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Product Name E1 Ubiquitin Activating Enzyme Chinese Name Ubiquitin激活酶E1抗体 Alias Uba1; A1S9; A1S9 protein; A1S9T and BN75 temperature sensitivity complementing; A1S9T; A1ST; GXP 1; GXP1; MGC4781; Protein A1S9; UBA1_HUMAN; UBE 1 ; UBE 1X; UBE1; UBE1X; Ubiquitin activating enzyme E1; Ubiquitin-activating enzyme E1; Ubiquitin-like modifier-activating enzyme 1. Research Area Cell biology Neurobiology Signal transduction Epigenetics Immunogen Species Rabbit Clonality Polyclonal React Species Human, Rat, (predicted: Mouse, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 118kDa Cellular localization The nucleus cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human UBE1: 961-1058/1058 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail The protein encoded by this gene catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. This gene complements an X-linked mouse temperature-sensitive defect in DNA synthesis, and thus may function in DNA repair. It is part of a gene cluster on chromosome Xp11.23. Alternatively spliced transcript variants that encode the same protein have been described. [provided by RefSeq, Jul 2008].
Function:
Activates ubiquitin by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding an ubiquitin-E1 thioester and free AMP.
Subunit:
Monomer (By similarity). Interacts with GAN (via BTB domain).
Post-translational modifications:
ISGylated.
DISEASE:
Defects in UBA1 are the cause of spinal muscular atrophy X-linked type 2 (SMAX2) [MIM:301830]; also known as X-linked lethal infantile spinal muscular atrophy, distal X-linked arthrogryposis multiplex congenita or X-linked arthrogryposis type 1 (AMCX1). Spinal muscular atrophy refers to a group of neuromuscular disorders characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. SMAX2 is a lethal infantile form presenting with hypotonia, areflexia, and multiple congenital contractures.
Similarity:
Belongs to the ubiquitin-activating E1 family.
SWISS:
P22314
Gene ID:
7317
Database links:Entrez Gene: 7317 Human
Entrez Gene: 22201 Mouse
Omim: 314370 Human
SwissProt: P22314 Human
SwissProt: Q02053 Mouse
Unigene: 533273 Human
Unigene: 1104 Mouse
Unigene: 474674 Mouse
Unigene: 11800 Rat
Product Picture Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (E1 Ubiquitin Activating Enzyme) Polyclonal Antibody, Unconjugated (SL6835R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.Blank control(blue):293T Cells(fixed with 2% paraformaldehyde (10 min) and then permeabilized with ice-cold 90% methanol for 30 min on ice).
Primary Antibody: Rabbit Anti-E1 Ubiquitin Activating Enzyme/AF488 Conjugated antibody (SL6835R-AF488), Dilution: 1ug in 100 ul 1X PBS containing 0.5% BSA;
Isotype Control Antibody: Rabbit IgG/AF488(orange) ,used under the same conditions.
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