Anti-HSS/SGSH Antibody Picoband® Fluoro647 Conjugated
Product Specifications
Background
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.
Synonyms
Thioredoxin, mitochondrial; MTRX; Mt-Trx; Thioredoxin-2; TXN2; TRX2
Gene Name
SGSH
Gene ID
6448
UniProt
P51688
Host
Rabbit
Reactivity
Human, Mouse, Rat
Cross Reactivity
No cross-reactivity with other proteins.
Immunogen
E.coli-derived human HSS/SGSH recombinant protein (Position: R206-L502) .
Clonality
Polyclonal
Tissue Specificity
Widely expressed in adult (at protein level) and fetal tissues.
Applications
Flow Cytometry
Field of Research
Hematopoietic Progenitors, Immunology, Innate Immunity, Stem Cells, Surface Molecules
Purification
Immunogen affinity purified.
Form
Liquid
Function
Important for the control of mitochondrial reactive oxygen species homeostasis, apoptosis regulation and cell viability. Possesses a dithiol-reducing activity.
References & Citations
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.
Storage Conditions
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
Calculated Molecular Weight
57 kDa
Applications Notes
6
Gene Name Synonym
Thioredoxin 2
Subcellular Location
Mitochondrion.
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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