FITC标记的HPS2蛋白抗体
产品名称： FITC标记的HPS2蛋白抗体
英文名称： Anti-HPS2/FITC
产品编号： HZ-17380R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-HPS2/FITC Conjugated antibody
FITC标记的HPS2蛋白抗体
| 英文名称 | Anti-HPS2/FITC |
| 中文名称 | FITC标记的HPS2蛋白抗体 |
| 别 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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 免疫学 信号转导 转运蛋白 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, Cat, Xenopus laevis |
| 产品应用 | 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 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HPS2 |
| 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. |
| 产品介绍 | 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: 767602 Cow Entrez Gene: 403459 Dog Entrez Gene: 8546 Human Entrez Gene: 11774 Mouse Entrez Gene: 100049670 Pig Entrez Gene: 443724 Xenopus laevis Omim: 603401 Human SwissProt: Q32PG1 Cow SwissProt: Q7YRF1 Dog 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 |
该基因编码一种蛋白质，可能在与黑色素体、血小板致密颗粒和溶酶体相关的细胞器生物发生中发挥作用。编码的蛋白是与支架蛋白网格蛋白相互作用的异四聚体AP-3蛋白复合物的一部分。该基因的突变与2型Helman氏综合征相关。发现了两个编码不同亚型的转录变体。[ RefSeq，2012月11日]