產(chǎn)品編號(hào) | bs-18647R-Gold |
英文名稱(chēng) | Rabbit Anti-MAN2B1/Gold Conjugated antibody |
中文名稱(chēng) | 膠體金標(biāo)記的溶酶體α-甘露糖苷酶抗體 |
別 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. |
規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買(mǎi) 大包裝/詢(xún)價(jià) |
說(shuō) 明 書(shū) | 100ul(10nm 15nm 35nm) |
研究領(lǐng)域 | 腫瘤 細(xì)胞生物 表觀遺傳學(xué) |
抗體來(lái)源 | Rabbit |
克隆類(lèi)型 | Polyclonal |
交叉反應(yīng) | Mouse, (predicted: Human, Rat, Dog, Pig, Cow, Rabbit, ) |
產(chǎn)品應(yīng)用 | IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 109kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 0.4mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human MAN2B1 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲(chǔ) 存 液 | 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300. |
保存條件 | Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles. |
產(chǎn)品介紹 |
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, 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 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. 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. |