FITC標記的內質網脂質轉運相關蛋白2抗體上海滬震實業有限公司

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產品名稱: FITC標記的內質網脂質轉運相關蛋白2抗體
英文名稱: Anti-SPFH2/FITC
抗體貨號: HZ-10720R-FITC
產品規格: 100ul
級    別: , , 分析純, , 分析純,
產品產地: 中國/上海
品牌商標: HZbscience
價    格: 2980元
抗原: Rabbit
抗原來源: Rabbit
抗體來源: Rabbit
適用物種: 
FITCconjugate:FITC
應用范圍: ICC=1:50-200 IF=1:50-200
更新時間: 2020/5/12 11:00:00
詳細資料:  實驗方法技術資料
瀏覽人數:787
誠信指數:5884點
了解更多:進入公司展臺
使用范圍:僅限科研使用,不能應用于臨床
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產品詳細描述

 Rabbit Anti-SPFH2/FITC Conjugated antibody

FITC標記的內質網脂質轉運相關蛋白2抗體

 

英文名稱 Anti-SPFH2/FITC
中文名稱 FITC標記的內質網脂質轉運相關蛋白2抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經生物學  信號轉導  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應  
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 38kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SPFH2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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:
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是一個普遍表達的339個氨基酸蛋白,屬于帶7/MEC-2家族。SPFH2定位于內質網(ER)膜中的脂筏狀結構域,在ER相關降解(ERAD)途徑中發揮關鍵作用,該途徑從ER中去除代謝調節的和異常的蛋白質。特別地,SPFH2與作為ERAD通路底物的IP3R-I(肌醇1,4,5-三***酸受體I)結合,并促進其多泛素化和隨后的降解。SPFH2的三種異構體由于選擇性剪接事件而表達。

生物在線聲明:以上所展示的信息由企業自行提供,內容的真實性、準確性和合法性由發布企業負責。生物在線對此不承擔任何保證責任。

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