Anti-ARSB抗体(ab85727)
Key features and details
- Goat polyclonal to ARSB
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
概述
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产品名称
Anti-ARSB抗体
参阅全部 ARSB 一抗 -
描述
山羊多克隆抗体to ARSB -
宿主
Goat -
经测试应用
适用于: WB, IHC-Pmore details -
种属反应性
与反应: Human
预测可用于: Cow, Cat -
免疫原
Synthetic peptide corresponding to Human ARSB (internal sequence) (Cysteine residue).
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阳性对照
- Skeletal muscle tissue; human heart lysate.
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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: 7.30
Preservative: 0.02% Sodium azide
Constituents: Tris buffered saline, 0.5% BSA -
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
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab85727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 |
Use a concentration of 0.03 - 0.1 µg/ml. Predicted molecular weight: 60 kDa.
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IHC-P |
Use a concentration of 5 µg/ml.
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说明 |
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WB
Use a concentration of 0.03 - 0.1 µg/ml. Predicted molecular weight: 60 kDa. |
IHC-P
Use a concentration of 5 µg/ml. |
靶标
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疾病相关
Defects in ARSB are the cause of mucopolysaccharidosis type 6 (MPS6) [MIM:253200]; also known as Maroteaux-Lamy syndrome. MPS6 is an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of dermatan sulfate. Clinical features can include abnormal growth, short stature, stiff joints, skeletal malformations, corneal clouding, hepatosplenomegaly, and cardiac abnormalities. A wide variation in clinical severity is observed.
Arylsulfatase B activity is defective in multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a disorder characterized by decreased activity of all known sulfatases. MSD is due to defects in SUMF1 resulting in the lack of post-translational modification of a highly conserved cysteine into 3-oxoalanine. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. -
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. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD). -
细胞定位
Lysosome. - Information by UniProt
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数据库链接
- Entrez Gene: 100216331 Cat
- Entrez Gene: 411 Human
- Omim: 611542 Human
- SwissProt: P33727 Cat
- SwissProt: P15848 Human
- Unigene: 149103 Human
- Unigene: 604199 Human
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别名
- Arsb antibody
- ARSB_HUMAN antibody
- Arylsulfatase B antibody
see all
图片
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ab85727, at 5 µg/ml, staining ARSB in skeletal muscle tissue by Immunohistochemistry.
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Anti-ARSB antibody (ab85727) at 0.03 µg/ml + human heart lysate at 35 µg
Predicted band size: 60 kDa
Observed band size: 35,40 kDa why is the actual band size different from the predicted?
数据表及文件
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Datasheet download
文献 (1)
ab85727 被引用在 1 文献中.
- Luo M et al. Changes in the metabolism of chondroitin sulfate glycosaminoglycans in articular cartilage from patients with Kashin-Beck disease. Osteoarthritis Cartilage 22:986-95 (2014). PubMed: 24857976