己醛醣酸盐水解酵素抗体
规格：1mg/1ml
英文名： IDUA
别名： Alpha L iduronidase; IDA; Iduronidase alpha L; MPS1.
分子量： 70kDa
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 IDUA
交叉反应：Human, Mouse,
细胞定位：
己醛醣酸盐水解酵素抗体产品介绍：background: This gene encodes an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). [provided by RefSeq, Jul 2008] Function: IDUA is an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Defects in IDUA are the cause of mucopolysaccharidosis type 1H (MPS1H) also known as Hurler syndrome, mucopolysaccharidosis己醛醣酸盐水解酵素抗体 type 1H/S (MPS1H/S) also known as Hurler-Scheie syndrome and mucopolysaccharidosis type 1S (MPS1S) also known as Scheie syndrome. MPS1S is a mild form whilst MPS1H is a severe form of this rare lysosomal storage disease characterized by progressive 己醛醣酸盐水解酵素抗体physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Subunit: Monomer. Subcellular Location: Lysosome Tissue Specificity: Ubiquitous. Similarity: Belongs to the glycosyl hydrolase 39 family. Gene ID: 3425 Database links: Entrez Gene: 3425 Human Omim: 252800 Human SwissProt: P35475 Human Unigene: 89560 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
己醛醣酸盐水解酵素抗体产品应用：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：肿瘤 细胞生物 **学 信号转导 细胞类型标志物
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid