- 抗体类型:多克隆
- 抗体来源:兔
- 抗体应用:ELISA, IP
- 特异性:Human GNS / G6S
产品详情
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产品名称
Anti-Glucosamine (N-acetyl)-6-Sulfatase antibody
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抗体类型
多克隆
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抗体来源
兔
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抗体亚型
兔IgG
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抗体描述
Rabbit polyclonal to Glucosamine (N-acetyl)-6-Sulfatase
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抗体应用
ELISA, IP
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应用推荐
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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特异性
Human GNS / G6S
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蛋白别名
2610016K11Rik, AU042285, C87209, G6S, G6S, Glucosamine (N-acetyl)-6-Sulfatase, Glucosamine (N-acetyl)-6-Sulfatase, Gns, GNS, MGC21274, N28088, G6S, G6S, C87209, N28088, AU042285, 2610016K11Rik
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制备方法
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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组分
0.2 μm filtered solution in PBS with 5% trehalose
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储存方法
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. -
背景介绍
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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参考文献
- 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.