| 英文名稱 | Anti-AFG3L2/FITC |
| 中文名稱 | FITC標記的AFG3樣蛋白2/脊髓小腦共濟失調蛋白28抗體 |
| 別 名 | SCA28; AFG3 (ATPase family gene 3, yeast) like 2; AFG3 ATPase family gene 3 like 2 (yeast); AFG3 ATPase family gene 3 like 2; AFG3 like protein 2; ATPase family gene 3 like 2; ATPase family gene 3 yeast; FLJ25993; Paraplegin like protein; SCA28; Spinocerebellar ataxia 28; AFG32_HUMAN . |
| 說 明 書 | 100ul |
| 研究領域 | 腫瘤 細胞生物 神經生物學 信號轉導 泛素 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應 | Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, |
| 產品應用 | 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 |
| 細胞定位 | 細胞膜 |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human AFG3L2 (531-600aa) |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 |
| 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. |
| 產品介紹 | background:
AFG3L2 is a multi-pass membrane metalloprotease that contains one AAA (ATPase associated with diverse cellular activities) domain, a zinc-dependent binding motif, an RNA-binding region and an ATP/GTP binding site. Localizing to the mitochondrial membrane, AFG3L2 is ubiquitously expressed with highest expression levels in skeletal muscle and heart. AFG3L2 shares 69% similarity with the yeast Afg3 protein and 49% similarity with Paraplegin, a protein of mitochondria that is thought to be involved in signal transduction and chaperone-like activities. In mitochondria, AFG3L2 forms a complex with Paraplegin that is believed to regulate essential protein quality control. Mutations in the gene encoding either one of these proteins can result in hereditary spastic paraplegia, a degenerative spinal cord disorder that is characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence.
Function:
AFG3L2 is a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. AFG3L2 is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders and is a putative ATP dependent protease
Subunit:
Homooligomer. Interacts with SPG7; the interaction is required for the efficient assembly of mitochondrial complex I.
Subcellular Location:
Mitochondrial membrane; multipass membrane protein |
