- 首页
- 公司介绍
- 热门促销
-
全部产品
-
试剂盒
- |
-
一抗
- |
-
二抗
- |
-
蛋白
- |
-
免疫组化试剂
- |
-
WB 试剂
- PonceauS Staining Solution
- PBST Washing Buffer, 10X
- 1.5M Tris-HCl Buffer, pH8.8
- 1M Tris-HCl Buffer, pH6.8
- 10% SDS Solution
- Prestained Protein Marker
- TBST Washing Buffer, 10X
- SDS PAGE Loading Buffer, 5X
- Stripping Buffered Solution
- Tris Buffer, pH7.4, 10X
- Total Protein Extraction Kit
- Running Buffer, 10X
- Transfer Buffer, 10X
- 30% Acr-Bis(29:1) Solution
- Tris电泳液速溶颗粒
- PBS(1X, premixed powder)
- TBS(1X, premixed powder)
- 快速封闭液
- 转膜液速溶颗粒
- Chemical reagents
- 公司新闻
- 营销网络
- 资源中心
- 联系我们
- 简介:
- >>Glycosaminoglycan degradation;>>Metabolic pathways;>>Lysosome
- 免疫原:
- Synthesized peptide derived from human ANAG AA range: 146-196
- 特异性:
- This antibody detects endogenous levels of ANAG at Human
- 组成:
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 来源:
- Polyclonal, Rabbit,IgG
- 纯化工艺:
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
- 储存:
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 背景:
- This gene encodes an enzyme that degrades heparan sulfate by hydrolysis of terminal N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides. Defects in this gene are the cause of mucopolysaccharidosis type IIIB (MPS-IIIB), also known as Sanfilippo syndrome B. This disease is characterized by the lysosomal accumulation and urinary excretion of heparan sulfate. [provided by RefSeq, Jul 2008],
- 功能:
- catalytic activity:Hydrolysis of terminal non-reducing N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides.,caution:A MPS3B mutation at position 100 was erroneously reported (PubMed:9950362) as an amino acid change from Arg to His. The right amino acid change is from His to Arg.,disease:Defects in NAGLU are the cause of mucopolysaccharidosis type 3B (MPS3B) [MIM:252920]; also known as Sanfilippo syndrome B. MPS3B is a 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.,functio
- 组织表达:
- Liver, ovary, peripheral blood leukocytes, testis, prostate, spleen, colon, lung, placenta and kidney.
![](/uploadfiles/PIC/YT6646wb-2019.jpg)
- Western blot analysis of lysates from A549 cells, primary antibody was diluted at 1:1000, 4°over night