Home > Product > Antibody > Rabbit Anti-DORFIN/FITC Conjugated antibody
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; UI
Cat:
SL11778R-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Sheep,)
Immunogen:
KLH conjugated synthetic peptide derived from human DORFIN/RNF19
Format:
Lyophilized or Liquid
Storage instructions:
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 ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
91kDa
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Unit:
Price: $
Product PDFs
Datasheet:


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 SLCterminus. 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: 36580 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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