内质网脂质转运相关蛋白2抗体
规格：1mg/1ml
英文名： SPFH2
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; E
分子量： 38kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human SPFH2
交叉反应：Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep,
细胞定位：细胞浆 细胞膜
内质网脂质转运相关蛋白2抗体产品介绍：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).内质网脂质转运相关蛋白2抗体 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 t内质网脂质转运相关蛋白2抗体he 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. Gene ID: 11160 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 applications.
内质网脂质转运相关蛋白2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 神经生物学 信号转导 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid
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