Anti-beta glucuronidase/GUSB Antibody Picoband® Fluoro594 Conjugated

Beta-glucuronidases are members of the glycosidase family of enzymes that catalyze breakdown of complex carbohydrates. This gene encodes a hydrolase that degrades glycosaminoglycans, including heparan sulfate, dermatan sulfate, and chondroitin-4,6-sulfate. The enzyme forms a homotetramer that is localized to the lysosome. Mutations in this gene result in mucopolysaccharidosis type VII. Alternative splicing results in multiple transcript variants. There are many pseudogenes of this locus in the human genome.

Auditory System, Domain Families, Epigenetics and Nuclear Signaling, Hlh/Leucine Zipper, Neurogenesis, Neurology Process, Neuroscience, Nuclear, Sensory System, Signaling Pathways, Stem Cells, Transcription

1. Kreamer, B. L., Siegel, F. L., Gourley, G. R. A novel inhibitor of beta-glucuronidase: L-aspartic acid. Pediat. Res. 50: 460-466, 2001. 2. Miller, R. D., Hoffmann, J. W., Powell, P. P., Kyle, J. W., Shipley, J. M., Bachinsky, D. R., Sly, W. S. Cloning and characterization of the human beta-glucuronidase gene. Genomics 7: 280-283, 1990. 3. Tomatsu, S., Orii, K. O., Vogler, C., Grubb, J. H., Snella, E. M., Gutierrez, M. A., Dieter, T., Sukegawa, K., Orii, T., Kondo, N., Sly, W. S. Missense models [Gus (tm (E536A) Sly), Gus (tm (E536Q) Sly), and Gus (tm (L175F) Sly) ] of murine mucopolysaccharidosis type VII produced by targeted mutagenesis. Proc. Nat. Acad. Sci. 99: 14982-14987, 2002.