Recombinant Human GALNS Protein, CF Summary
Ala27-His522, with an N-terminal 6-His tag
CF stands for Carrier Free (CF). We typically add Bovine Serum Albumin (BSA) as a carrier protein to our recombinant proteins. Adding a carrier protein enhances protein stability, increases shelf-life, and allows the recombinant protein to be stored at a more dilute concentration. The carrier free version does not contain BSA.
In general, we advise purchasing the recombinant protein with BSA for use in cell or tissue culture, or as an ELISA standard. In contrast, the carrier free protein is recommended for applications, in which the presence of BSA could interfere.
|Formulation||Supplied as a 0.2 μm filtered solution in Tris and NaCl.|
|Shipping||The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.|
|Stability & Storage:||Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
- Assay Buffer: 50 mM Sodium Actetate, 250 mM NaCl, pH 5.0
- Recombinant Human N‑Acetylgalactosamine-6-Sulfatase/GALNS (rhGALNS) (Catalog # 8269-SU)
- Substrate: 4-Nitrocatechol Sulfate (PNCS) (Sigma, Catalog # N-7251), 100 mM stock in deionized water
- Sodium Hydroxide (NaOH) (Sigma, Catalog # 221465), 2 M stock in deionized water
- 96-well Clear Plate (Catalog # DY990)
- Plate Reader (Model: SpectraMax Plus by Molecular Devices) or equivalent
- Dilute rhGALNS to 40 µg/mL in Assay Buffer.
- Dilute PNCS to 4 mM in Assay Buffer.
- Combine 200 µL of 40 µg/mL of rhGALNS and 200 µL of 4 mM of PNCS in microtubes. Include a Substrate Blank with 200 µL of Assay Buffer and 200 µL of 4 mM of PNCS.
- Incubate at 37 °C overnight (16-20 hours).
- Load 100 µL from each reaction and Substrate Blank vial into a plate in triplicate, minimally.
- Stop reaction by adding 100 µL of 0.2 M NaOH to each well containing reactions and Substrate Blank.
- Read plate at 510 nm (absorbance) in endpoint mode.
- Calculate specific activity:
Specific Activity (pmol/min/µg) =
|Adjusted Abs* (OD) x Conversion Factor** (pmol/OD)|
|Incubation time (min) x amount of enzyme (µg)|
*Adjusted for Substrate Blank
** Derived using calibration standard 4-Nitrocatechol (PNC) (Sigma, Catalog # N15553).
- rhGALNS: 2 µg
- Substrate: 1 mM
GALNS is a lysosomal sulfatase that hydrolyzes the 6-sulfate groups of the N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate and of the D-galactose 6-sulfate units of keratan sulfate (1). The conversion to 3-oxoalanine (also known as C-formylglycine, FGly) of a cysteine residue is critical for catalytic activity of the enzyme (2). Deficiencies of this enzyme lead to Morquio A syndrome or mucopolysaccharidosis 4A (MPS4A), a lysosomal storage disorder characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate (3). Key clinical features of this disease include short stature, skeletal dysplasia, dental anomalies, and corneal clouding (4). Current therapeutic method for lysosomal storage diseases is enzyme replacement therapy (5).
- Tomatsu, S. et al. (1991) Biochem. Biophys. Res. Commun. 181:677.
- Dierks, T. et al. (1997) Proc. Natl. Acad. Sci. U. S. A. 94:11963.
- Rivera-Colon, Y. et al. (2013) J. Mol. Biol. 423:736.
- Fukuda, S. et al. (1992) J. Clin. Invest. 90:1049.
- Ohashi, T. (2012) Pediatr. Endocrinol. Rev. 10 Suppl 1:26.
Citations for Recombinant Human GALNS Protein, CF
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Citations: Showing 1 - 2
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Sex Difference Leads to Differential Gene Expression Patterns and Therapeutic Efficacy in Mucopolysaccharidosis IVA Murine Model Receiving AAV8 Gene Therapy
Authors: M Piechnik, PC Amendum, K Sawamoto, M Stapleton, S Khan, N Fnu, V Álvarez, AMH Pachon, O Danos, JT Bruder, S Karumuthil, S Tomatsu
International Journal of Molecular Sciences, 2022;23(20):.
Sample Types: Plasma
Applications: ELISA Capture
Bio-Plex immunoassay measuring the quantity of lysosomal N-acetylgalactosamine-6-sulfatase protein in dried blood spots for the screening of mucopolysaccharidosis IVA in newborn: a pilot study
Authors: CK Chuang, HY Lin, TJ Wang, SF Huang, SP Lin
BMJ Open, 2017;7(7):e014410.
Sample Types: Plasma
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