. Metab. (2004) [Pubmed]
  4. Gene therapy ameliorates cardiovascular disease in dogs with mucopolysaccharidosis VII. Sleeper, M.M., Fornasari, B., Ellinwood, N.M., Weil, M.A., Melniczek, J., O'Malley, T.M., Sammarco, C.D., Xu, L., Ponder, K.P., Haskins, M.E. Circulation (2004) [Pubmed]
 
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