| 产品编号 | bs-19002R |
| 英文名称 | Rabbit Anti-NAGLU antibody |
| 中文名称 | N-乙酰基-α葡萄糖苷酶抗体 |
| 别 名 | Alpha N acetylglucosaminidase; alpha N acetylglucosaminidase, lysosomal; Alpha-N-acetylglucosaminidase 77 kDa form; ANAG; ANAG_HUMAN; MPS IIIB; MPS3B; N acetyl alpha glucosaminidase; N acetylglucosaminidase, alpha; N-acetyl-alpha-glucosaminidase; NAG; NAGLU; UFHSD 1; UFHSD; UFHSD1. |
| 研究领域 | 细胞生物 神经生物学 信号转导 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | (predicted: Human,Mouse,Rabbit,Pig,Sheep,Dog,Horse) |
| 产品应用 | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 80kDa |
| 细胞定位 | 细胞浆 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human NAGLU: 121-220/743 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
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] Function: Involved in the degradation of heparan sulfate. Subcellular Location: Lysosome. Tissue Specificity: Liver, ovary, peripheral blood leukocytes, testis, prostate, spleen, colon, lung, placenta and kidney. 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. SWISS: P54802 Gene ID: 4669 Database links: Entrez Gene: 4669 Human Entrez Gene: 27419 Mouse Omim: 609701 Human SwissProt: P54802 Human Unigene: 50727 Human Unigene: 95452 Mouse |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
