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Anti-HSS/SGSH Antibody Picoband®, PE Conjugate

Boster Bio Anti-HSS/SGSH Antibody Picoband® catalog # A04608-1. Tested in ELISA, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

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

thioredoxin 2

Gene ID

6448

UniProt

P51688

Host

Rabbit

Reactivity

Human

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

WB,ELISA

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Function

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

Components

Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

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℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Observed Molecular Weight

70 kDa

Fragment

Rabbit IgG

Applications Notes

Western blot, 0.25-0.5 μg/ml, Human<br> ELISA, 0.1-0.5 μg/ml, -<br>

Subcellular Location

Mitochondrion.

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