| 產(chǎn)品編號 | bs-19002R |
| 英文名稱 | Rabbit Anti-NAGLU antibody |
| 中文名稱 | N-乙酰基-α葡萄糖苷酶抗體 |
| 別 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. |
| 研究領(lǐng)域 | 細胞生物 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 新陳代謝 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rabbit,Pig,Sheep,Dog,Horse) |
| 產(chǎn)品應(yīng)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. |
| 理論分子量 | 80kDa |
| 細胞定位 | 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human NAGLU: 121-220/743 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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 |
