SGSH anticorps (AA 206-502)

N° du produit ABIN7600581

Cet anticorps Lapin Polyclonal détecte spécifiquement SGSH dans WB et ELISA. Il présente une réactivité envers Humain.

Aperçu rapide pour SGSH anticorps (AA 206-502) (ABIN7600581)

Antigène: SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SGSH est non-conjugé

Application: Western Blotting (WB), ELISA

Détail du produit anti-SGSH anticorps

Épitope: AA 206-502

Fonction: Anti-HSS/SGSH Antibody Picoband®

Réactivité croisée (Details): No cross-reactivity with other proteins.

Attributs du produit: Anti-HSS/SGSH Antibody Picoband® (ABIN7600581). 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.

Purification: Immunogen affinity purified.

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

Isotype: IgG

Information d'application

Indications d'application: Western blot, 0.25-0.5 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
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.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

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

Stock: 4 °C,-20 °C

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

Détails sur SGSH

Antigène: SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))

Autre désignation: SGSH

Sujet:

Synonyms: Thioredoxin, mitochondrial, MTRX, Mt-Trx, Thioredoxin-2, TXN2, TRX2

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

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.

Poids moléculaire: 70 kDa

ID gène: 6448

UniProt: P51688

Pathways: Glycosaminoglycan Metabolic Process