N-乙酰基-α葡萄糖苷酶抗体
规格：1mg/1ml
英文名： NAGLU
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 acetylglucosami
分子量： 80kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human NAGLU
交叉反应：Human, Mouse, Dog, Pig, Horse, Rabbit, Sheep,
细胞定位：
N-乙酰基-α葡萄糖苷酶抗体产品介绍：background: 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 N-乙酰基-α葡萄糖苷酶抗体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 occursN-乙酰基-α葡萄糖苷酶抗体 typically during the second or third decade of life. Gene ID: 4669 Database links: Entrez Gene: 4669 Human Entrez Gene: 789125 Cow Entrez Gene: 27419 Mouse Omim: 609701 Human SwissProt: P54802 Human Unigene: 50727 Human Unigene: 95452 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
N-乙酰基-α葡萄糖苷酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 神经生物学 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid