Anti-HSS/SGSH Antibody Picoband® fluoro594 Vervoeging

N-sulphoglucosamine sulphohydrolase is an enzyme that in humans is encoded by the SGSH gene. This gene encodes the enzyme sulfamidase; one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with the lysosomal storage disease mucopolysaccaridosis IIIA, also known as Sanfilippo syndrome A, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.

Thioredoxin, mitochondrial; MTRX; Mt-Trx; Thioredoxin-2; TXN2; TRX2

SGSH

6448

P51688

Human, Mouse, Rat

No cross-reactivity with other proteins.

E.coli-derived human HSS/SGSH recombinant protein (Position: R206-L502) .

Widely expressed in adult (at protein level) and fetal tissues.

Flow Cytometry

Hematopoietic Progenitors, Immunology, Innate Immunity, Stem Cells, Surface Molecules

Immunogen affinity purified.

Liquid

Important for the control of mitochondrial reactive oxygen species homeostasis, apoptosis regulation and cell viability. Possesses a dithiol-reducing activity.

1. Bhattacharyya, R., Gliddon, B., Beccari, T., Hopwood, J. J., Stanley, P. A novel missense mutation in lysosomal sulfamidase is the basis of MPS IIIA in a spontaneous mouse mutant. Glycobiology 11: 99-103, 2001. 2. Bielicki, J., Hopwood, J. J., Melville, E. L., Anson, D. S. Recombinant human sulphamidase: expression, amplification, purification and characterization. Biochem. J. 329: 145-150, 1998. 3. Blanch, L., Weber, B., Guo, X.-H., Scott, H. S., Hopwood, J. J. Molecular defects in Sanfilippo syndrome type A. Hum. Molec. Genet. 6: 787-791, 1997.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

57 kDa

6

Thioredoxin 2

Mitochondrion.

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.