溶酶体α-甘露糖苷酶抗体
规格：1mg/1ml
英文名： MAN2B1
别名： EC 3.2.1.24; Laman; Lysosomal acid alpha mannosidase; Lysosomal alpha mannosidase; MANB; Mannosidase alpha class 2B member 1; Mannosidase, alpha B; Mannosidase, alpha B, lysosomal.
分子量： 109kDa
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 MAN2B1
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Rabbit,
细胞定位：
溶酶体α-甘露糖苷酶抗体产品介绍：background: This gene encodes an enzyme that hydrolyzes terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides. Its activity is necessary for the catabolism of N-linked carbohydrates released during glycoprotein turnover and it is member of family 38 of glycosyl hydrolases. The full length protein is processed in two steps. First, a 49 aa leader sequence is cleaved off and the remainder of the protein is processed into 3 peptides of 70 kDa, 42 kDa (D) and 13/15 kDa (E). Next, the 70 kDa peptide is further processed into three peptides (A, B and C). The A, B and C peptides are disulfide-linked. Defects in this gene have been associated with lysosomal alpha-mannosidosis. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq,溶酶体α-甘露糖苷酶抗体 Mar 2010] Function: Necessary for the catabolism of N-linked carbohydrates released during glycoprotein turnover. Cleaves all known types of alpha-mannosidic linkages. Subcellular Location: Lysosome. Post-translational modifications: First processed into 3 peptides of 70 kDa, 42 kDa (D) and 13/15 kDa (E). The 70 kDa peptide is further processed into three peptides (A, B and C). The A, B and C peptides are disulfide-linked. DISEASE: Mannosidosis, alpha B, lysosomal (MANSA) [MIM:248500]: A lysosomal storage disease characterized by accumulation of unbranched oligosaccharide chains. This accumulation is expressed histologically as cytoplasmic vacuolation predominantly in the CNS and parenchymatous溶酶体α-甘露糖苷酶抗体 organs. Depending on the clinical findings at the age of onset, a severe infantile (type I) and a mild juvenile (type II) form of alpha-mannosidosis are recognized. There is considerable variation in the clinical expression with mental retardation, recurrent infections, impaired hearing and Hurler-like skeletal changes being the most consistent abnormalities. Note: The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the glycosyl hydrolase 38 family. Gene ID: 4125 Database links: Entrez Gene: 4125 Human Omim: 609458 Human SwissProt: O00754 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
溶酶体α-甘露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：肿瘤 细胞生物 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid