FITC标记的SPG48蛋白抗体
产品名称： FITC标记的SPG48蛋白抗体
英文名称： Anti-SPG48/FITC
产品编号： HZ-11760R-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-SPG48/FITC Conjugated antibody
FITC标记的SPG48蛋白抗体
| 产品编号 | bs-11760R-FITC |
| 英文名称 | Anti-SPG48/FITC |
| 中文名称 | FITC标记的SPG48蛋白抗体 |
| 别 名 | Hypothetical protein LOC9907; KIAA0415; Uncharacterized protein KIAA0415; AP5Z1_HUMAN. |
| 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 神经生物学 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, |
| 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 | 89kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SPG48 (288-370aa) |
| 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: Chromosome 7 is about 158 milllion bases long, encodes over 1000 genes and makes up about 5% of the human genome. Chromosome 7 has been linked to Osteogenesis imperfecta, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome. The deletion of a portion of the q arm of chromosome 7 is associated with Williams-Beuren syndrome, a condition characterized by mild mental retardation, an unusual comfort and friendliness with strangers and an elfin appearance. Deletions of portions of the q arm of chromosome 7 are also seen in a number of myeloid disorders including cases of acute myelogenous leukemia and myelodysplasia. KIAA0415 is a 807 amino acid protein that exists as three alternatively spliced isoforms. The KIAA0415 gene product has been provisionally designated KIAA0415 pending further characterization. Function: The function of SPG48 remains unknown. There are 3 named isoforms produced by alternative splicing. Subunit: Probably part of the adapter protein complex 5 (AP-5) atetramer composed of AP5B1, AP5M1, AP5S1 and AP5Z1. Interacts withZFYVE26 and SPG11. Subcellular Location: Cytoplasm. Nucleus. Note=By SDS-PAGE, 2isoforms have been observed, the shorter seems to be predominantlynuclear and the longer is mostly cytoplasmic (PubMed:20613862). DISEASE: Defects in AP5Z1 are the cause of spastic paraplegiaautosomal recessive type 48 (SPG48) [MIM:613647]. A form of spasticparaplegia, a neurodegenerative disorder characterized by a slow,gradual, progressive weakness and spasticity of the lower limbs.Rate of progression and the severity of symptoms are quitevariable. Initial symptoms may include difficulty with balance,weakness and stiffness in the legs, muscle spasms, and dragging thetoes when walking. In some forms of the disorder, bladder symptoms(such as incontinence) may appear, or the weakness and stiffnessmay spread to other parts of the body. Database links: UniProtKB/Swiss-Prot: O43299.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
7号染色体长约158个碱基，编码超过1000个基因，约占人类基因组的5%。第7号染色体与成骨不全、潘德红综合征、Lissencephaly、瓜氨酸血症和SuWakman钻石综合征有关。第7号染色体Q臂的缺失与威廉姆斯Bururn综合征有关，该症状以轻度智力迟钝、异常的舒适和对陌生人的友好和小精灵的外表为特征。7号染色体Q臂部分的缺失也见于许多髓系疾病中，包括急性髓性白血病和骨髓增生异常症。KIAA0415是一种807个氨基酸的蛋白质，以三个交替剪接的亚型存在。KIAA0415基因产物已暂时被指定为KIAA0415，有待进一步鉴定。