| 供应商 | 上海联迈生物工程有限公司 |
| 数量 | 大量 |
| 目录编号 | LM-12741R |
| 克隆性 | 多克隆 |
| 抗原来源 | Rabbit |
| 保质期 | 1年 |
| 抗体英文名 | PMM2 |
| 抗体名 | 磷酸甘露糖变位酶2抗体 |
| 宿主 | Rabbit |
| 适应物种 | Human, Mouse, Rat, Chicken, Dog, Cow, Sheep, |
| 免疫原 | KLH conjugated synthetic peptide derived from human PMM2:1-100/246 |
| 亚型 | IgG |
| 形态 | Lyophilized or Liquid |
| 应用范围 | WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） |
| 浓度 | 1mg/ml |
| 保存条件 | Store at -20 °C |
| 规格 | 100ul 200ul |
| 英文名称 | PMM2 |
| 中文名称 | 磷酸甘露糖变位酶2抗体 |
| 别 名 | AI585868; BOS_22465; C86848; CDG 1; CDG1; CDG1a; CDGS; MGC127449; Phosphomannomutase 2; PMM 2; Pmm2; PMM2_HUMAN. |
| 规格价格 | 100ul/1380元 购买 200ul/2200元 购买 大包装/询价 |
| 说 明 书 | 100ul 200ul |
| 研究领域 | 肿瘤 细胞生物 免疫学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Cow, Sheep, |
| 产品应用 | WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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. |
| 分 子 量 | 28kDa |
| 细胞定位 | 细胞浆 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PMM2:1-100/246 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| PubMed | PubMed |
| 产品介绍 | background: The protein encoded by this gene catalyzes the isomerization of mannose 6-phosphate to mannose 1-phosphate, which is a precursor to GDP-mannose necessary for the synthesis of dolichol-P-oligosaccharides. Mutations in this gene have been shown to cause defects in glycoprotein biosynthesis, which manifests as carbohydrate-deficient glycoprotein syndrome type I. [provided by RefSeq, Jul 2008] Function: Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions. Subcellular Location: Cytoplasm. DISEASE: Defects in PMM2 are the cause of congenital disorder of glycosylation type 1A (CDG1A) [MIM:212065]; also known as carbohydrate-deficient glycoprotein syndrome type Ia (CDGS1A) or Jaeken syndrome. Congenital disorders of glycosylation are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1A is an autosomal recessive disorder characterized by a severe encephalopathy with axial hypotonia, abnormal eye movement, and pronounced psychomotor retardation, as well as peripheral neuropathy, cerebellar hypoplasia, and retinitis pigmentosa. Patients show a peculiar distribution of subcutaneous fat, nipple retraction, and hypogonadism. Similarity: Belongs to the eukaryotic PMM family. SWISS: O15305 Gene ID: 5373 Database links: Entrez Gene: 510978 Cow Entrez Gene: 5373 Human Entrez Gene: 54128 Mouse Entrez Gene: 302915 Rat Omim: 601785 Human SwissProt: Q3SZJ9 Cow SwissProt: O15305 Human SwissProt: Q9Z2M7 Mouse Unigene: 625732 Human Unigene: 9699 Mouse Unigene: 58278 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 产品图片 |  Sample: Hela(Human) Cell Lysate at 40 ug Primary: Anti-PMM2 (bs-12741R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 28 kD Observed band size: 13 kD  Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PMM2) Polyclonal Antibody, Unconjugated (bs-12741R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining. |
