产品名称
UBA1 Rabbit Polyclonal Antibody
别名
UBA1; A1S9T; UBE1; Ubiquitin-like modifier-activating enzyme 1; Protein A1S9; Ubiquitin-activating enzyme E1
蛋白名称
Ubiquitin-like modifier-activating enzyme 1
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=7317
Human Swissprot No.
P22314
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P22314/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=22201
Mouse Swissprot No.
Q02053
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q02053
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=314432
Rat Swissprot Link
http://www.uniprot.org/uniprot/Q5U300
免疫原
The antiserum was produced against synthesized peptide derived from the N-terminal region of human UBA1. AA range:91-140
特异性
UBA1 Polyclonal Antibody detects endogenous levels of UBA1 protein.
稀释度
WB 1:500 - 1:2000. ELISA: 1:20000. Not yet tested in other applications.
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Detected in erythrocytes (at protein level). Ubiquitous.
细胞定位
Cytoplasm . Mitochondrion . Nucleus .; [Isoform 1]: Nucleus .; [Isoform 2]: Cytoplasm .
信号通路
Ubiquitin mediated proteolysis;Parkinson's disease;
功能
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.,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.,miscellaneous:There are two active sites within the E1 molecule, allowing it to accommodate two ubiquitin moieties at a time, with a new ubiquitin forming an adenylate intermediate as the previous one is transferred to the thiol site.,pathway:Protein modification; protein ubiquitination.,similarity:Belongs to the ubiquitin-activating E1 family.,subunit:Monomer (By similarity). Interacts with GAN (via BTB domain).,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
