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Rabbit Anti-SPFH2/FITC Conjugated antibody
FITC标记的内质网脂质转运相关蛋白2抗体
| 英文名称 | Anti-SPFH2/FITC |
| 中文名称 | FITC标记的内质网脂质转运相关蛋白2抗体 |
| 别 名 | C8orf2; Chromosome 8 open reading frame 2; Endoplasmic reticulum lipid raft associated protein 2; Endoplasmic reticulum lipid raft-associated protein 2; ER lipid raft associated 2; ERLIN 2; Erlin-2; ERLIN2; ERLN2_HUMAN; HGNC:1356; MGC87072; NET32; SPFH 2; SPFH domain containing protein 2; SPFH domain family member 2; SPFH domain-containing protein 2; SPFH2; SPG18; Stomatin prohibitin flotillin HflC/K domain containing protein 2; Stomatin-prohibitin-flotillin-HflC/K domain-containing protein 2. |
| 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 信号转导 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | |
| 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 | 38kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SPFH2 |
| 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: SPFH2 is a ubiquitously expressed 339 amino acid protein that belongs to the band 7/mec-2 family. Localized to lipid raft-like domains in the membrane of the endoplasmic reticulum (ER), SPFH2 plays a crucial role in the ER-associated degradation (ERAD) pathway that removes metabolically regulated and aberrant proteins from the ER. Specifically, SPFH2 associates with IP3R-I (Inositol 1,4,5-triphosphate receptor I), a substrate of the ERAD pathway, and facilitates its polyubiquitination and subsequent degradation. Three isoforms of SPFH2 are expressed due to alternative splicing events. Function: Component of the ERLIN1/ERLIN2 complex which mediates the endoplasmic reticulum-associated degradation (ERAD) of inositol 1,4,5-trisphosphate receptors (IP3Rs). Also involved in ITPR1 degradation by the ERAD pathway. Subunit: Interacts with activated ITPR1, independently of the degree of ITPR1 polyubiquitination (By similarity). Forms a heteromeric complex with ERLIN1. In complex with ERLIN1, interacts with RNF170. Subcellular Location: Endoplasmic reticulum membrane. Associated with lipid raft-like domains of the endoplasmic reticulum membrane. Tissue Specificity: Ubiquitous. DISEASE: Defects in ERLIN2 are the cause of spastic paraplegia autosomal recessive type 18 (SPG18) [MIM:611225]. A form of spastic paraplegia, 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 quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG18 is a severe form with onset in early childhood. Most affected individuals have severe psychomotor retardation. Some may develop significant joint contractures. Similarity: Belongs to the band 7/mec-2 family. Database links: Entrez Gene: 11160 Human Entrez Gene: 244373 Mouse Entrez Gene: 290823 Rat Omim: 611605 Human SwissProt: O94905 Human SwissProt: Q8BFZ9 Mouse SwissProt: B5DEH2 Rat Unigene: 705490 Human Unigene: 277699 Mouse Unigene: 12619 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic application |
SPFH2是一个普遍表达的339个氨基酸蛋白，属于带7／MEC-2家族。SPFH2定位于内质网（ER）膜中的脂筏状结构域，在ER相关降解（ERAD）途径中发挥关键作用，该途径从ER中去除代谢调节的和异常的蛋白质。特别地，SPFH2与作为ERAD通路底物的IP3R-I(肌醇1，4，5-三***酸受体I)结合，并促进其多泛素化和随后的降解。SPFH2的三种异构体由于选择性剪接事件而表达。
