Anti-SGSH/HSS抗体(ab96029)
Key features and details
- Rabbit polyclonal to SGSH/HSS
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
概述
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产品名称
Anti-SGSH/HSS抗体
参阅全部 SGSH/HSS 一抗 -
描述
兔多克隆抗体to SGSH/HSS -
宿主
Rabbit -
经测试应用
适用于: WB, IHC-Pmore details -
种属反应性
与反应: Human -
免疫原
Recombinant fragment corresponding to Human SGSH/HSS aa 318-466.
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阳性对照
- Molt-4 whole cell lysate, OVCAR3 xenograft, A431 cells
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常规说明
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
存储溶液
pH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 1.21% Tris, 0.75% Glycine, 10% Glycerol (glycerin, glycerine) -
Concentration information loading...
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纯度
Immunogen affinity purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
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Related Products
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab96029于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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WB |
1/500 - 1/3000. Predicted molecular weight: 57 kDa.
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IHC-P |
1/100 - 1/500.
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说明 |
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WB
1/500 - 1/3000. Predicted molecular weight: 57 kDa. |
IHC-P
1/100 - 1/500. |
靶标
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疾病相关
Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. -
序列相似性
Belongs to the sulfatase family. -
翻译后修饰
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. -
细胞定位
Lysosome. - Information by UniProt
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数据库链接
- Entrez Gene: 6448 Human
- Omim: 605270 Human
- SwissProt: P51688 Human
- Unigene: 31074 Human
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别名
- Heparan sulfate sulfatase antibody
- Heparan sulphate sulphatase antibody
- HSS antibody
see all
图片
数据表及文件
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SDS download
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Datasheet download
文献 (2)
ab96029 被引用在 2 文献中.
- Duncan FJ et al. Broad functional correction of molecular impairments by systemic delivery of scAAVrh74-hSGSH gene delivery in MPS IIIA mice. Mol Ther 23:638-47 (2015). ICC/IF . PubMed: 25592334
- Boado RJ et al. Insulin receptor antibody-sulfamidase fusion protein penetrates the primate blood-brain barrier and reduces glycosoaminoglycans in Sanfilippo type A cells. Mol Pharm 11:2928-34 (2014). WB . PubMed: 24949884