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Rabbit Anti-DORFIN/BF594 Conjugated antibody (bs-11778R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11778R-BF594
英文名稱 Rabbit Anti-DORFIN/BF594 Conjugated antibody
中文名稱 BF594標記的環指蛋白19抗體
別    名 Dorfin; Double ring finger protein; Double ring-finger protein; E3 ubiquitin-protein ligase RNF19A; p38; p38 protein; Ring finger protein 19; RING finger protein 19A; Ring IBR ring domain containing protein Dorfin; RN19A_HUMAN; RNF19; RNF19A; Ubce7ip2; UIP117; XYbp; AA032313; DKFZP566B1346.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞生物  神經生物學  泛素  
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 91kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DORFIN/RNF19
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:
Dorfin is a multi-pass membrane, RING-IBR type, E3 ubiquitin-protein ligase. It is widely expressed with highest levels found in heart and ubiquitous expression throughout the central nervous system. Dorfin functions by accepting ubiquitin in the form of a thioester from UBCH7 and UBC8 and then transferring it to the targeted substrates. Dorfin is responsible for ubiquitylating synphilin-1, CaSR and mutant variants of SOD-1, a protein at fault for familial ALS (amyotrophic lateral sclerosis). Dorfin physically interacts with VCP (Valosin-containing protein) via its C-terminus. Together these two proteins are associated with the formation of ubiquitylated inclusions (UBIs) that characterize many neurodegenerative disorders, such as Parkinson’s disease and ALS. This association with UBIs suggests that Dorfin plays an important role in the disease process.

Function:
E3 ubiquitin-protein ligase which accepts ubiquitin from E2 ubiquitin-conjugating enzymes UBE2L3 and UBE2L6 in the form of a thioester and then directly transfers the ubiquitin to targeted substrates, such as SNCAIP or CASR. Specifically ubiquitinates pathogenic SOD1 variants, which leads to their proteasomal degradation and to neuronal protection.

Subcellular Location:
Membrane. Cytoplasm.

Tissue Specificity:
Widely expressed, with highest levels in heart. Ubiquitously expressed in the central nervous system.

Post-translational modifications:
Phosphorylated upon DNA damage, probably by ATM or ATR.

Similarity:
Belongs to the RBR family. RNF19 subfamily.
Contains 1 IBR-type zinc finger.
Contains 2 RING-type zinc fingers.

Database links:

Entrez Gene: 25897 Human

Entrez Gene: 30945 Mouse

Entrez Gene: 654326 Pig

Entrez Gene: 362900 Rat

Entrez Gene: 540195 Cow

Entrez Gene: 475049 Dog

NCBI: 35493782 Human

Omim: 607119 Human

SwissProt: Q9NV58 Human

SwissProt: P50636 Mouse

SwissProt: Q2VJ60 Pig

Unigene: 292882 Human

Unigene: 5181 Mouse

Unigene: 94631 Pig



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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