FITC标记的AFG3样蛋白2/脊髓小脑共济失调蛋白28抗体
产品名称： FITC标记的AFG3样蛋白2/脊髓小脑共济失调蛋白28抗体
英文名称： Anti-AFG3L2/FITC
产品编号： HZ-11704R-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-AFG3L2/FITC Conjugated antibody
FITC标记的AFG3样蛋白2/脊髓小脑共济失调蛋白28抗体
| 产品编号 | bs-11704R-FITC |
| 英文名称 | Anti-AFG3L2/FITC |
| 中文名称 | FITC标记的AFG3样蛋白2/脊髓小脑共济失调蛋白28抗体 |
| 别 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 . |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 神经生物学 信号转导 泛素 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, |
| 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 |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human AFG3L2 (531-600aa) |
| 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: 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 Tissue Specificity: Ubiquitous. Highly expressed in the cerebellar Purkinje cells. DISEASE: Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) [MIM:610246]. It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment. Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5) [MIM:614487]. A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy. Similarity: In the N-terminal section; belongs to the AAA ATPase family. In the C-terminal section; belongs to the peptidase M41 family. Database links: Entrez Gene: 10939 Human Omim: 604581 Human SwissProt: Q9Y4W6 Human Unigene: 726355 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
AFG3L2是一种多通道膜金属蛋白酶，含有一个与多种细胞活性相关的AAA（ATP酶）结构域、一个锌依赖结合基序、一个RNA结合区和一个ATP/GTP结合位点。定位于线粒体膜，AGF3L2在骨骼肌和心脏中普遍表达，表达量最高。AFG3L2与酵母AFG3蛋白具有69%的相似性，与PalaPGEL的49%相似，这是一种被认为参与信号转导和伴侣样活性的线粒体蛋白。在线粒体中，AFG3L2与对虾蛋白形成一个复合物，相信它可以调节必需的蛋白质质量控制。这些蛋白质中任一种基因的突变可导致遗传性痉挛性截瘫，这是一种以肌肉痉挛、腿部僵硬和某些情况下失禁为特征的退行性脊髓疾病。