| 產(chǎn)品編號 | bs-18644R |
| 英文名稱 | MAN1B1 Rabbit pAb |
| 中文名稱 | 內(nèi)質(zhì)網(wǎng)α-甘露糖苷酶1抗體 |
| 別 名 | alpha 1 2 mannosidase; Endoplasmic reticulum alpha mannosidase 1; Endoplasmic reticulum mannosyl oligosaccharide 1 2 alpha mannosidase 1; Endoplasmic reticulum mannosyl oligosaccharide 1 2 alpha mannosidase; ER alpha 1 2 mannosidase; Man9GlcNAc2 specific processing alpha mannosidase; MANA ER; Mannosidase alpha class 1B member 1. |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Pig,Horse) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/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 | 80 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MAN1B1: 211-320/699 |
| 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 belonging to the glycosyl hydrolase 47 family. This enzyme functions in N-glycan biosynthesis, and is a class I alpha-1,2-mannosidase that specifically converts Man9GlcNAc to Man8GlcNAc isomer B. It is required for N-glycan trimming to Man5-6GlcNAc2 in the endoplasmic-reticulum-associated degradation pathway. Mutations in this gene cause autosomal-recessive intellectual disability. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 11. [provided by RefSeq, Dec 2011] Function: Involved in glycoprotein quality control targeting of misfolded glycoproteins for degradation. It primarily trims a single alpha-1,2-linked mannose residue from Man9GlcNAc2 to produce Man8GlcNAc2, but at high enzyme concentrations, as found in the ER quality control compartment (ERQC), it further trims the carbohydrates to Man5-6GlcNAc2. Subcellular Location: Endoplasmic reticulum membrane; Single-pass type II membrane protein. Tissue Specificity: Widely expressed. DISEASE: Mental retardation, autosomal recessive 15 (MRT15) [MIM:614202]: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. Note: The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the glycosyl hydrolase 47 family. SWISS: Q9UKM7 Gene ID: 11253 Database links: Entrez Gene: 11253 Human Entrez Gene: 227619 Mouse Omim: 604346 Human SwissProt: Q9UKM7 Human Unigene: 591887 Human |
