UBE1, also known as UBA1, belongs to the ubiquitin-activating E1 family. UBE1 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. UBE1 catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. It also catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation 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 a ubiquitin-E1 thioester and free AMP. Defects in UBA1 can cause spinal muscular atrophy X-linked type 2 (SMAX2), 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.
规格 | 价格/CNY | 货期 | 数量 | |
---|---|---|---|---|
50 μg | ¥ 2,530 | 5日内发货 |
生物活性 | Testing in progress |
产品描述 | UBE1, also known as UBA1, belongs to the ubiquitin-activating E1 family. UBE1 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. UBE1 catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. It also catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation 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 a ubiquitin-E1 thioester and free AMP. Defects in UBA1 can cause spinal muscular atrophy X-linked type 2 (SMAX2), 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. |
种属 | Human |
表达系统 | Baculovirus-Insect Cells |
标签 | His,GST |
蛋白编号 | A0A024R1A3 |
别名 | UBE1, ubiquitin-like modifier activating enzyme 1, UBA1A, SMAX2, CTD-2522E6.1, A1S9, POC20, CFAP124, A1ST, GXP1, AMCX1, A1S9T, UBE1X |
蛋白构建 | A DNA sequence encoding the human UBA1 (NP_003325.2) (Ser 2-Arg 1058) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus. |
蛋白纯度 | > 90 % as determined by SDS-PAGE |
分子量 | Approxiamtely 146 kDa |
内毒素 | < 1.0 EU per μg of the protein as determined by the LAL method |
缓冲液 | Lyophilized from sterile 50mM Tris, 100mM NaCl, pH 7.4, 10% gly, 0. 5mM GSHPlease contact us for any concerns or special requirements. Normally 5 % - 8 % trehalose, mannitol and 0. 01% Tween 80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA. |
复溶方法 | A hardcopy of datasheet with reconstitution instructions is sent along with the products. Please refer to it for detailed information. |
存储 |
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles. |
运输方式 |
In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature.Bulk packages of recombinant proteins are provided as frozen liquid. They are shipped out with blue ice unless customers require otherwise. |
研究背景 | UBE1, also known as UBA1, belongs to the ubiquitin-activating E1 family. UBE1 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. UBE1 catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. It also catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation 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 a ubiquitin-E1 thioester and free AMP. Defects in UBA1 can cause spinal muscular atrophy X-linked type 2 (SMAX2), 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. |
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Ubiquitin Activating Enzyme E1/UBA1 Protein, Human, Recombinant (His & GST) UBE1 ubiquitin-like modifier activating enzyme 1 UBA1A SMAX2 SMAX-2 UBE 1 CTD-2522E6.1 CFAP-124 GXP-1 A1S9 POC20 CFAP124 A1ST GXP 1 CFAP 124 SMAX 2 GXP1 AMCX1 UBE-1 A1S9T POC-20 UBE1X AMCX-1 AMCX 1 POC 20 recombinant recombinant-proteins proteins protein