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Product Name
Anti-Glucosamine (N-acetyl)-6-Sulfatase antibody
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Description
Rabbit polyclonal to Glucosamine (N-acetyl)-6-Sulfatase
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Tested applications
ELISA, IP
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Species reactivity
Human GNS / G6S
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Alternative names
2610016K11Rik antibody; AU042285 antibody; C87209 antibody; G6S antibody; G6S antibody; Glucosamine (N-acetyl)-6-Sulfatase antibody; Glucosamine (N-acetyl)-6-Sulfatase antibody; Gns antibody; GNS antibody; MGC21274 antibody; N28088 antibody; G6S antibody; G6S antibody; C87209 antibody; N28088 antibody; AU042285 antibody; 2610016K11Rik antibody
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Isotype
Rabbit IgG
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Preparation
Produced in rabbits immunized with purified, recombinant Human GNS / G6S (rhGNS; NP_002067.1; Met 1-Leu 552). GNS specific IgG was purified by human GNS affinity chromatography.
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Clonality
Polyclonal
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Formulation
0.2 μm filtered solution in PBS with 5% trehalose
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Storage instructions
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free.
Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles. -
Applications
ELISA: 0.5-1.0 μg/mL
This antibody can be used at 0.5-1.0 μg/mL with the appropriate secondary reagents to detect Human GNS. The detection limit for Human GNS is 0.00975 ng/well.
IP: 0.5-2 μg/mg of lysate
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Validations
Glucosamine (N-acetyl)-6-Sulfatase / G6S Antibody, Rabbit PAb, Antigen Affinity Purified
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Background
Glucosamine (N-acetyl)-6-sulfatase (GNS), also known as G6S, a hydrolase, which is one of the enzymes involved in heparan sulfate catabolism leading to lysosomal storage. GNS is required for the catabolism of the glycosaminoglycans (GAG) including heparin, heparan sulphate, and keratan sulphate through the hydrolysis of 6-sulfate group from the N-acetyl-D-glucosamine 6-sulfate units. Mucopolysaccharidosis type IIID (MPS IIID) is the least common of the four subtypes of Sanfilippo syndrome. It is caused by a deficiency of N-acetylglucosamine-6-sulphatase. A mutation in GNS resulting in MPS IIID indicates the potential utility of molecular diagnosis for this rare condition. As the least common type of the four subtypes of Sanfilippo syndrome, MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations.
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References
- Fuchs W, et al. (1985) Intralysosomal formation and metabolic fate of N-acetylglucosamine 6-sulfate from keratan sulfate. Eur J Biochem. 151(3): 551-6.
- Beesley CE, et al. (2003) Sanfilippo syndrome type D: identification of the first mutation in the N-acetylglucosamine-6-sulphatase gene. J Med Genet. 40(3): 192-4.
- Mok A, et al. (2003) Genomic basis of mucopolysaccharidosis type IIID (MIM 252940) revealed by sequencing of GNS encoding N-acetylglucosamine-6-sulfatase. Genomics. 81(1): 1-5.
- Elioglu NH, et al. (2009) A novel loss-of-function mutation in the GNS gene causes Sanfilippo syndrome type D. Genet Couns. 20(2): 133-9.
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"