DORFIN,环指蛋白19抗体

产品编号HZ-11778R
英文名称DORFIN
中文名称环指蛋白19抗体
别 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.
说 明 书0.1ml 0.2ml
研究领域心血管 细胞生物 神经生物学 泛素
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
DORFIN,环指蛋白19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.
分 子 量91kDa
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human DORFIN/RNF19
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.
DORFIN,环指蛋白19抗体PubMedPubMed
产品介绍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

DORFIN,环指蛋白19抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.