| 產(chǎn)品編號 | bs-17380R-BF647 |
| 英文名稱1 | Rabbit Anti-HPS2/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的HPS2蛋白抗體 |
| 別 名 | Adapter related protein complex 3 beta 1 subunit; Adapter-related protein complex 3 subunit beta-1; Adaptor protein complex AP-3 subunit beta-1; Adaptor protein complex AP3 beta1 subunit; ADTB3; ADTB3A; AP-3 complex subunit beta-1; AP3 complex beta1 subunit; AP3B1; AP3B1_HUMAN; Beta-3A-adaptin; Beta3A adaptin; Clathrin assembly protein complex 3 beta 1 large chain; Clathrin assembly protein complex 3 beta-1 large chain; HPS; PE. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 信號轉(zhuǎn)導(dǎo) 轉(zhuǎn)運(yùn)蛋白 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human, Mouse, (predicted: Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, Cat, Xenopus laevis) |
| 產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 121kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HPS2 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
background: This gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. The encoded protein is part of the heterotetrameric AP-3 protein complex which interacts with the scaffolding protein clathrin. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 2. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2012] Function: Subunit of non-clathrin- and clathrin-associated adaptor protein complex 3 that plays a role in protein sorting in the late-Golgi/trans-Golgi network (TGN) and/or endosomes. The AP complexes mediate both the recruitment of clathrin to membranes and the recognition of sorting signals within the cytosolic tails of transmembrane cargo molecules. AP-3 appears to be involved in the sorting of a subset of transmembrane proteins targeted to lysosomes and lysosome-related organelles. Subcellular Location: Golgi apparatus. Cytoplasmic vesicle > clathrin-coated vesicle membrane. Golgi apparatus. Component of the coat surrounding the cytoplasmic face of coated vesicles located at the Golgi complex. Tissue Specificity: Ubiquitously expressed. Post-translational modifications: Phosphorylated on serine residues. DISEASE: Defects in AP3B1 are the cause of Hermansky-Pudlak syndrome type 2 (HPS2) [MIM:608233]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. HPS2 differs from the other forms of HPS in that it includes immunodeficiency in its phenotype and patients with HPS2 have an increased susceptibility to infections. Similarity: Belongs to the adaptor complexes large subunit family. Database links: Entrez Gene: 8546 Human Entrez Gene: 11774 Mouse Entrez Gene: 443724 Xenopus laevis Omim: 603401 Human SwissProt: O00203 Human SwissProt: Q9Z1T1 Mouse Unigene: 532091 Human Unigene: 21185 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
